Conglomerated Lymph Nodes Research Articles

Hearing loss, why bronchial tree may be involved?

A 62-years-old man former occasional smoker was hospitalized for progressive hearing loss and Magnetic Resonance Imaging (MRI) detected multiple round hyperdense lesions in each cerebral hemisphere. Total body Computed Tomography (CT) scan showed a lobulated consolidative lesion on the right lung lower lobe associated to conglomerate lymph nodes (11R) suspected for primary lung cancer. Endoscopy showed an endobronchial invasion and integrated endobronchial ultrasound did not demonstrate any accessible lymph node for sampling. Forceps biopsy report on the endobronchial specimen led to histopathological diagnosis of metastatic melanoma. Skin and ophthalmologic examinations were negative for suspicious pigmented lesions findings and patient had no history of familiarity for melanoma. Malignant melanoma is rarely observed to metastasize to endobronchial tissue and it is represented only in the 4.5% of cases. The vast majority of endobronchial metastases are metachronous, even after several years. Nevertheless, the anachronous manifestations are possible.

Refractory/Relapsed High-Grade B-Cell Lymphoma in the Elderly: A Special Therapeutic Challenge

BACKGROUND: According to the 2016 World Health Organization classification of lymphoid neoplasms, high- grade B-cell lymphoma (HGBL) is defined as a new category for aggressive diffuse large B-cell lymphomas (DLBCL), including DLBCL NOS as well. CASE REPORT: An 80-year-old patient was admitted because of an enlargement of the lymph node located in the right neck region. The patient underwent 6 cycles of R-CHOP, which is considered to be the standard treatment for non-Hodgkin lymphomas (NHL) DLBCL (NOS) CSII B “Bulky,” and he subsequently achieved complete remission (CR). A lymph node biopsy was performed, PH No. 12742/21, which revealed the following: LCA+, CD79 α CD20+, and CD5+, in approximately 50% of the cells, NHL DLBCL (NOS) was of GCB origin, etc. The international prognostic index risk score was 2, the cumulative illness rating scale score was 4. In addition, no bone marrow infiltration was reported. A COP-based chemotherapy protocol was administered, after which the evaluation showed disease relapse. Multi-slice spiral computed tomography (MSCT) follow-up showed an enlarged conglomerate of lymph nodes in all the neck levels. Dg: NHL DLBCL (NOS) CS IVB “Bulky” E (the hypopharynx). The echocardiography results were the following: (EF 50%, aortic insufficiency with MR1+, TR1+ of the aortic valve 3+). The patient was presented to the Consilium, who decided to apply the following therapy (Polatuzumab vedotin + Rituximab + Bendamustine). After III cycle of treatment, MSCT follow-up was performed. The patient gained weight, oral food intake was enabled, whereas the quality of life was significantly improved. CONCLUSION: The administration of ta treatment may be provided to elderly patients with relapsed HGBL.

A Case of Metastatic Melanoma in a Female Patient with Vitiligo

Patients with metastatic melanoma occasionally develop de novo hypopigmentation, pathologically indistinguishable from Sutton's nevus and common vitiligo, respectively. The case of a 68-year-old female with vitiligo of 2 years of evolution is documented, presenting a left inguinal mass and a para-aortic lymph node conglomerate. In the left inguinal region, a ganglion with thickened cortex and liquid content was identified. The laboratory results report hemoglobin of 8.30 g/dL, leukocytes 19.12 u/L, albumin 2.2 mg/dl, DHL 1222 Ul/L, alpha-fetoprotein 2 ng/ml, Ca-125 14.30 U/ml, Ca-19.9 8.60 U/ml and carcinoembryonic antigen of 2.14 ng/ml. Upon inspection, hypochromic, bilateral, and symmetrical lesions can be seen on the back of the hands, wrists, scalp, facial region, and neck. Biopsy showing malignant metastatic melanoma is performed. Starting management with Pembrolizumab 1 mg/kg every 21 days. Currently in follow-up and adjuvant treatment. Melanoma is the most aggressive form of skin cancer. The association of malignant melanoma with de novo hypopigmentation has been found, synchronously or following the diagnosis of the tumor. In recent years, it has led to the development of monoclonal antibodies. However, its diagnosis has implications for life prognosis due to high mortality.

Cutaneous Sarcoidosis Masquerading as Papular Amyloidosis Revealed Post--Hair Removal.

Cutaneous Sarcoidosis Masquerading as Papular Amyloidosis Revealed Post--Hair Removal.

