Retroperitoneal Lymphadenopathy Research Articles

Whipple’s disease presenting as weight gain and constipation in a Chinese woman

BackgroundWhipple’s disease is a chronic infection due to Tropheryma whipplei, commonly reported in the Caucasian but not in the Chinese population.Case presentationA 52-year-old female with good past health, was diagnosed with Whipple’s disease, presenting with constipation, unintentional weight gain, and fleeting polyarthralgia. Investigations prior to admission showed raised CA125 and computed tomography of the abdomen showed multiple retroperitoneal mesenteric lymphadenopathies. Extensive investigations performed on secondary causes of weight gain were unrevealing. Subsequent PET-CT scan revealed generalized lymphadenopathy involving the left deep cervical, supraclavicular, and retroperitoneal mesenteric area. Excisional biopsy of the left supraclavicular lymph node was performed, with histology showing infiltrations of Periodic acid-Schiff positive foamy macrophages. T. whipplei DNA was detected in her serum, saliva, stool, and lymph node by PCR targeting the 16S ribosomal RNA gene. She was started on intravenous ceftriaxone, and then stepped down to oral antibiotics for a total of 44 months. The recurrence of fever after 12 days of ceftriaxone raised the suspicion of Immune Reconstitution Inflammatory Syndrome (IRIS). Serial imaging showed a gradual reduction in the size of retroperitoneal lymphadenopathies. Literature review on Whipple’s disease in the Chinese population identified 13 reports of detectable T. whipplei DNA in clinical specimens. The majority of the cases were pneumonia, followed by culture-negative endocarditis, encephalitis, and skin and soft tissue infection. However, most patients with pneumonia were diagnosed based on next generation sequencing alone, with the resolution of pulmonary infiltrates without adequate duration of antibiotics, suggesting the possibility of colonization instead of infection. The recommendation of long-term doxycycline suppression after treatment may be supported by the slow response of retroperitoneal lymphadenopathies to antibiotics in our patient.ConclusionsUnintentional weight gain and constipation could be atypical presentations of Whipple’s disease. It is a rare disease in the Chinese population despite the advancement of molecular techniques in the diagnosis of infections. A prolonged course of antibiotics may be required due to slow clinical response as documented by serial imaging in our case. The possibility of IRIS should be considered in patients with breakthrough fever during treatment of Whipple’s disease.

EBV-related lymphoproliferative disease in Wiskott-Aldrich Syndrome patient

CaseWiskott-Aldrich Syndrome (WAS) is a rare X-linked inborn error of immunity caused by mutations in the WAS gene. It is classically characterized by immunodeficiency, eczema, and micro-thrombocytopenia. It has been known since the 1960s that patients with WAS have an increased risk of lymphoproliferative disease though the exact incidence remains unknown in the American population. Limited case reports have discussed EBV-related lymphoproliferative disease in patients with WAS. We present a case of a 9-year-old boy with known WAS complicated by eczematous rash, thrombocytopenia, recurrent ear infections, and monoclonal gammopathy who was found to have submandibular EBV-associated lymphoid hyperplasia with associated lung and retroperitoneal lymphadenopathy. Family had been offered treatment with hematopoietic stem cell transplant but declined multiple times in the past. Earlier in the year, he presented with possible MIS-C with negative SARS-CoV-2 PCR. He presented to our hospital with mastoiditis and lymphadenopathy. Physical examination showed severe eczema on hands and tender right mastoid. Laboratory evaluation showed thrombocytopenia, elevated IgG of 6290, IgA of 744, IgE of 827, low IgM of 41, and 14% response to pneumococcal titers. He was empirically treated with intravenous antibiotics. ENT performed right postauricular incision and drainage and the culture grew Hemophilus influenza. Throughout his hospital stay, his submandibular lymphadenopathy became more prominent despite treatment. Core needle biopsy of right submandibular lymph node was suggestive of EBV-associated lymphoid hyperplasia. EBV PCR and antibodies were both positive. CT chest, abdomen, and pelvis revealed multifocal pulmonary lymphadenopathy and a diffuse, bilateral nodularity as well as retroperitoneal and mesenteric lymphadenopathy. He was given four doses of weekly Rituximab, which successfully decreased EBV viremia below linear detectability. Immunoglobulin replacement therapy (IgRT) was initiated. Bronchoalveolar lavage and lung biopsy were performed and are results are currently pending. DiscussionWe present a case of a 9-year-old boy with known WAS awaiting transplant who was found to have submandibular EBV-associated lymphoid hyperplasia with associated lung and retroperitoneal lymphadenopathy. While lymphoproliferative disease is a known complication of WAS, EBV-related lymphoproliferative disease in WAS patients has only been reported as case reports and remains a rare but known complication of patient with WAS.

Burned-out Testicular Tumor Presenting as Cervical and Retroperitoneal Lymphadenopathy

A burned-out tumor of the testis is a rare clinical entity. It usually regresses spontaneously without any treatment and presents as metastasis to the retroperitoneum, mediastinum, cervical region, lungs, and liver. Clinical examination and ultrasound of the testis are pivotal to raising suspicion for the diagnosis of the burned-out tumor. Here, we report a case of a 23-year-old male who presented to us with a complaint of swelling on the left side of the neck. Biopsy from the neck mass suggested metastatic malignant mixed germ cell tumor, consistent with yolk sac tumor and embryonal carcinoma. The whole-body fluorodeoxyglucose-positron emission tomography-computed tomography suggested conglomerated left neck mass and retroperitoneal lymph node enlargement. Ultrasound of the scrotum suggested heterogeneously hypoechoic echotexture with few foci of microcalcification in the right testes. He underwent a right high inguinal orchidectomy, which showed no evidence of the tumor. The patient received four cycles of chemotherapy with bleomycin, etoposide, and a cisplatin-based combination regimen with a favorable response to the therapy. In view of residual viable disease and raised tumor markers, the patient received second-line chemotherapy with paclitaxel, ifosfamide, and carboplatin.

