Enlarged Retroperitoneal Lymph Nodes Research Articles

Primary Renal Well-Differentiated Neuroendocrine Tumor (Carcinoid) in a Horseshoe Kidney

A female patient was found to have a horseshoe kidney on imaging studies when undergoing evaluation for recurrent urinary tract infections when she was in her 20s. Now in her late 40s, she presented with gross hematuria. A computed tomography urogram revealed the presence of a 14.9-cm mass arising from the isthmus of the horseshoe kidney (Figure 1). Also present was an enlarged retroperitoneal lymph node suspicious for metastatic disease.

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A 26-year-old male presented to general medicine department on 16th March 2020 with complaints of 3 episodes of epistaxis for 2 days. The epistaxis was spontaneous, sudden in onset, recurring in nature, copious in amount, not relieved on its own. No history of nasal trauma, head injury, fever, joint pain, cough, breathlessness, difficulty in swallowing, pain abdomen, haematuria, rashes. No history of bleeding diathesis from any other site. Past history revealed similar complaints of nasal bleeding for past 2 - 3 months for which he used to get admitted and administered platelets transfusions after which the symptoms used to subside. No history of any bleeding disorders or other comorbidities. Family history revealed that the younger brother is a known case of Bernard-Soulier syndrome diagnosed at the age of 15 years. Drug history was insignificant. Based on patient’s presentation and family history, a congenital bleeding disorder was considered as provisional diagnosis. On clinical examination pallor was observed in lower palpebral conjunctiva and dorsum of tongue and crusts were present in nose. Moderate splenomegaly was also observed. His laboratory data revealed presence of microcytic hypo-chromic anaemia with hyponatremia, with prolonged bleeding time, normal clotting time and coagulation time. Complete blood count revealed severe microcytic hypochromic anaemia (haemoglobin - 5.8 gram / decilitre) (Figure 1) and giant platelets (Figure 2). Bleeding time was 12 minutes and clotting time was 8 minutes. Factor VIII levels were normal in the patient. Prothrombin time was prolonged with 13.9 seconds. Liver function test revealed only hypoalbuminemia with no derangement of liver enzymes. Renal function tests were normal. Serum electrolytes revealed hyponatremia with 130 millimole / litre. Ultrasound abdomen and pelvis revealed a heterogeneous focus which was noted in spleen with peripheral colour uptake and necrotic areas within it suggesting of splenic abscess / splenic haematoma / infarct. Contrast enhanced computed tomography of abdomen and pelvis revealed mild splenomegaly with heterogeneous foci with peripheral colour uptake and necrotic areas within it measuring 6.4 * 4.8 centimeters, areas were hypo dense and few enlarged retroperitoneal lymph nodes noted in para-aortic region. Computed tomography of brain plain was normal. Contrast enhanced computed tomography of thorax was normal. Platelet function tests revealed normal aggregation with adenosine diphosphate, collagen and arachidonic acid and markedly reduced with ristocetin. Flow cytometry could not be assessed. His clinical examination revealed findings suggestive of anaemia and splenomegaly was also present with vital parameters within normal limits. He underwent thorough work-up with working diagnosis of bleeding disorder under evaluation.

A Prediction Model for Optimal Primary Debulking Surgery Based on Preoperative Computed Tomography Scans and Clinical Factors in Patients With Advanced Ovarian Cancer: A Multicenter Retrospective Cohort Study.

ObjectiveThis study assessed the predictive value of preoperative computed tomography (CT) scans and clinical factors for optimal debulking surgery (ODS) in patients with advanced ovarian cancer (AOC).MethodsPatients with AOC in International Federation of Gynecology and Obstetrics (FIGO) stage III-IV who underwent primary debulking surgery (PDS) between 2016 and 2019 from nine tertiary Chinese hospitals were included. Large-volume ascites, diffuse peritoneal thickening, omental cake, retroperitoneal lymph node enlargement (RLNE) below and above the inferior mesenteric artery (IMA), and suspected pelvic bowel, abdominal bowel, liver surface, liver parenchyma and portal, spleen, diaphragm and pleural lesions were evaluated on CT. Preoperative factors included age, platelet count, and albumin and CA125 levels.ResultsOverall, 296 patients were included, and 250 (84.5%) underwent ODS. The prediction model included age >60 years (P=0.016; prediction index value, PIV=1), a CA125 level >800 U/ml (P=0.033, PIV=1), abdominal bowel metastasis (P=0.034, PIV=1), spleen metastasis (P<0.001, PIV=2), diaphragmatic metastasis (P=0.014, PIV=2), and an RLNE above the IMA (P<0.001, PIV=2). This model had superior discrimination (AUC=0.788>0.750), and the Hosmer-Lemeshow test indicated its stable calibration (P=0.600>0.050). With the aim of maximizing the accuracy of prediction and minimizing the rate of inappropriate explorations, a total PIV ≥5 achieved the highest accuracy of 85.47% and identified patients who underwent suboptimal PDS with a specificity of 100%.ConclusionsWe developed a prediction model based on two preoperative clinical factors and four radiological criteria to predict unsatisfactory debulking surgery in patients with AOC. The accuracy of this prediction model needs to be validated and adjusted in further multicenter prospective studies.