Not every retroperitoneal mass is malignant

Background: Cystic lymphangioma is a rare benign tumor of the lymphatic system, most commonly observed in the neck and axilla (in 95% of cases). Less than 1% of lymphangiomas occur in the retroperitoneum. Case report: A 46-year-old female was evaluated for bilateral flank pain, asthenia, and dyspnea with a one-year history. Abdominal computed tomography scan revealed an extensive conglomerate of lymph nodes in the middle retroperitoneum with a predominantly cystic appearance. An excisional biopsy confirmed the diagnosis of cystic lymphangioma on anatomopathological examination. Due to compression of retroperitoneal structures, an almost total cytoreductive surgery was proposed. However, in the immediate postoperative period, the patient developed thrombosis of the left renal vessels, resulting in renal hypoperfusion. No disease recurrence was observed after 3 years of follow-up. Conclusion: This case presents a rare occurrence of cystic lymphangioma in adults, highlighting the importance of considering this entity in the differential diagnosis of retroperitoneal lymphatic masses.

Lymphoproliferative disorder – Castleman’s disease with early childhood-onset in a child (a clinical case)

Castleman’s disease (angiofollicular lymphoma, giant lymph node hyperplasia, Castleman’s pseudotumor) is an understudied orphan lymphoproliferative disease with a long period of asymptomatic course and a high risk of malignancy, and variability of its clinical features can cause difficulties in diagnosis. The aim of the study. To acquaint clinicians and increase the effectiveness of early diagnosis and treatment of Castleman’s disease (CD) in children to prevent malignancy and improve prognosis. Results. The article presents a clinical case of CD in a 3.5-year-old boy with a long history of fever, stunted growth, sweating, arthralgia, anemia, high laboratory inflammatory factors, elevated IL-6. There was a complex diagnostic search except for infectious, immunodeficiency, autoimmune conditions. The diagnosis of hyaline-vascular type of CD was confirmed by immunohistochemical examination after lymph node excisional biopsy. Rapid positive dynamics was noted after radical removal of the lymph node conglomerate. At follow-up after 8 months, the patient met criteria for clinical and laboratory remission. Conclusions. Castleman’s disease should be included in the search algorithm for lymphoproliferative diseases. Determining the role of genetic mutations in interferon regulatory factor (IRF8) is of scientific interest to clarify the etiology of this disease.

Diagnostic difficulties of small intestinal neuroendocrine tumors: clinical case

The objective of the study was to demonstrate the difficulties of diagnosing the neuroendocrine tumor (NET) of the small intestine at the example of a concrete clinical case. In the case presented, clinical manifestations of the disease were nonspecific and the examination began from a low-informative method multispiral computed tomography (MSCT) without a bolus contrast study that was the reason of wrong diagnosis for 7 months. The detection of the swollen lymph node in the mesentery was interpreted incorrectly; additionally, only endoscopic investigation of the stomach and large intestine was used. By means of MSCT-angiography we succeeded to diagnose not only the lymph node conglomerate in the mesentery, but to reveal a tumor sized 915 cm in the ileac wall. Laparoscopy with lymph node biopsy after its morphological study permitted to verify the diagnosis before the surgery.
 To reduce the period of diagnostics of small intestinal NET, general practitioners should be informed on specific features of clinical manifestations of the disease and rational diagnostic methods. MSCT-angiography showed its efficiency in detection of small-sized small intestinal NET, and laparoscopy with biopsy of the mesentery lymph node will permit to verify the diagnosis before surgery.

Radical surgical treatment of locally advanced thyroid cancer

The result of radical treatment of locally advanced thyroid cancer with metastases to the regional lymph nodes of the neck on the left, with tissue breakdown and skin fistula formation, is presented. An 84-year-old female patient has severe concomitant pathology, such as ischemic heart disease, widespread atherosclerosis, cardiosclerosis, and permanent atrial fibrillation (tachy form, Grade 2A). A conglomerate of lymph nodes with disintegration in the projection of the external jugular vein and possible profuse bleeding was considered an absolute indication for surgery, which was performed on February 10, 2015. Considering the conglomeration of the lymph nodes of the neck, which displaced the trachea to the right, an endoscopist was called in, who performed tracheal intubation through the nose using a fiberoptic bronchoscope. The surgical treatment included combined thyroidectomy, a Criles operation on the left side, and the removal of the conglomerate of the lymph nodes of the neck as well as the adjacent area of the skin in a single block. The postoperative period was uneventful. On the second day after surgery, the patient was examined by an endocrinologist. The prescribed treatment was L-thyroxine 75 mg once a day in the morning. On the fifth day, the patient was discharged with recovery to be treated in the outpatient clinic by an oncologist and an endocrinologist.