P025 The knee that gave it away: a case report of melioidosis

Abstract Background/Aims Melioidosis, an infectious disease caused by the Burkholderia pseudomallei is very rare within the UK. This gram-negative bacterium is endemic in Southeast Asia predominately Thailand and Northern Australia which is found within the soil and fresh surface water. We present a challenging case of melioidosis who was diagnosed following a culture of a discharging knee swelling. Methods N/A Results 35-year-old, Caucasian gentleman with no previous past medical history presented with a ten week history of diffuse abdominal pain, weight loss, dry cough, fatigue and night sweats. On examination, he appeared pale and initial bloods showed a CRP of 229 mg/L. His other initial observations and baseline blood tests were normal. Abdominal ultrasound detected free peritoneal fluid and a subsequent CT chest and abdominal showed abnormal thickening of his small bowel, mediastinal and retroperitoneal lymphadenopathy along with bilateral pulmonary nodules. A laparoscopy and laparotomy with peritoneal, intraperitoneal and mesenteric lymph node biopsies were inconclusive. Microscopy and culture (MC&S) as well as acid fast bacilli MC&S were both negative. He continued to have systemic symptoms, as well as elevated inflammatory markers. He underwent a bronchoscopy which showed erythematous and a nodular endobronchial mucosa with white plaques seen within the lower trachea and minor narrowing of the lumen. Endobronchial biopsies showed chronic inflammation with no evidence of any malignant cells. Repeated bloods including retroviral screening, anti-nuclear antibody (ANA) and antinuclear cytoplastic antibodies (ANCA) and serum ACE were normal. At this stage, the clinical picture was suggestive of sarcoidosis, however a subsequent rigid bronchoscopy and biopsy did not evidence of granulomas or vasculitis. The patient started to develop increasing right knee swelling with difficulty in walking. The swelling burst open, and the discharging fluid was sent for MC&S. This was positive for Burkholderia pseudomallei. The gentleman completed a 20-week course of antibiotics with clinical resolution. Conclusion Meliodosis can cause a wide range of clinical manifestations varying from asymptomatic to septicemia, osteomyelitis, abscesses, septic arthritis and encephalomyelitis. Our case had travelled to Thailand around 8 months prior to the onset of symptoms. This highlights the possible long latent period between exposure and clinical presentation. Definite diagnosis is through isolation of Burkholderia pseudomallei in cultures. This proved challenging in our case, as multiple biopsies and cultures from different sites did not grow the bacterium. The diagnosis was confirmed following knee fluid culture. Musculoskeletal involvement of melioidosis is a known presentation and its potential to mimic other conditions, may render a diagnostic challenge especially when it occurs outside an endemic area. The case highlights the need for rheumatologists to have a high level of clinical suspicion for early detection, isolation of the organism and proper disease management. Disclosure A. Maksoud: None. L. Sammut: Other; LS has received funding for educational events and activities - from Lilly, Roche/Chugai.

Perirenal Mass: An Unusual Presentation of Primary Renal Lymphoma

Primary renal lymphoma (PRL) is a rare type of Non-Hodgkin's Lymphoma (NHL), with B cell lymphoma being the most common subtype, to date; no more than 100 cases of PRL have been described and less commonly, when the involvement of perirenal space is isolated, without any renal parenchymal infiltration or contiguous abdominal adenopathy. In this article, we report a case of non-Hodgkin’s lymphoma (NHL) in 65-year-old man, presented initially for flank pain, fever and unintentional weight loss associated with dysuria and pollakiuria. Abdominal ultrasound revealed a hyperechoic masse of the left perinephric space without focal lesions in the kidneys. The CT scan confirmed the solid character of the perirenal abnormality. No intra or retroperitoneal lymphadenopathy was noted. Biopsy of the left perirenal mass using local anesthesia was performed under ultrasound guidance. The histopathological report was compatible with non-Hodgkin’s lymphoma and FDG PET-SCAN confirmed the primary localization of renal lymphoma in the left perinephric space. The aim of this work is to report a new case of primary renal lymphoma, as one of neoplasic causes of perirenal masses, describe the spectrum of imaging findings of these entities and discuss how to differentiate it from other causes of perirenal masses.