The timing of initial imaging in testicular cancer: impact on radiological findings and clinical decision making

Background In testicular cancer determination of clinical stage and recommendation of therapeutic strategy after inguinal orchiectomy are based on primary imaging by CT-scan of the chest and CT- or MRI- abdomen. It has not been investigated so far, whether the imaging should be performed before or after primary testicular surgery. Staging before surgery means exposing all patients to CT radiation irrespective of ensured histologic malignancy while postoperative staging could pose a risk in biased clinical decision making by increased presence of unspecific lymph node enlargement caused by postsurgical effects. Therefore, we aimed to investigate the association between the timing of initial staging and occurrence of unspecific lymph node enlargement and adjuvant therapies after inguinal orchiectomy. Methods We retrospectively evaluated clinical and radiological data from 236 patients who had undergone inguinal orchiectomy for testicular cancer at our department. Statistical analysis was performed to determine whether the occurrence of unspecific lymph node enlargement or the rate of adjuvant therapies were influenced by timing of initial staging (preoperative vs. postoperative). Results The postoperative imaging cohort showed significant more inguinal, pelvic and retroperitoneal unspecific lymph node enlargement than the preoperative imaging cohort. Simultaneous occurrence of inguinal or pelvic lymph node enlargement together with retroperitoneal enlargements could only be found in the postoperative imaging cohort. No difference regarding adjuvant therapies could be found. Conclusions Timing of imaging affects the detection rate of unspecific lymph node enlargements but does not show a significant effect on the rate of adjuvant therapies.

S2462 A Patient Presenting With Acute Hepatitis and Retroperitoneal Lymphadenopathy Due to Cytomegalovirus and Epstein-Barr Virus Co-Infection: A Rare Complication in an Immunocompetent Patient

INTRODUCTION: Acute hepatitis refers to an acute inflammation of a liver and hepatic cell necrosis. Viral infection accounts for 95% of cases. Acute viral hepatitis due to cytomegalovirus (CMV) and Epstein-Barr virus (EBV) co-infection in an immunocompetent individual is very rare. We report a patient who presented with fatigue, fever, and retroperitoneal lymphadenopathy due to EBV and CMV infection. CASE DESCRIPTION/METHODS: A 26-year-old man presented with intermittent high-grade fever, nausea, fatigue and generalized body weakness of 1-week duration. The patient noticed a pinkish rash on his abdomen and legs while he was febrile. Otherwise patient denied vomiting, hematemesis, melena, jaundice, or contact with a patient with similar illness. He was not immunocompromised and had no personal history of hematological disorders or autoimmune disease. On examination, the patient was sick looking; blood pressure was 120/68 mmHg, heart rate 110 beats/min, temperature 102 F. He had pink conjunctiva and non-icteric sclera. No palpable lymphadenopathy was felt in accessible areas. The abdomen was nontender, no organomegaly, and no sign of ascites. Laboratory tests revealed a white blood cell count 7.6K, hemoglobin 13.8gm/dl, platelet 163K. Peripheral blood smear revealed lymphocytosis (59%). Alanine transferase 279units/L, aspartate transaminases 217units/L, alkaline phosphatase 121units/L, and total bilirubin of 0.4 mg/dl. Both CMV IgM and IgG were positive, both EBV IgM and IgG were positive. Hepatitis viral panel A, B, C, human immunodeficiency virus, ANA and rheumatoid factor were all negative. Computed tomography of chest/abdomen/pelvis revealed multiple enlarged axillary and retroperitoneal lymph nodes, normal liver and spleen size. DISCUSSION: CMV and EBV are a family of herpes virus commonly encountered during childhood. CMV transmission is either congenital or contact with body secretions or blood transfusion. EBV is transmitted by close contact during child hood or by kissing in adults. Hepatitis as a manifestation of CMV/EBV is rare in an immune-competent patient, but both can present with infectious mononucleosis. Although acute hepatitis can happen in both cases, it is more likely due to CMV. This patient did not have typical symptoms of infectious mononucleosis on presentation, and lymphoma and other causes were considered. A high degree of suspicion helped us to do serology that confirmed the diagnosis. Treatment is supportive in an immune-competent patient.