SEVERE PRURITUS AS A PRESENTING SYMPTOM OF HODGKIN'S LYMPHOMA

<h3>Introduction</h3> Pruritus has a broad differential diagnosis. Rarely, this symptom may be the sole manifestation of an underlying malignancy. A patient with 5 years of severe upper body pruritus with no other systemic symptoms was subsequently diagnosed with Hodgkin's lymphoma. <h3>Case Description</h3> A 43-year-old male presented to the allergy-immunology clinic for evaluation of severe pruritus of 3 years' duration; he reported nocturnal worsening awakening him from sleep. The pruritus was refractory to antihistamines but was partially responsive to ice packs. There were no associated constitutional or respiratory symptoms, or lymphadenopathy. CBC with differential and CMP were normal. Omalizumab therapy was administered for 3 months; however, the severe pruritus persisted. Neuropathy work-up by neurology was negative. Dermatology evaluation yielded a diagnosis of folliculitis. Work-up for auto-immune disease by rheumatology was negative. After a total of 5 years of pruritus without identifiable cause, he presented for evaluation of a new-onset supraclavicular mass. Chest CT revealed a large anterior mediastinal mass and a massive conglomerate of lymph nodes. Subsequent biopsy revealed classic Hodgkin's lymphoma. <h3>Discussion</h3> This case underscores that persistent pruritus may be the presenting symptom of malignancy. Pruritus associated with malignancy is classically refractory to antihistamines and may not be accompanied by classic systemic symptoms. While rare, it is important to keep this atypical presentation of malignancy in the differential.

Development of scalable lymphatic system in the 4D XCAT phantom: Application to quantitative evaluation of lymphoma PET segmentations.

Digital anthropomorphic phantoms, such as the 4D extended cardiac-torso (XCAT) phantom, are actively used to develop, optimize, and evaluate a variety of imaging applications, allowing for realistic patient modeling and knowledge of ground truth. The XCAT phantom defines the activity and attenuation for a simulated patient, which includes a complete set of organs, muscle, bone, and soft tissue, while also accounting for cardiac and respiratory motion. However, the XCAT phantom does not currently include the lymphatic system, critical for evaluating medical imaging tasks such as sentinel node detection, node density measurement, and radiation dosimetry. In this study, we aimed to develop a scalable lymphatic system in the XCAT phantom, to facilitate improved research of the lymphatic system in medical imaging. Using this scalable lymphatic system, we modeled the lymph node conglomerate pathology that is characteristically observed in primary mediastinal B-cell lymphoma (PMBCL). As an extended application, we evaluated positron emission tomography (PET) image quantification of metabolic tumor volume (MTV) and total lesion glycolysis (TLG) of these simulated lymphomas, though the phantoms may be applied to other imaging modalities and study design paradigms (e.g., image quality, detection). A template model for the lymphatic system was developed based on anatomical data from the Visible Human Project of the National Library of Medicine. The segmented nodes and vessels were fit with non-uniform rational basis spline surfaces, and multichannel large deformation diffeomorphic metric mapping was used to propagate the template to different XCAT anatomies. To model conglomerates observed in PMBCL, lymph nodes were enlarged, converged within the mediastinum, and tracer concentration was increased. We used the phantoms as inputs to a PET simulation tool, which generated images using ordered subsets expectation maximization reconstruction with 2-8mm Gaussian filters. Fixed thresholding (FT) and gradient segmentation were used to determine MTV and TLG. Percent bias (%Bias) and coefficient of variation (COV) were computed as measures of accuracy and precision, respectively, for each MTV and TLG measurement. Using the methodology described above, we introduced a scalable lymphatic system in the XCAT phantom, which allows for the radioactivity and attenuation ground truth to be generated in 116±2.5s using a 2.3GHz processor. Within the Rhinoceros interface, lymph node anatomy and function were modified to create a cohort of 10 phantoms with lymph node conglomerates. Using the lymphoma phantoms to evaluate PET quantification of MTV, mean %Bias values were -9.3%, -41.3%, and 20.9%, while COV values were 4.08%, 7.6%, and 3.4% using 25% FT, 40% FT, and gradient segmentations, respectively. Comparatively for TLG, mean %Bias values were -27.4%, -45.8%, and -16.0%, while COV values were 1.9%, 5.7%, and 1.4%, for the 25% FT, 40% FT, and gradient segmentations, respectively. In this work, we upgraded the XCAT phantom to include a lymphatic system, comprised of a network of 276 scalable lymph nodes and corresponding vessels. As an application, we created a cohort of phantoms with lymph node conglomerates to evaluate lymphoma quantification in PET imaging, which highlights an important application of this work.