MP33-18 SURGICAL AND ONCOLOGIC OUTCOMES OF SURGERY IN EARLY METASTATIC SEMINOMA: MULTI-INSTITUTIONAL RETROSPECTIVE STUDY

You have accessJournal of UrologyCME1 Apr 2023MP33-18 SURGICAL AND ONCOLOGIC OUTCOMES OF SURGERY IN EARLY METASTATIC SEMINOMA: MULTI-INSTITUTIONAL RETROSPECTIVE STUDY Sanam Ladi-Seyedian, Muhannad Alsyouf, Brian Hu, Clint Cary, Timothy Masterson, Lawrence Einhorn, Nabil Adra, Stephen A. Boorjian, Christian Kollmannsberger, Anne Schuckman, Alan So, Peter Black, Aditya Bagrodia, Eila Skinner, Mehrdad Alemozaffar, Timothy Brand, Scott Eggener, Phillip Pierorazio, Kelly Stratton, Lucia Nappi, Craig Nichols, Axel Heidenreich, and Siamak Daneshmand Sanam Ladi-SeyedianSanam Ladi-Seyedian More articles by this author , Muhannad AlsyoufMuhannad Alsyouf More articles by this author , Brian HuBrian Hu More articles by this author , Clint CaryClint Cary More articles by this author , Timothy MastersonTimothy Masterson More articles by this author , Lawrence EinhornLawrence Einhorn More articles by this author , Nabil AdraNabil Adra More articles by this author , Stephen A. BoorjianStephen A. Boorjian More articles by this author , Christian KollmannsbergerChristian Kollmannsberger More articles by this author , Anne SchuckmanAnne Schuckman More articles by this author , Alan SoAlan So More articles by this author , Peter BlackPeter Black More articles by this author , Aditya BagrodiaAditya Bagrodia More articles by this author , Eila SkinnerEila Skinner More articles by this author , Mehrdad AlemozaffarMehrdad Alemozaffar More articles by this author , Timothy BrandTimothy Brand More articles by this author , Scott EggenerScott Eggener More articles by this author , Phillip PierorazioPhillip Pierorazio More articles by this author , Kelly StrattonKelly Stratton More articles by this author , Lucia NappiLucia Nappi More articles by this author , Craig NicholsCraig Nichols More articles by this author , Axel HeidenreichAxel Heidenreich More articles by this author , and Siamak DaneshmandSiamak Daneshmand More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000003266.18AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: The long-term toxicities of chemotherapy and radiotherapy can represent a significant burden to testicular cancer survivors. Although retroperitoneal lymph node dissection (RPLND) is an established treatment for testicular germ cell tumors, little data exists on its efficacy as a front-line treatment in early metastatic (stage II) seminoma. This study aims to assess the surgical and oncologic outcomes of RPLND as the primary treatment of stage II seminoma using multi-institutional dataset. METHODS: In this retrospective study, we included adult patients with Stage II testicular seminoma who underwent RPLND as the first-line treatment for their early metastatic disease. Patients’ characteristics, perioperative findings, and recurrence data were reviewed from 12 institutions in the North America and 1 site in Europe. RESULTS: A total of 94 patients with a median age of 35 (IQR 31 – 43) were enrolled with retroperitoneal lymphadenopathy between 1 - 5 cm. Two cases were performed robotically. An average of 23 (IQR 16 – 37) lymph nodes were removed. Pathologic upstaging occurred in 30% of patients. Of the 94 enrolled patients,11% were pN0, 30% pN1, 56% pN2, and 3% pN3. Overall, the recurrence free survival at 3 years was 82% and overall survival was 100%. Patient with pN2-3 showed significantly worse recurrence free survival than pN1 disease (78.5% versus 92%; P=0.04) (Figure 1). shows the surgical and postoperative outcomes of the cohort stratified by pathologic staging. In total, 7 (7.5%) patients experienced post-operative complications including ileus, incision ulceration, pulmonary embolism and chylous ascites. CONCLUSIONS: The finding of this retrospective multi-institutional study establishes RPLND as a viable option for first-line treatment of testicular seminoma with clinically low-volume retroperitoneal lymphadenopathy. It is associated with a low complication rate and high recurrence-free survival. Source of Funding: None © 2023 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 209Issue Supplement 4April 2023Page: e457 Advertisement Copyright & Permissions© 2023 by American Urological Association Education and Research, Inc.MetricsAuthor Information Sanam Ladi-Seyedian More articles by this author Muhannad Alsyouf More articles by this author Brian Hu More articles by this author Clint Cary More articles by this author Timothy Masterson More articles by this author Lawrence Einhorn More articles by this author Nabil Adra More articles by this author Stephen A. Boorjian More articles by this author Christian Kollmannsberger More articles by this author Anne Schuckman More articles by this author Alan So More articles by this author Peter Black More articles by this author Aditya Bagrodia More articles by this author Eila Skinner More articles by this author Mehrdad Alemozaffar More articles by this author Timothy Brand More articles by this author Scott Eggener More articles by this author Phillip Pierorazio More articles by this author Kelly Stratton More articles by this author Lucia Nappi More articles by this author Craig Nichols More articles by this author Axel Heidenreich More articles by this author Siamak Daneshmand More articles by this author Expand All Advertisement PDF downloadLoading ...

Abdominal Pain—An Ambiguous Pancreatic Cyst

Question: A 74-year-old man presented with vague abdominal pain. There was no personal history of malignancy or weight loss. Serum laboratory tests were significant for a total bilirubin of 3.4 mg/dL but direct bilirubin of 0.20 mg/dL. Liver enzymes, lactate dehydrogenase, carcino-embryonic antigen, and cancer antigen 19-9 were all within normal range. Computed tomographic (CT) imaging (Figure A and B) revealed a 6 × 2.9 × 2.7 cm pancreatic mass in the body of the pancreas with associated mesenteric and left-side retro-peritoneal para-aortic lymphadenopathy without significant ductal dilation. Endoscopic ultrasound (EUS) examination (Figure C and D) demonstrated a nearly completely cystic mass measuring 2.5 × 2.2 cm. A plane appeared to separate the cystic mass from the pancreatic body itself. Multiple abnormal, oval, hypo-echoic lymph nodes were visualized in the peri-gastric region as well. EUS-guided core biopsies were obtained from the cyst wall.

Primary Thromboprophylaxis and the Risk of Venous Thromboembolic Events in Patients With Testicular Germ Cell Tumors Treated With Cisplatinum-Based Chemotherapy.

Cisplatinum-based chemotherapy is associated with an increased risk of venous thromboembolism (VTE). We hypothesized that primary thromboprophylaxis in patients with testicular germ cell tumors (GCT) undergoing cisplatinum-based chemotherapy can reduce the risk of VTE. In this single-center retrospective cohort study, we investigated the increased use of primary thromboprophylaxis between January 2000 and December 2021 at our institution and its effect on the risk of VTE. Patients with GCT undergoing adjuvant or curative cisplatinum-based chemotherapy were included. Three hundred forty-six patients with GCT initiating a cisplatinum-based therapy were included in the study, of whom 122 (35%) were treated in the adjuvant and 224 (65%) in the curative setting, respectively. VTE events occurred in 49 (14.2%) patients. In univariable competing risk analysis, a higher clinical tumor stage and large retroperitoneal lymphadenopathy (RPLND >5 cm) were the strongest predictors of an elevated VTE risk (SHR for stage IIC - IIIC: 2.6 (95%CI: 5.0-24.7, P < .001), SHR for RPLN: 2.36 (95%CI: 1.27-4.4, P < .007)). The proportion of patients receiving primary thromboprophylaxis strongly increased over time and reached 100% in CS IIC-III patients from 2019 onwards. After adjusting for tumor stage, primary thromboprophylaxis was associated with a 52% relatively lower risk of VTE (SHR=0.48, 95% CI: 0.24-0.97; P=.032). In this retrospective cohort study, we showed that TGCT patients undergoing cisplatinum-based chemotherapy have a lower VTE risk when receiving primary thromboprophylaxis. For the duration of chemotherapy, primary thromboprophylaxis should be considered on a risk-benefit ratio.