S1380 A Rare Case of Pancreatic Plasma Cell Myeloma Presenting as Obstructive Jaundice: A Case Report and Review of Literature

INTRODUCTION: Hematological malignancies affect the pancreas very rarely, with lymphomas being the most common. Pancreatic plasma cell myeloma (PCM) is exceptionally uncommon. We present a case of obstructive jaundice in a patient due to pancreatic PCM. CASE DESCRIPTION/METHODS: A 50-year old man with a history of hypertension, hyperlipidemia, and nephrolithiasis presented to the emergency department with a 3-month history of worsening mid-epigastric pain, weight loss, and jaundice. Initial laboratory workup showed low hemoglobin (11.0 g/dL), hyperbilirubinemia, and elevated protein gap. C-reactive protein and erythrocyte sedimentation rate were elevated, while vitamin B-12 and folate levels were found normal. Computed tomography revealed intra and extrahepatic biliary ductal dilatation and a hyperenhancing mass within the pancreas and porta hepatis with portal vein compression (Figure 1). Multiple enlarged retroperitoneal lymph nodes, splenomegaly, and lytic lesions in the right pubic bone were also noted. Prostate-specific antigen (PSA) and carcinoembryonic antigen (CEA) levels were normal, while CA-19-9 was found to be 191 (Normal: 0-37 U/mL). Endoscopic retrograde cholangiopancreatography (ERCP) and stenting were performed for a malignant appearing common bile duct stricture (Figure 2). Urine protein electrophoresis (UPEP) revealed a monoclonal M spike (4.7 g/dL) and gamma globulins with lambda light chains in the urine. Immunofixation confirmed the monoclonality and demonstrated IgG lambda protein. Quantitative testing showed a high level of IgG at 6.5 g/dL (Reference range: 0.7-1.6 g/dL) with decreased IgA and IgM. Biopsy of pancreatic mass and bone lesions revealed plasmacytoma with lambda light chain restriction. Bortezomib and dexamethasone were initiated with the resolution of the biliary obstruction (Figure 3). The presence of extramedullary plasmacytoma (EMP), lytic bone lesions, anemia, and monoclonal immunoglobulin production was suggestive of multiple myeloma. DISCUSSION: Pancreatic PCM typically presents with obstructive jaundice, but pancreatitis, gastrointestinal bleeding, and nausea can also occur. Pancreatic PCM offers a diagnostic challenge and should not be overlooked when evaluating hyperbilirubinemia due to obstructive jaundice if retroperitoneal lymphadenopathy and osteolytic lesions are present.Figure 1.: Arrow pointing towards pancreatic mass.Figure 2.: Arrow pointing towards common bile duct stricture.Figure 3.: Arrow pointing towards resolution of common bile duct stricture.

Abstract P3-01-09: Clonal heterogeneity and tumor evolution inform treatment resistance in a patient with HER2+ breast cancer

Abstract Resistance to therapy in metastatic breast cancer patients results from acquired mutations and selection of resistant clones. Understanding the mechanisms of resistance in the clinical setting is complicated by the lack of genomic characterization of tumor specimens. This case report describes a patient with ER+ and dynamic HER2+ status with exposure to a range of different therapies, suggesting that inference of tumor subclonal composition and evolution can be leveraged to study treatment resistance. A female patient was diagnosed with estrogen receptor positive (ER+) and human epidermal growth factor receptor positive (HER2+) stage I (T1N0) breast cancer. After receiving standard of care adjuvant chemotherapy (AC-TH) and Tamoxifen for two years, the patient developed metastatic disease to the liver. Upon metastatic disease, she received first line treatment with the trastuzumab emtansine antibody-drug conjugate (T-DM1). After fifteen months, enlargement of supraclavicular and retroperitoneal lymph nodes was observed, Letrozole was added to the treatment regimen until the patient progressed after eight months. Subsequent therapy with Fulvestrant and the CDK4/6 inhibitor Palbocicilb was administered for three months until the patient developed brain metastases. We analyzed biopsies taken at different time points of metastatic disease and, using whole exome sequencing (WES), reconstructed the subclonal architecture of each of the tumor specimens and inferred the evolutionary history of these clones. These results suggest potential mechanisms of resistance to the different therapies administered during disease progression. Biopsies obtained originated from a retroperitoneal lymph node at 3 years after diagnosis, a liver metastasis at 4 years after diagnosis and a brain metastasis after 4 years and 3 months after diagnosis. WES was performed on these tumor specimens and characterization of mutational events and copy number alterations was made using standard analytical pipelines. Inference of subclonal populations in the three tumor specimens analyzed revealed presence of a founding clone with mutations common to the three metastatic biopsies with alterations in TP53 p.K305* and PIK3CA p.E545K. A clone unique to the ER+/HER2- retroperitoneal lymph node metastasis shows no known driver mutations. In the liver metastasis (ER+/HER2-), we observed the presence of a dominant clone (cellular prevalence 93.5%) with mutation in ESR1 p.G442R and another subclone detected at low cellular prevalence (~5%) with an amplification in the ERBB2 gene. In the brain metastasis (ER+/HER2-), the latest tumor specimen obtained, we observed clonal selection of the ERBB2-amplified subpopulation (clonal prevalence 60%). The subclonal population dynamics and the different alterations in the clones present at different time points of tumor evolution provide a rationale to understand the therapeutic sensitivity and resistance observed in this patient. The ER+/HER2- retroperitoneal lymph node metastasis which appeared under T-DM1 treatment, suggests that this subclone is resistant to T-DM1, explained by the HER2 negative status, and was selected while the patient was on this regime. At the time of appearance of this clone, Letrozole was added to the treatment and a clone with an ESR1 p.G442R mutation appeared and dominated the tumor population, suggesting that this mutation provides resistance to the aromatase inhibitor. When treatment was switched to Fulvestrant and Palbociclib, a potentially dormant clone with ER+ and HER2+ features re-appeared in the tumor. Evolutionary analysis of this patient's tumor specimens underscores the importance of multiple therapies directed at all tumor vulnerabilities present, since targeting a single driver feature of the tumor will result in selection of other resistant subclones. Citation Format: Jorge E. Buendia-Buendia, Sonia Pernas, Ofir Cohen, Ping Ping Mao, Kailey Kowalski, Nancy Lin, Eric Winer, Nikhil Wagle. Clonal heterogeneity and tumor evolution inform treatment resistance in a patient with HER2+ breast cancer [abstract]. In: Proceedings of the 2019 San Antonio Breast Cancer Symposium; 2019 Dec 10-14; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2020;80(4 Suppl):Abstract nr P3-01-09.