A Novel Candidate Gene for Predisposition of Hodgkin Lymphoma; Pregnan X Receptor (PXR)

ObjectiveHodgkin lymphoma (HL) is a lymphoproliferative malignancy of B-cell origin and one of the common tumors in adolescent and young adults. Less than one percent of HL patients have a family history and siblings of the affected individual may have an increased risk of developing the disease. HL risk is associated with immunodeficiency syndromes. Clustering of cases of HL within families may also suggest a genetic predisposition. In cases with familial clustering, recognition of genetic predisposition genes in the pathogenesis of HL may offer therapeutic benefits, and provide genetic counseling and surveillance for the family members for early diagnosis. We had previously reported CD70 signaling defect in an extended family with multiple consanguineous marriages and individuals affected with Hodgkin lymphoma and/or immune deficiency (J Clin Immunol.2020, 40(6):883-892). The aim of this study is to investigate for a cancer predisposition gene/condition in two siblings with HL, whose parents had a consanguineous marriage.Material and MethodsA 10 year-old girl with progressive HL was referred for further evaluation and treatment. She had been diagnosed with stage IIIBs HL, nodular sclerosing subtype and had received chemotherapy (ABVD) where she was reported to have partial response, followed by progressive disease. The tumor was CD30 positive. At admission, the patient had a massive enlarged mediastinum due to conglomerate lymph nodes, nodules in the lung, lymph adenomegalies in the cervical, supraclavicular, axillary regions. She was treated with ifosfamide+mesna, etoposide, carboplatinum (ICE) and brentuximab and achieved a complete metabolic response in PET-CT evaluation after six courses. She is transferred to receive stem cell transplantation. Her 24 year-old sister was also diagnosed with early stage HL during pregnancy, had a healthy delivery, was treated with chemotherapy and was in remission.In this study, we performed exome sequencing of the index case (IV-7). The elder sister who was diagnosed with HL and the parents, whose informed consents were taken. There were five children in the family; two of the girls were (IV-7 and IV-11) diagnosed with HL, a boy (IV-8) with inflammatory skin findings, a sister (IV-9) and a cousin (IV-13) with suspected lymphoma, and a healthy girl (IV-10) (Figure 1). After quality control and alignment steps, variants were annotated using ANNOVAR, dbSNP, and ExAC. Variant evaluation was performed by in-house filtering steps based on MAF<0.1%, CADD score >10, SIFT, PolyPhen, MutationTaster, FATHMM scores. We also used Pediatric Cancer Variant Pathogenicity Information Exchange (PeCanPIE) tool for identification and classification of the variants.ResultsA germline homozygous missense gene variation (c.928 G>A, p.D310N, rs144833620) was identified on the ligand binding domain of the Pregnane X Receptor Gene (PXR or NR1I2) gene. Sanger sequencing confirmed the familial segregation; IV-7 and IV-11 HL cases were homozygous and parents (III-13 and III-14) were heterozygous. The index case did not have elevated EBV-DNA PCR levels. Index case had increased CD3, CD2 and CD8 and reduced CD4 levels.(Conclusion)The PXR encodes a member of the nuclear receptor (NR) superfamily and functions in the metabolism and elimination of xenobiotics and several commonly used drugs. Heterodimerization of PXR and RXR (functional partner) lead to conformational changes upon ligand binding, functions as the transcriptional regulator of PXR target genes including metabolizing enzymes, drug transporters, inflammation and cell cycle associated genes. Previously reported Pxr-/- animal model showed that mice lacking the steroid and xenobiotic receptor develop B cell lymphoma. However, there is no case of HL published linked to variation in PXR gene. Clustering of cases of HL within families, especially in families with consanguineous marriages should suggest a genetic predisposition. The PXR seems to be a novel candidate gene for predisposition of HL.Funding: This study was funded by Scientific Research Projects Coordination Unit of Istanbul University with a project number TDK-2020-37127. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Mediastinal Mass with Unusual Etiology.

Mediastinal Mass with Unusual Etiology.

Unilateral Lower Extremity Swelling as an Unusual Presentation of Non-hodgkin’s B-cell Lymphoma

Non-Hodgkin’s B-cell lymphoma is a hematological condition with different types based on clinical presentation, pathological features, and therapeutic response, like Diffuse Large B-cell Lymphoma (DLBCL), follicular lymphoma, and Chronic Lymphocytic Leukemia (CLL). We report A 33-year-old male presented with a complaint of unilateral leg swelling, firstly supposed to be a Deep Vein Thrombosis (DVT); however, in multiple Doppler ultrasonography, DVT was ruled out. The patient’s precise history revealed a swollen mass in the left knee region for two months that slowly enlarged and was confirmed with a physical examination. Doppler ultrasonography of the lower extremities conducted in our center presented no sign of deep vein thrombosis; however, it demonstrated some complex conglomerate lymph nodes in the left inguinal compressing the ipsilateral femoral and iliac vein. It also revealed bulky adenopathy, with a 95×65 mm left knee mass. The performed biopsy reported the existence of malignant lymphoma, suggesting the existence of DLBCL. Consequently, he was referred to a hematology/oncology department for appropriate treatment. This unusual presentation of DLBCL and the importance of noticing an accurate history taking and physical examination, instead of conducting unnecessary paraclinical tests (several Doppler ultrasonography in this case), have led us to report this case.