Locally advanced carcinosarcoma of the uterine cervix – a case report

Carcinosarcoma, also known as malignant mixed Mullerian tumour, is a rare but aggressive biphasic tumour comprising both epithelial and mesenchymal components.1 A number of theories have been proposed for its pathogenesis, of which the conversion and combination theories have been the most widely accepted.1 Carcinosarcoma constitutes 3% of uterine malignancies, mostly arising from the uterine corpus.1 Carcinosarcoma of the uterine cervix represents only 0.005% of cervical malignancies, and there is a limited understanding of its treatment, prognosis and outcome.2 We describe a case of FIGO stage IVA carcinosarcoma of the uterine cervix in a 48-year-old woman who presented with abdominal pain and heavy menstrual bleeding. Clinical examination and imaging studies showed a cervical mass involving the lower third of the uterus and upper half of the vagina, with infiltration of the posterior bladder wall. Bilateral pelvic and lower retroperitoneal lymphadenopathy was present. Cervical biopsy showed a malignant tumour consisting of homologous sarcomatous areas and nests of moderately to poorly differentiated squamous cell carcinoma. Immunohistochemistry confirmed a diagnosis of carcinosarcoma of the uterine cervix. The patient was referred for urgent palliative radiotherapy and died of disease within three months.

THE UNFORTUNATE PERFECT STORM - SEPTIC SHOCK DUE TO CHRONIC IMMUNOSUPPRESIVE MEDICAL THERAPY AND MEDICATION-INDUCED LYMPHOMA

Abstract INTRODUCTION Pharmacological treatment of Crohn's Disease (CD) involves immunomodulators such as adalimumab, a tumor necrosis factor (TNF) inhibitor and azathioprine, a purine analogue. These medications have risks associated with severe illness from infectious etiologies as well as lymphoma development. This is due to the immunosuppressive effect of the medications as well as a chronic inflammatory state(1). Anaplastic large-cell lymphoma (ALCL) is a subtype of non-Hodgkin's lymphoma (NHL). We present a rare case of sepsis and ALCL in a patient on immunomodulator therapy. CASE DESCRIPTION A 31-year-old G1P0 female at 19 weeks gestation with history of Crohn’s disease presents with abdominal pain. Her medications are adalimumab and azathioprine which were started one year ago due to poorly controlled CD resulting in anal fistulas. She was recently treated for right thigh folliculitis. This progressed to abdominal pain and right inguinal lymphadenopathy. She met SIRS criteria on presentation and was admitted to the intensive care unit for respiratory failure requiring intubation. Despite broad-spectrum antibiotics for pneumonia, she remained febrile with an increasing leukocytosis. Blood, lower respiratory, and fungal cultures, acid-fast staining, viral cultures and smears, and urine tests were negative for a source of infection. Commuted tomography (CT) of the abdomen and pelvis was significant for extensive retroperitoneal lymphadenopathy but found no infectious source. An excisional biopsy of the right groin lymph node was a preliminary read as Hodgkin's lymphoma (HL), but the initial immunohistochemical stain was consistent with NHL. Her immunomodulator therapy was stopped due to sepsis and her risk of lymphoma. She subsequently developed septic shock, acute renal failure requiring continuous renal replacement therapy, atrial fibrillation with rapid ventricular response, and suffered a spontaneous vaginal delivery of a 20-week stillborn baby. A final diagnosis of ALCL was confirmed by pathology. The molecular studies were significant for positivity for CD45, CD30, EMA, CD2, CD4, CD25, ALK1, granzyme B, perforin, and TIA1. She was started on brentuximab, cyclophosphamide, and prednisone. DISCUSSION ALCL is a rare subtype of NHL that accounts for roughly one percent of all NHL (3). The development of lymphomas occurs when the TNF alpha subunit binds to the cell surface. This inhibits both the apoptosis pathway and cytokine-mediated inhibition of CD30+ lymphoma cells resulting in cell proliferation (4). There is limited documentation of adalimumab or azathioprine associated ALCL and this association should be studied further. The patient suffered from severe consequences of her immunosuppression from her chronic immunomodulator therapy and immunosuppression from her malignancy resulting in septic shock with multiorgan failure. Right inguinal lymph node biopsy showing atypical large lymphoid infiltrate, background small lymphocytes, plasma cells, and histiocytes. The large atypical cells are characterized by large irregular nuclei with occasional bilobed nuclei and horseshoe-shaped nuclei, multiple small nucleoli, and abundant amphophilic cytoplasm.

Diagnostic Challenges in a Patient with Metastatic Merkel Cell Carcinoma and Abdominal Pain

We examined a 51-year-old white male patient diagnosed with metastatic Merkel cell tumor, treated with immunotherapy, who came to the emergency room with upper abdominal pain and elevated pancreatic serum enzymes. He was suspected to have acute pancreatitis and was referred for computed tomography examination, which didn’t depict any sign of acute pancreatitis, showing a solid mass adjacent to the pancreatic head, retroperitoneal lymphadenopathies, solid nodules in the subcutaneous fat, and retroperitoneum.

Acute Kidney Injury Secondary to Abdominal Tuberculosis: A Diagnostic Dilemma - A Case Report

Tuberculosis is a serious public health issue in developing countries. Hypercalcemia, though reported in granulomatous disease, is mild and asymptomatic in tuberculosis. A 16 year old girl female presented with significant weight loss, loss of appetite and on examination hepatosplenomegaly. Ultrasound abdomen (USG) showed mesenteric and retroperitoneal lymphadenopathy with hepatosplenomegaly with mild ascites. We report an unusual case of hypercalcemia with renal injury due to abdominal tuberculosis in an immunocompetent female.