2721 Back Pain Can Be Hard to Digest

INTRODUCTION: With over 1,000,000 new cases of gastric cancer in 2018, and 783,000 estimated deaths, gastric cancer remains an important cancer worldwide.1 Gastric malignancy will commonly present in patients with weight loss and persistent abdominal pain. Patients can also present with findings of metastatic disease. This clinical vignette is an example of a rare metastatic location of gastric cancer. CASE DESCRIPTION/METHODS: 55-year-old Middle Eastern male presents with intractable low back pain. An MRI of the thoracic and lumbar spine revealed multiple osseous lesions of the thoracic vertebral bodies and fractures of the L2 and L3 vertebral bodies, concerning for metastasis (Figure 1). A CT of the chest, abdomen and pelvis revealed enlarged mediastinal and hilar lymph nodes. Enlarged lymph nodes at the gastrohepatic interval and celiac level measuring up to 2 cm were noted, and multiple enlarged mesenteric and retroperitoneal lymph nodes. The patient underwent bone biopsy during kyphoplasty of the L2 and L3 vertebrae, which revealed metastatic carcinoma. Serum markers of malignancy were unrevealing, a CA19-9 of 15, CEA of 7.1, and PSA of 0.74. Urology was consulted and cystoscopy performed. No mass or filling defect was noted, and urine cytology returned negative for malignant cells. Gastroenterology was consulted and upon further history, the patient did acknowledge a history of H. Pylori, in 2016, with eradication confirmed by a negative stool antigen test. Gastroenterology recommended EGD and colonoscopy, but patient was unable to tolerate the bowel prep. The patient underwent an EGD, which revealed a 3.0 × 3.5 cm, cratered gastric ulcer without stigmata of bleeding at the incisura (Figure 2). Biopsy of this ulcer was positive for adenocarcinoma. DISCUSSION: This case demonstrates a presentation of bony metastasis of gastric cancer. This metastatic location is rare and unusual. It highlights the importance of maintaining high clinical suspicion in patients with metastatic disease. Additionally, it reinforces the relevance of a multidisciplinary approach when evaluating malignancy.

A 28-Year-Old Woman with Ascites and Multiple Focal Spleen Lesions.

Serous effusions complicating the course of lymphomas occur commonly in the pleural space but seldom in the peritoneum, where they most often present as chylous ascites with diagnostic cytology. Almost invariably, in these rare cases, the serum to ascites albumin gradient is low. We describe a 28-year-old woman with anasarca, ascites and a serum to ascites albumin gradient of 1.1 g/dl, consistent with portal hypertension. No tumour cells were detected in the ascitic fluid. However, a CT scan of the chest and abdomen disclosed liver and spleen enlargement and multiple enlarged retroperitoneal lymph nodes, suspicious for a lymphoproliferative disorder. Bone marrow aspiration and biopsy were not diagnostic, so a decision was made to proceed with a splenectomy despite the onset of low-grade disseminated intravascular coagulation.Surgery was uneventful. Diffuse large B cell lymphoma was diagnosed. A liver biopsy taken at the time of surgery demonstrated that the liver parenchyma was massively infiltrated by reactive T lymphocytes surrounding rare large CD20+ tumour cells. This infiltrate had likely led to increased portal pressure attended by ascites formation, which resolved completely after chemotherapy. The case emphasizes the rewards of pursuing a diagnosis supported by a high prior probability even in the presence of apparently discordant laboratory findings, as well as the importance of performing a diagnostic splenectomy in case of splenomegaly with unexplained focal lesions.LEARNING POINTSLymphomas may present with serous effusion, which is usually chylous and with positive cytology when represented by ascites accumulation; non-chylous effusions can be due to altered lymphatic drainage, extrinsic compression of the portal vein by enlarged lymph nodes as well as massive infiltration of the liver by lymphoma.If the cause of splenomegaly is unclear, diagnostic splenectomy remains a viable option.The diagnosis of lymphoma should always be pursued, even if it requires apparently unwise surgery, since this type of cancer can be treated effectively only if thoroughly characterized pathologically and molecularly.