S46 Persistent Risks of TNF Inhibitors Despite Benefits: 31 Years Old Pregnant Patient With Crohn’s Disease

Introduction: TNF inhibitors have been used to treat inflammatory bowel diseases for the past 2 decades. These medications act to control acute flares as well as reach and maintain remission. However, side effects remain a large concern. Physicians should maintain a low threshold of suspicion for both opportunistic infection and malignancy, particularly lymphoma and leukemia, in their patients taking TNF inhibitors. Case: A 31-year-old 19 weeks pregnant female (G1P0, GA 19w0d) with Crohn’s disease presented with one month of right lower quadrant pain, fever, chills and fatigue. She had an incision and drainage performed for right thigh folliculitis 6 weeks before, and since then had continued pain in the right inguinal and lower quadrant area. She has been on adalimumab and azathioprine since 2015 with no recent flare ups; her last dose of adalimumab was given just before this admission. On admission, patient was febrile, tachypneic, and tachycardic, with leukocytosis. Given concern for sepsis in the setting of pregnancy, she was admitted to ICU and started on vancomycin and ampicillin-sulbactam for broad coverage. Imaging revealed extensive retroperitoneal lymphadenopathy along the right iliac chain and right inguinal region. She underwent an excisional biopsy from right inguinal lymph nodes. While final pathology results were pending, respiratory symptoms worsened, leading to an increased oxygen requirement. Chest CT showed progressive patchy ground glass opacities. Azithromycin and steroids were started, with pentamidine to cover possible Pneumocystis carinii pneumonia (PCP). On hospital day 15, the patient had a spontaneous vaginal delivery of a nonviable fetus. She experienced multi-system organ failure in following days, requiring vasopressor support, intubation, mechanical ventilation, and continuous renal replacement therapy due to severe metabolic acidosis. Antibiotics were broadened to vancomycin, meropenem, clindamycin, micafungin, azithromycin and pentamidine without meaningful clinical improvement. Multiple cultures, including a bronchoalveolar lavage, showed no pathogen. Final pathology revealed ALK-positive anaplastic large-cell lymphoma, a type of non-Hodgkin lymphoma, and the patient was started on chemotherapy with cyclophosphamide and doxorubicin. She had drastic clinical improvement following the first dose. She was then weaned off vasopressors and extubated. Antibiotics were discontinued, except atovaquone for PCP prophylaxis, and the patient was discharged on hospital day 26. Discussion: It is hypothesized that T cell dysfunction is involved in the pathogenesis of Crohn’s disease and studies have reported that patients with chronic inflammatory disorders might have an increased baseline risk of lymphomas regardless of TNF antagonist treatments. As physicians, we maintain high suspicion for opportunistic infections in IBD patients on TNF inhibitors presenting with signs of a systemic inflammatory response. However, clinical features associated with sepsis– such as fever, tachycardia, tachypnea, and leukocytosis– can indicate malignancy as well. Some lymphomas cause inflammatory responses that respond to chemotherapy. This case emphasizes the importance of avoiding singular focus on opportunistic infection in IBD patients.

Posterior Reversible Encephalopathy Syndrome: A Rare Side Effect of Nivolumab

Introduction: Posterior reversible encephalopathy syndrome (PRES) is a novel clinico-radiological syndrome which is characterized by variable symptoms including visual disturbances, headache, vomiting, seizures and altered consciousness, usually accompanied by hypertension. Several cases of immunotherapy-related PRES have been reported as a rare cause of PRES. We present a case of a 52-year-old male who was started on the immunotherapy nivolumab for his esophageal cancer. He came to the hospital with progressive vision loss and was diagnosed with PRES along with optic neuritis. Case Presentation: A 52-year-old male with no significant past medical history presented to the hospital with left sided numbness and spatial recognition issues. A computed tomography (CT) of the head revealed a 3 cm right parietal mass with vasogenic edema later confirmed with a magnetic resonance imaging (MRI) of the brain. He was started on steroids along with 500 mg Keppra twice a day. CT of the chest, abdomen, and pelvis showed multiple hepatic masses and retroperitoneal lymphadenopathy. He had a colonoscopy about a year prior that was unremarkable. He underwent right frontal parietal craniotomy for single brain metastatic lesion. The biopsy of both the brain and liver mass revealed metastatic adenocarcinoma. He was discharged and started on Fluorouracil, Folinic Acid, Oxaliplatin (FOLFOX) chemotherapy as outpatient for possible upper gastrointestinal (GI) cancer. After two cycles of chemotherapy, a positron emission tomography (PET) scan revealed suspected primary esophageal cancer. Though patient had zero PDL-1 biomarker, nivolumab was added to cycle 3 of FOLFOX with the expectation of having a better response early in the disease. He continued to have stereotactic radiosurgery (SRS) along with steroid taper for local control of brain metastasis. Nivolumab was held after cycle 2 as he developed mild headache, fever and elevated lipases. He was found to be hypertensive to 144/85 mmHg. MRI brain showed improving post-surgical changes. On improvement of symptoms, Nivolumab was reintroduced to cycle 6 of FOLFOX. Unfortunately, a week later he developed progressive vision loss more on right eye. He was sent to optometrist who referred patient to emergency room (ER) because of concern for bilateral papilledema. On admission to ER, he was hypertensive to 182/104 and tachycardic to 107 beats per minute. Physical exam was significant for left inferior quadrantanopia more prominent in right eye. CT head was unrevealing for any infarct. MRI of the orbit and brain showed increased patchy T 2 signal in bilateral parieto-occipital lobes without enhancement suggestive of posterior reversible encephalopathy syndrome (PRES) along with right optic neuritis. These findings were attributed to the immunotherapy nivolumab. He was started on a high dose of prednisone, 1mg/kg/day along with 20 mg lisinopril for strict blood pressure control with a goal of 120/80 mmHg. He was discharged with tapering steroids dose and his symptoms improved within 1 week of treatment. Discussion: Posterior reversible encephalopathy syndrome is a clinico-radiological diagnosis. The underlying pathophysiology involves hypertension and endothelial dysfunction. Hypertension leads to cerebral hyper-perfusion which may cause vascular damage and vasogenic edema leading to blood brain barrier dysfunction. The posterior circulation is the most susceptible to blood pressure changes, thus it is the most common imaging abnormality. PRES frequently develops in the context of cytotoxic medication, immunotherapy, (pre)eclampsia, sepsis, renal disease or autoimmune disorders. MRI is an important diagnostic tool and displays hyperintense lesions in T2-weighted or FLAIR sequences in the parietal-occipital lobes in about 70% of cases but other parts can also be involved. Electroencephalogram (EEG) and lumbar puncture (LP) can be done to rule out other causes. Differentiation is important from an ischemic event as management is different in both cases. Treatment of PRES is symptomatic. Recommendations are to avoid fluctuations in blood pressure with a goal of 130-150/80-100mmHg. If any triggering factor or condition is identified like immunotherapy, it should be avoided or treated accordingly. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal

NCMP-16. UNIQUE PARANEOPLASTIC SYNDROME ASSOCIATED WITH CLL

Abstract Chronic lymphocytic leukemia is a hematologic malignancy characterized by overproduction of monoclonal B cells. Most people diagnosed with CLL do not immediately undergo treatment due to treatment guidelines. There are few cases of paraneoplastic syndrome associated CLL. This case is unique in that it is a paraneoplastic KLHL 11 antibody associated CLL. The patient is a 68-year-old female who originally presented to the hospital with abdominal pain and upon further imaging was found have retroperitoneal lymphadenopathy. After further studies including flow cytometry, she was diagnosed with CLL. She failed trials of Pembrolizumab, ibrutinib, and Obinutuzumab due to side effects. The patient then started experiencing excessive fatigue, bilateral temporal headache, nausea, vomiting, vertigo, tremor of bilateral upper extremities, and head bobbing. Her brain imaging was negative for acute abnormalities. Lumbar puncture was positive for CLL cells and the paraneoplastic panel came back positive with KLHL 11 antibodies. The patient’s symptoms continued to persist and her tremor and dysmetria continued to worsen. Due to the positive paraneoplastic panel, the patient was started on intravenous immunoglobulin but did not experience improvement. She was then trialed on intravenous methylprednisolone and still experienced no relief. She then underwent plasma exchange. Finally, she was given rituximab infusions with slight improvement. Her imaging remained negative for signs of leptomeningeal enhancement. Given the patient’s symptoms, negative brain imaging, and positive paraneoplastic panel, it was determined that the patient developed an irreversible paraneoplastic syndrome related to her CLL versus possible leptomeningeal disease. There are a few cases of paraneoplastic syndrome associated CLL involving paraneoplastic phemphigus, hypercalcemia, and nephrotic syndrome. This case is unique in that her symptoms presented with an ataxic syndrome with positive KLHL11 antibodies. It is important to be aware that some cases of CLL can present with unique paraneoplastic syndromes.

Metastasis of a Primary Sinonasal Alveolar Rhabdomyosarcoma to the Testicle in an Adult Patient

Abstract Introduction/Objective Alveolar rhabdomyosarcoma is an aggressive tumor of skeletal muscle differentiation that predominantly affects adolescents and young adults, and is rare over 45 years of age. It has poor prognosis with metastasis, and most commonly spreads to the lymph nodes, bone marrow, and lungs. In this report, we describe a middle-aged patient with a primary sinonasal alveolar rhabdomyosarcoma which metastasized to the right testicle, an unusual metastatic site reported only a few times in literature. Methods/Case Report Our patient is a 53 year old male who presented in 2020 with recurrent epistaxis and bilateral cervical lymphadenopathy over a 2 month period. Maxillofacial CT/MRI imaging revealed a 4-cm sized mass within the left nasal cavity eroding into the ethmoid sinuses. The patient underwent endoscopic sinus surgery of the sinonasal mass, which on histology, appeared as poorly differentiated small round blue cells with numerous mitoses infiltrating as single cells and nests. These cells stained positive for vimentin, myogenin, desmin, and CD56, and negative for AE1/AE3, chromogranin, S-100, CD99, and CD45. The cells showed very high Ki67 proliferation index (70-80%) and tested positive for FOXO1 gene rearrangement, which supported the diagnosis of alveolar rhabdomyosarcoma. The patient completed chemoradiation therapy, and MRI scans over the next 2 years were negative for residual sinonasal tumor. However, the patient returned in 2022 after having undergone a right orchiectomy at an outside hospital for testicular pain, and abdominal CT showed diffuse retroperitoneal lymphadenopathy concerning for metastasis. On histology, the resected testicle showed seminiferous tubules infiltrated and destroyed by sheets of poorly differentiated small round blue cells with fibrous septae separating the nests of tumor cells into alveolar-like spaces with central loss of cohesion. The tumor cells showed the same immunohistochemical staining pattern as the primary sinonasal lesion, thus confirming it to be metastatic alveolar rhabdomyosarcoma. Results (if a Case Study enter NA) NA. Conclusion It is unusual for alveolar rhabdomyosarcomas to occur in adults over 45 years old, and is even rarer for these tumors to metastasize to the testicle. Metastases also only account for 1% of testicular tumors. Our report documents a very rare case of an alveolar rhabdomyosarcoma in a middle-aged adult which metastasized to the testicle after chemoradiation therapy.