Management of Residual Mass in Germ Cell Tumors After Chemotherapy.

The purpose of this review is to educate medical oncologists on the management of patients with residual germ cell tumors and the role of surgical resection after platinum-based chemotherapy. Patients with non-seminomatous testicular cancer and residual enlarged retroperitoneal lymph nodes > 1cm following induction chemotherapy with normal tumor markers should undergo a post-chemotherapy retroperitoneal lymph node dissection. All patients with primary mediastinal non-seminoma should undergo surgical resection of the mediastinal mass post-chemotherapy. These are complex surgeries and require expert surgeons in high-volume centers. Patients with advanced testicular seminoma who have residual masses less than 3cm after chemotherapy can be observed without further intervention. Patients with a residual mass > 3cm should be evaluated with PET scan after 6weeks of chemotherapy. Residual mass with negative PET scan can be followed by surveillance while a positive PET scan requires further work up to rule out active disease.

Intraductal Papillary Mucinous Neoplasm of the Duct of Santorini in the Absence of Pancreatic Divisum

Introduction: Intraductal Papillary Mucinous Neoplasm (IPMN) is a mucin-producing cystic neoplasm of the pancreas that arises from the main pancreatic duct or one of the branch ducts. The literature has described a small number of IPMNs arising from the accessory duct of Santorini, with the majority of these cases in the setting of pancreatic divisum. We report a case of IPMN of the accessory duct of Santorini in a transgendered male patient without pancreatic divisum. We provide an endoscopic image of the fish-mouth appearance of the minor papilla, which has not been previously reported in the literature. Case Report: A 59-year-old transgender male presented with right-sided abdominal pain. Abdominal KUB revealed a severely enlarged liver silhouette. History revealed the patient to be transgendered, on estradiol, conjugated estrogens, and medroxyprogesterone acetate. Physical exam revealed no abnormalities and all labs, including liver function tests (LFTs) were within normal limits. CT showed hepatomegaly and a 5.5 X 5.5 X 21 mm cystic structure in the head and uncinate process, which was not contiguous with the extrahepatic common bile duct or main pancreatic duct with several enlarged retroperitoneal lymph nodes. On MRI/MRCP a tubular markedly hyperintense T2 lesion measuring 27 X 11 X 9 mm was present. This lesion appeared in continuity with the distal accessory pancreatic duct, which appeared mildly dilated with communication to the main pancreatic duct. On endoscopy, the major papilla appeared normal. The minor papilla had a fish-mouth appearance exuding mucoid material. EUS revealed tubulocystic dilation of the duct of Santorini measuring 1.3 X 0.6 cm. There was also dilation of a side-branch towards the communication between the accessory duct and the main pancreatic duct. The pancreatic duct was normal in contour and caliber through the remainder of the gland. Fine needle aspiration (FNA) was done and fluid analysis revealed amylase of 14780 Units/L and carcinoembryonic antigen (CEA) of 89.1. Cytological analysis of the fluid was nondiagnostic. Patient referred for Whipple, but declined surgical intervention.1257_A.tif Figure 1: No Caption available.1257_B.tif Figure 2: No Caption available.1257_C.tif Figure 3: No Caption available.Discussion: IPMNs are generally found incidentally on imaging. While guidelines for managmenent of MD- and BD-IPMNs are clear, an IPMN of the duct of Santorini is a rare entity with no diagnostic recommendations currently in place, though one study suggests surgical resection. Our patient has done well with endoscopic monitoring for 7 years.

Sunitinib rechallenge with dose escalation in progressive metastatic renal cell carcinoma: A case report and literature review.

Rationale:We aimed to present a case of sunitinib rechallenge with dosage escalation after disease progression, hopefully, providing an optional approach to the personalized medication management of progressive metastatic renal cell carcinoma (mRCC).Patient concerns:The patient was admitted to hospital due to right kidney mass, with merged enlargement of retroperitoneal lymph nodes. Subsequent surgery and sunitinib treatment was administered.Diagnoses:Postoperative pathologic diagnosis was type II papillary renal cell carcinoma (pRCC) (Fuhrman grade 3) with metastases of retroperitoneal lymph nodes (T1aN1M0).Interventions:The patient underwent cytoreductive nephrectomy followed by treatment of sunitinib standard therapy (4/2 schedule) and alternative schedules according to different disease status. The patient received alternative 2/1 schedule while experiencing grade 3/4 adverse events. Re-challenge with sunitinib upon disease progression and metastasectomy were given. After second disease progression, sunitinib rechallenge with dose escalation was administered. Around 2/1 schedule showed desirable efficacy and better tolerance.Outcomes:After 4 months of sunitinib individualized treatment, a complete response with retroperitoneal metastases was achieved. Rechallenge with sunitinib after disease progression and also rechallenge with dose escalation after second disease progression were effective.Lessons:Cessation of sunitinib in patients with complete response is not suggested. Also, strategy of subsequently administered sunitinib after metastasectomy is seemed to be effective. What is more, sunitinib rechallenge with escalation to 62.5 mg probably possess value in progressive mRCC and has a well tolerance when sunitinib is rechallenged. Based on this case, we probe a feasible alternative strategy in personalized therapy of sunitinib, hoping for providing referable insights into the detailed strategies of individual treatment for patients with mRCC.

IgG4 related disease involving the renal pelvis: a case report and the literature review

Objective To summarize the characteristics of IgG4 related disease(IgG4-RD), and to avoid unnecessary surgical procedures due to misdiagnosis. Methods Retrospective analysis of the clinical data of one case of IgG4 related disease involving the renal pelvis in our hospital, and the clinical features of IgG4 related disease involving the renal pelvis were reviewed and discussed. A 56-year-old man presented with microscopic hematuria, CT showed right renal pelvis and ureteral wall thickening, local soft tissue density, post contrast CT showed low-density renal pelvic mass and the wall of the ureter-pelvic was irregularly thicken, indicating renal pelvic cancer and lymph node metastasis. PET-CT(18F-FDG) findings indicated that the renal pelvic mass was a malignant tumor, because the glucose metabolism was very high. The preoperative diagnosis was retroperitoneal lesion, suspecting renal pelvic carcinoma or lymphoma. The patient underwent 3D laparoscopic nephroureterectomy with out preoperative biopsy. During operation, we found extensive enlargement of the right renal portal and retroperitoneal lymph nodes, including that the renal portal and the renal pelvis mass had a serious adhesion to the vena cava and renal arterial vein. Results Postoperative pathology results revealed lymphatic tissue hyperplasia and germinal center formation. Immunohistochemical staining showed that most plasma cells positive expression of IgG4 (IgG4>40%, IgG4>100 /HPF), reactive hyperplasia of lymph node, considering the IgG4 related diseases. Serum IgG4 level was 15.2 g/L(0.03-2.01 g/L)after operation. Combined with serological examination, IgG4 related disease was confirmed. There was no evidence of IgG4-related disease at any other site during the follow up period, thereby obviating the need for any additional therapy. Conclusions IgG4 related disease is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells, which leads to the enlargement of the organ or nodular/proliferative lesions. The disease can be revealed as a soft tissue tumor of the renal pelvis and be misdiagnosed as malignant tumor. Surgical intervention could be avoided according to the correct diagnosis. Key words: IgG4 related disease; Renal pelvic carcinoma; Surgery; Diagnosis and therapy

ПАЛЛИАТИВНО-КУРАТИВНЫЕ ХИРУРГИЧЕСКИЕ ВМЕШАТЕЛЬСТВА У БОЛЬНЫХ РАКОМ ЖЕЛУДКА С МЕТАСТАЗАМИ В ПЕЧЕНЬ

Objective: To assess the effectiveness of relatively curative surgical interventions in the treatment of patients with gastric cancer with metastases to the liver and to show the role of active surgical tactics in improving the quality of life of patients. Methods: The study included 28 patients with gastric cancer with metastases to the liver. Patients with type I and type II Borrmann’s tumor growth (17 (85.7%) patients) were prevalent. It was found that in the predominant majority of patients had lesions of regional lymph nodes (91.6%). Detection of ultrasound and CT studies of enlarged retroperitoneal lymph nodes, lymph nodes of the aortocaval space, as well as massive invasion of the serous cover of the stomach with the extra organ component detected by laparoscopy, is a contraindication for performing simultaneous operations on the stomach and liver. In 14 (50%) patients,surgical interventions were carried out against the background of a complicated clinical course of gastric cancer: in the presence of pylorostenosis in 9 (32%) cases and torpid (sluggish) bleeding in 5 (17.85%) cases. Results: Analyzing the primary tumour and metastatic hearth in the liver, we came to the conclusion that gastric cancer with gastric metastases in the liver should be resected in cases of highly or moderately differentiated adenocarcinoma that has exophytic or mixed forms of growth, preferably with the intactness of the serous cover of the stomach. Practically in half of the patients in our series, the metastasis in regional lymph nodes are revealed,however, only in two, the defeat of lymph nodes of the second – order was diagnosed. Thus, “resectable” liver metastases can occur even at the N0 stages. In the studied group, patients with type I and II Borrmann’s prevailed with type I and II, most of them showed the intestinal histological type of a tumour. Conclusion: Liver resection for gastric cancer metastases should be performed under the following conditions: the absence of other distant metastases (peritoneum, non-regional lymph nodes, etc.); performing “radial” gastrectomy with D2-D3 lymphadenectomy; the possibility of carrying out macroscopic radical resection of the liver; functional portability of the operation; physiological operability. Keywords: Gastric cancer with liver metastases, palliative operations, indications, prognosis.