PSAT014 Pheochromocytoma in a Pregnant Woman with VHL leading to First Trimester Fetal Demise

Abstract Introduction Pheochromocytomas (PCCs) are rare neuroendocrine tumors that secrete catecholamines. They may be associated with familial syndromes such as Multiple Endocrine Neoplasia type 2 (MEN 2), von Hippel-Lindau disease (VHL), and Neurofibromatosis type 1 (NF 1). PCCs are a common cause of malignant hypertension in pregnancy and can lead to adverse maternal and fetal outcomes if left undiagnosed or untreated. Case Description A 39-year-old Latin American primigravid woman at 8 weeks 4 days gestation was referred to the endocrinology clinic for labile blood pressure (160/90 mmHg at recent ER visit) and a lab finding of elevated catecholamines (plasma norepinephrine 4720 pg/ml; normal 0-874 pg/ml). She reported chronic headaches and 2-lb unintentional weight loss during pregnancy. Past medical history was significant for right adrenal PCC diagnosed at age 13 (currently status post adrenalectomy), and a cerebrovascular accident at age 29. Family history was relevant for PCC in the patient's sister and mother. The patient's home medications were nifedipine 30 mg daily and prenatal vitamins. On physical exam, her blood pressure was 126/76 mm Hg, heart rate was 67/min and a scar of previous right adrenalectomy was noted. Doxazosin 1 mg daily was added to her regimen and nifedipine was discontinued. The patient was initially planned for an elective cesarean section at term. However, at 10 weeks 5 days of gestation, she had a missed abortion. Further genetic testing of syndromic PCC revealed a heterozygous mutation in the VHL gene. Abdominal MRI showed a left adrenal mass (3.6×2.6×3.6 cm3) and retroperitoneal lymphadenopathy. The patient underwent left adrenalectomy and is currently hemodynamically stable on maintenance hydrocortisone and fludrocortisone. Discussion We report a case of PCC complicating pregnancy in a 39-year-old primigravida who had a family history of PCC. In females of reproductive age with a known PCC diagnosis, we suggest early genetic testing as it can further advise family planning, antepartum monitoring, and management. Medically, these patients can be managed with α-adrenergic blockers and calcium-channel blockers, followed by β-blockers. This can improve maternal and fetal outcomes primarily by minimizing paroxysmal elevations in catecholamines. Definitive treatment of PCC is surgical excision, usually performed during the second trimester or postpartum. Management of patients with VHL requires lifelong clinical, laboratory, and imaging surveillance for other manifestations of the disease. References Bancos, Irina et al. Maternal and fetal outcomes in phaeochromocytoma and pregnancy: a multicentre retrospective cohort study and systematic review of literature. Lancet Diabetes Endocrinol 2021; 9: 13–21 Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

PSUN260 Diabetic Myconecrosis: a Rare Complication of Uncontrolled Diabetes Mellitus

Abstract Introduction Spontaneous diabetic myonecrosis is a rare and underdiagnosed microvascular complication of long standing and poorly controlled diabetes mellitus. The pathogenesis of this infarction of the skeletal muscle appears to be related to vasculopathic changes without an atheroembolic event or occlusion of major arteries. The factors responsible for the acute onset of symptoms are unknown. Since the initial description in 1965, only 170 cases have been described, including 126 initial episodes and 44 episodes of recurrence, with a mean age at presentation of 45 years (range 20 to 67 years). Most of the patients have other microvascular complications. Characteristic symptoms at presentation include acute onset unilateral lower limb pain and swelling, most often in the thigh, without a history of trauma. Bilateral involvement is present in approximately 30% of the cases. Fever has been reported only in about 10% of cases. Case Report A 23-year-old female presented with ten days history of fever, left groin pain and inability to walk. Other than chronic fatigue, her review of symptoms was mostly negative. She denied any trauma. She had a history of poorly controlled type 1 diabetes mellitus (HbA1c 10.5%) with diabetic nephropathy (proteinuria),but no evidence of retinopathy or neuropathy. On examination, her left groin and proximal thigh were warm and tender to palpation without noticeable erythema or swelling. Labs showed elevated inflammatory markers. CT scan showed inguinal and retroperitoneal lymphadenopathy with subcutaneous edema of the visualized left lower extremity. Fevers persisted despite appropriate administration of antibiotics for cellulitis. Infectious workup was negative. A biopsy of her inguinal lymph node showed reactive changes. MRI of her left thigh showed large, confluent areas of intramuscular hypo enhancement compatible with diabetic myonecrosis. Her symptoms improved over the next 3-4 weeks with supportive care. Discussion Spontaneous diabetic myonecrosis is a very rare complication of uncontrolled diabetes. Knowledge about his condition is required to make the correct diagnosis and avoid the use of unnecessary laboratory tests and treatments. The presence of the characteristic clinical features such as pain and swelling in typically affected areas such as the thigh, will frequently suggest the diagnosis. MRI with intravenous contrast enhancement is the diagnostic imaging of choice and shows increased signal on T2-weighted images from muscle edema and areas of non-enhancement from myonecrosis. Treatment involves symptomatic management with rest, optimal glycemic control, analgesia, and low-dose aspirin, although the optimal treatment approach is uncertain. The short-term prognosis is good, but the recurrence rate remains high and long-term prognosis is poor given underlying poorly controlled diabetes. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.