Extramedullary Involvement of Lymph Nodes in Multiple Myeloma

Abstract Myeloma multiplex is a malignant disease of bone marrow plasma cells. It is usually confined to the bone marrow, but in rare cases, patients can develop extramedullary disease. The involvement of lymph nodes is rare and can be a diagnostic challenge. Here, we describe a 36-year-old male patient who presented with abdominal pain and discomfort initially. An abdominal ultrasound followed by computed tomography (CT) revealed retroperitoneal and mesenteric lymph node enlargement. Biopsies of the abdominal lymph node and infiltrated colon showed a plasma cell infiltrate positive for CD79α, CD38, CD138, kappa light chain and VEGF2. Multiple myeloma with extramedullary localization was diagnosed. After six cycles of chemotherapy consisting of doxorubicin, dexamethasone and thalidomide followed by autologous haematopoietic cell transplantation, the patient achieved complete remission. Specifically, a CT scan after therapy showed enlarged lymph nodes in the abdomen, but PET CT scans did not detect any metabolically active foci. Thee years after the completion of therapy, the patient remains in remission. This case illustrates a rare presentation of extramedullary myeloma involving the abdominal lymph nodes, which could have been potentially mistaken for a lymphoid malignancy.

Prediction of high-grade ureteral urothelial carcinoma on CT urography.

Objective:To evaluate the correlation between CT urography (CTU) findings and histological grade of ureteral urothelial carcinoma (UUC), and to identify predictors of high-grade UUC.Methods:CTU images of 73 patients with pathologically proven UUC via nephroureterectomy were independently reviewed by two radiologists for tumour size, tumour location, hydronephrosis grade, periureteral infiltration, presence of enlarged retroperitoneal lymph nodes and tumour enhancement value. Interobserver agreement was assessed with kappa statistics. Histological grade was classified as either low or high according to the WHO 2004 classification system and pathologic T stage was assessed according to the TNM staging system. Binary logistic regression, Spearman correlation analysis and receiver operating characteristic curves were used to evaluate relationships between CTU findings and histological grade.Results:58 patients had high-grade UUCs and 15 had low-grade UUCs. Among CTU features, only hydronephrosis grade was significantly correlated with high tumour grade for both readers (p < 0.001). Multivariate logistic regression revealed that hydronephrosis of Grade 3 or higher was a significantly independent predictor of high-grade UUC for both readers (p ≤ 0.004). Interobserver agreement was excellent for hydronephrosis grade (к = 0.862). With the cut-off value of hydronephrosis Grade 3, the sensitivity, specificity and area under the curve for predicting high-grade UUC were, respectively, 88%, 79% and 0.830 for reader 1 and 86%, 80% and 0.763 for reader 2.Conclusion:Hydronephrosis of Grade 3 or higher on CTU may be predictive of high-grade UUC.Advances in knowledge:Radical surgery should be considered for UUC causing hydronephrosis of Grade 3 or higher on CTU, even in small tumours without periureteral infiltration.

Symptomatic pancreatic sarcoidosis

Objective: Report the case of female patient with symptomatic pancreatic sarcoidosis. Methods: Case Report. Results: A 65-year-old female was referred for the evaluation of severe fatigability, poor appetite, weight loss over the past two months and abdominal pain. She denied smoking cigarettes or alcohol intake. A review of systems was unremarkable. Her physical exam revealed the abdomen was soft, non-distended and non-tender with normal liver and spleen span. She presented with leukopenia, hypercalcemia, elevated liver enzyme an elevated alkaline phosphatase and an elevated erythrocyte sedimentation rate. An abdominal CT scan demonstrated a pancreatic head mass with retroperitoneal lymph node enlargement Magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP) were not performed. A CT scan guided pancreatic mass biopsy was performed with uncertain results. Laparoscopic pancreatic biopsy demonstrated noncaseating granulomas with Langerhan giant cells and fibrosis. Both an acid fast bacilli stain and gram stain for a fungal infection were negative. The patient presented elevated Angiotensin converting enzyme levels. Oral prednisone 40 mg daily for 7 days followed by a 7 week tapering schedule was initiated. The patient's signs and symptoms improved abdominal CT scan 6 weeks after treatment revealed no change in the size of the pancreas and peripancreatic lymph nodes. After 8 week of prednisone she remains clinically stable without any abnormal laboratory parameters. Conclusion: Sarcoidosis is a chronic multiorgan disease that uncommonly involves the pancreas. Pancreatic involvement is usually asymptomatic and discovered on autopsy. However, doctors should be cognizant that sarcoidosis can present with symptomatic pancreatic mass, albeit rarely.