PSAT387 An Unusual Case of Sporadic Metastatic Medullary Thyroid Cancer Without a Primary Tumor Presenting with Paraneoplastic Cushing's Syndrome

Abstract Introduction Medullary thyroid carcinoma (MTC) accounts for 5-10% of all thyroid cancers and up to 30% of MTCs are hereditary. Only 0.6% of MTCs are associated with a paraneoplastic ACTH-dependent Cushing's syndrome (CS) (PMID: 16029131). Even rarer are MTCs without a primary intra-thyroidal tumor. Here we describe a patient with metastatic MTC without a known primary intrathyroidal tumor presenting with paraneoplastic ACTH-dependent CS. Clinical Case A 47-year-old Ukrainian man was referred to our center for management of metastatic MTC. During evaluation of refractory diarrhea two years earlier, an elevated serum calcitonin (1758 pg/mL, normal: &amp;lt;11.5) led to the diagnosis. Thyroid ultrasound revealed a normal thyroid with bulky cervical lymphadenopathy. Cervical lymph node biopsy revealed metastatic MTC with positive calcitonin, synaptophysin, and chromogranin-A on immunohistochemistry (IHC) and RET protooncogene M918T pathogenic variant on molecular testing. Germline testing for RET pathogenic variants was negative. Prior to referral, the patient was on octreotide to control diarrhea and vandetanib for a few months, but they were discontinued due to financial stress. At our center, patient complained of 4-6 watery bowel movements daily. Physical examination revealed facial plethora, dorsocervical fat pad, central adiposity, wide purple striae, and pustular acne on the chest wall and proximal muscle weakness. Biochemical testing revealed elevated serum calcitonin (158,535 pg/mL, normal: &amp;lt;14.3), carcinoembryonic antigen (3,634 ng/mL, normal: 0.8–6.2), and chromogranin-A (5504 ng/mL, normal&amp;lt;93) levels. Morning serum cortisol (40.4 mcg/dL, normal: 3.7–19.4), and plasma ACTH (116.0 pg/mL, normal: 5.0-46.0) levels were elevated, along with markedly elevated 24-hour urine free cortisol levels (12,460.8 mcg/24-hour, normal: 35-45.0), consistent with ACTH-dependent Cushing's syndrome. Further IHC testing of the tumor biopsy sample revealed mild ACTH positivity. A pituitary MRI was normal. Stool osmolality studies showed a secretory diarrhea pattern. Computed tomography (CT) of neck and torso demonstrated bulky cervical, retroperitoneal, and inguinal lymphadenopathy, and extensive liver metastasis, all of which were strongly avid on an 18-fluorodeoxyglucose positron emission tomography CT scan, but poorly avid on a gallium-68 DOTATATE scan. Sodium fluoride bone scan revealed multiple osseous metastases in the spine, ribs, pelvis, and in the right femoral shaft. An adrenal CT scan revealed bilateral adrenal hyperplasia. The patient was unfortunately not eligible for any ongoing local clinical trials on RET-mutated cancer therapies. After extensive discussion with the patient, a palliative approach was pursued. The cervical lymphadenopathy and right femoral shaft metastasis were treated with external beam radiation. Diarrhea was controlled with loperamide. Cushing's syndrome was initially managed with metyrapone and ketoconazole, followed by bilateral adrenalectomy and hydrocortisone and fludrocortisone replacement. Conclusion Sporadic metastatic MTC can rarely present without an intrathyroidal primary tumor and may originate from ectopic C-cells. Paraneoplastic syndromes such as Cushing's syndrome can be observed in MTC due to ectopic-ACTH production. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

ODP094 Hypercalcemia caused by Mycobacterium Avium Complex, with Excellent Response to Intravenous Pamidronate.

Abstract Background Hypercalcemia caused by granulomatous diseases such as sarcoidosis and tuberculosis, is a well-recognized entity. The pathophysiology of hypercalcemia associated with granulomatous diseases is related to increase conversion of 25 hydroxyvitamin D to its active form, 1,25 dihydroxyvitamin D, via extra-renal 1 -α hydroxylase activity. We present a patient with moderately sever hypercalcemia caused byMycobacterium Avium complex (MAC) infection in association with Human Immunodeficiency Virus (HIV) infection. Case Presentation The patient is 53-year-old male with longstanding HIV infection since 2003. He was referred to the endocrine clinic after an acute presentation to a local hospital in August 2021 with pancytopenia, lymphadenopathy, and hypercalcemia (corrected total calcium 13.8 mg/dl) with acute kidney injury. He was treated with intravenous fluids and subcutaneous Calcitonin injection. Endocrine work up showed low parathyroid hormone (PTH) at 3 pg/ml (14-95 pg/ml) and Parathyroid related peptide (PTHrP) level was &amp;lt;0.4 pmol/l. His 1,25 dihydroxy-vitamin D was elevated at 172 pg/ml (18-64 pg/ml) . Computed Tomography (CT) study for neck, chest and abdomen showed hepatosplenomegaly, generalized lymphadenopathy involving the left cervical area, left supraclavicular area, mediastinal, retroperitoneal, mesenteric and inguinal lymphadenopathy. Bone marrow study from August 2021 showed no evidence of leukemia, lymphoma, tuberculosis or other bone marrow inflammatory or infiltrative processes. Left sided neck lymph node biopsy showed diffuse granulomatous disease and numerous acid-fast organism consistent with Mycobacterium Avium complex (MAC) He was treated with azithromycin 250 mg daily, ethambutol 1200 mg daily, and rifabutin 300 mg daily in addition to anti-retroviral therapy (bictegravir/emtricitabine/tenofovir). Hypercalcemia was treated with one dose of pamidronate 90 mg which normalized serum calcium within one week. Glucocorticoids was not considered due to concerns over sever immunosuppression due to HIV/ AIDS and pancytopenia. Denosumab, ketoconazole and hydroxychloroquine were also not considered due to concerns over side effects. His 1,25 dihydroxy-vitamin D level normalized after four months from starting treatment with anti MAC therapy. Most recent calcium level 9.1 mg/dl, with 1,25 dihydroxy-vitamin D level of 31 pg/ml. Conclusion Mycobacterium Avium Complex infection is a possible cause of hypercalcemia, especially in immunocompromised individuals. Glucocorticoids, usually the first line treatment for acute hypercalcemia induced by 1,25 dihydroxyvitamin D overproduction, might not be appropriate in all patients. Alternatives include hydroxychloroquine, ketoconazole and parenteral bone anti- resorptive agents. Our patient had an excellent response to one dose of pamidronate. Ultimately his1,25 dihydroxy-vitamin D normalized with effective anti MAC therapy. Presentation: No date and time listed