Merkel Cell Carcinoma.

Merkel cell carcinoma is a primary neuroendocrine tumour of the skin arising from the dermis and extending into the subcutis, occurring commonly in sun-exposed areas of the head and neck of the elderly. Clinically, non-specific erythematous or violaceous firm nodules or small plaques which may be surrounded by small adjacent satellite tumours are seen. Diagnosis of Merkel cell carcinoma is difficult by light microscopy alone and need of ancillary techniques like electron microscopy and immunohistochemistry may be necessary. Treatment modalities are dependent on the stage of the disease. We report a 73-year-old male who presented with multiple nodular swellings on the right buttock, right side of the lower abdomen, and right upper thigh since 2months. Biopsy revealed Merkel cell carcinoma and immunohistochemistry confirmed it. In order to stage the disease, an ultrasound abdomen was done which showed multiple enlarged para-aortic lymph nodes and retroperitoneal lymph nodes with necrotic centres and right-sided hydroureteronephrosis and computed tomography is advised for further co-relation. Computed tomography of the abdomen showed extensive lymphadenopathy with perinodal fat forming conglomerate masses in the para-aortic, aortocaval, retrocaval, bilateral iliac, and inguinal region encasing the aorta, vena cava, and bilateral iliac arteries; extensive soft tissue density nodules in the skin extending into the subcutaneous plane and superficial muscular plane of the gluteal region and lower abdomen; and mild hydronephrosis of the right kidney due to encasement of lower ureter by lymphadenopathy. We report a case of Merkel cell carcinoma with distant metastases with the same relevant discussion.

CT characteristics of the sero-hepatic type of hepatic tuberculosis

Objective To study the CT features of the sero-hepatic type of hepatic tuberculosis. Methods This is a retrospective, multi-center and cross-sectional study. 17 patients with the sero-hepatic type of hepatic tuberculosis from the Yueqing People’s Hospital (n=5), Second Affiliated Hospital of Wenzhou Medical College (n=5), and Wenzhou People’s Hospital (n=7) were studied. All these patients were fasted for 8 h prior to CT scanning. They underwent enhanced after conventional CT breathless scanning with no abdominal pressure. Results The CT scans displayed 41 lesions in these 17 patients with the sero-hepatic type of hepatic tuberculosis, including a solitary lesion in 13 patients and multiple lesions in 4 patients. The diameter of the lesions varied from 0.8 to 4.7 cm, with a mean ± S. D. of 2.38±4.82 cm. The CT features showed a localized spindle-shaped focal lesion just underneath the liver capsule with resultant concave compression of the adjacent liver tissue (n=22), spotted calcifications in the center of the focal nodules (n=1); and aggregation of multiple low density nodular foci (n=19). Additional CT features included focal nodules surrounded by a small amount of liquid (n=15), compression of adjacent liver tissue (n=22), a small amount of ascites (n=8), and retroperitoneal lymph nodes enlargement (n=2). These nodules showed moderate (n=5) and slight enhancement in the arterial phase (n=36); moderate (n=32) and mild enhancement in the portal venous phase (n=9); and moderate (n=32) and mild enhancement in the parenchymal phase (n=9), respectively. The nodules showed ring-shaped (n=26), honeycomb or multiple ring-shaped enhancement (n=15). The enhanced ring-shaped wall thickness varied from 0.2 to 0.9 cm, with a thin wall (n=30) and a thick wall (n=11). The center of the focal nodule was a low density sac-shaped area, with no obvious contrast enhancement. The CT value was 21 to 39 hu. The infiltrative liver tissues which surrounded the focal nodules were shown as lamellar areas of obvious arterial enhancement, with equidensity in the portal vein phase and equilibrium phase. Conclusions CT showed characteristic features of the sero-hepatic type of hepatic tuberculosis. Clinical and laboratory findings could provide important supplementary information to make the diagnosis. Key words: Hepatic tuberculosis, sero-hepatic type; Tomography, X-ray computed

Unusual Soft Tissue Uptake of F-18 Sodium Fluoride in Three Patients Undergoing F-18 NaF PET/CT Bone Scans for Prostate Cancer.

Three males aged 71 to 80years with known stage IV metastatic prostate cancer underwent F-18 sodium fluoride (NaF) PET/CT to assess osseous metastatic disease burden and stability. In addition to F-18 NaF avid known osseous metastases, each patient also exhibited increased F-18 NaF activity in soft tissues. The first patient exhibited multiple F-18 NaF avid enlarged retroperitoneal and pelvic lymph nodes on consecutive PET/CT scans. The second patient demonstrated an F-18 NaF avid thyroid nodule on consecutive PET/CT scans. The third patient exhibited increased F-18 NaF activity in a hepatic metastasis.