Abdominal Positron Emission Tomography Combined With Magnetic Resonance Imaging in Chronic Granulomatous Disease

Abdominal Positron Emission Tomography Combined With Magnetic Resonance Imaging in Chronic Granulomatous Disease

Uncommon case of the acral melanoma with chondro-osseous metaplasia

Aim. This study aimed to improve the diagnostics of acral melanoma and investigate tumor morphological features of the chondroid and osseous matrix.&#x0D; Materials and methods. The article presents a clinical case of metastatic acral melanoma with chondroid and osseous metaplasia in a 60-year-old patient. Cytological, histologic, immunohistochemical, and molecular genetic studies were performed.&#x0D; Results. A conglomerate of lymph nodes was noted in the inguinal region of the patient. Oxyphilic chondroid masses and tumor cells with morphological features of sarcoma were revealed using thin-needle aspiration biopsy. On physical examination, pink-colored subcutaneous neoplasm was discovered in the skin of the heel. Histological examination of the primary neoplasm and inguinal lymph node was performed. Melanoma with chondro-osseous metaplasia was diagnosed. Immunohistochemical examination of the tumor revealed some pronounced diffuse expressions of S-100 protein, HMB-45, and focal expression of Melan A in tumor cells. The sample was tested to detect BRAF mutation, and no mutations were found.&#x0D; Conclusion. Morphological diagnostics of acral melanoma with chondro-osseous metaplasia is characterized by a high risk of diagnostic error. Therefore, additional immunohistological, molecular, and genetic studies should be used in the diagnostics of acral melanoma with chondro-osseous metaplasia.

TRIPLE THREAT: A CASE OF MULTIPLE PRIMARY LUNG CANCERS

SESSION TITLE: Medical Student/Resident Lung Cancer Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Multiple primary lung cancers (MPLC) are a rare occurrence and have 2 forms – synchronous (sMPLC) and metachronous (mMPLC). The sMPLCs are physically distinct with different histological subtypes. If they are the same subtype, they must be in different lobes of the lung, originating from carcinoma in situ, and no tumor in common lymphatics and extrapulmonary metastases. The mMPLCs tumors must be histologically different or if they are histologically identical, there must be a >2 year cancer free interval, originate from carcinoma in situ, and second cancer must be in a different lobe or lung with no carcinoma in the common lymphatics and no extrapulmonary metastases. mMPLC is common and secondary to treatment of initial lesions. Tobacco smoke may also create a “field effect” in which multicentric lung cancers manifest more frequently. CASE PRESENTATION: A 66-year-old female presented for follow up of sMPLC Stage IB left upper lobe adenocarcinoma/Stage IA right middle lobe squamous cell carcinoma. Patient was treated in 2015 via left upper lobectomy and right middle lobe pulmonary wedge resection. Patient history includes 40-pack-years of smoking, COPD, and continued smoking after treatment of prior sMPLC. Surveillance chest CT showed increase in size of subcarinal lymph node and right lymph node conglomerate encasing the right upper lobe pulmonary artery, consistent with metastasis. Fine needle aspiration of level 4R lymph nodes revealed cells clusters with nuclear molding, high nuclear to cytoplasmic ratio, inconspicuous nuclei, minimal cytoplasm, and crush artifacts. Immunohistochemically tumor cells were positive for TTF-1 and synaptophysin, and negative for P40 – confirming diagnosis of small cell carcinoma. DISCUSSION: Small cell lung cancer (SCLC) and non-small cell lung cancer (NSCLC) are known to be linked with tobacco, creating a “field effect” that promotes the possibility of MPLCs. NSCLC include adenocarcinoma and squamous cell carcinoma (SCC). SCLC and NSCLC are differentiated based on morphology and immunohistochemical staining, the results of which confirmed diagnosis of small cell carcinoma and thus qualified as mMPLC in our case. Treatment of mMPLC is often surgical resection, however, feasibility must be assessed based on pulmonary reserve. Limited resections should be attempted in all cases as segmentectomies have similar outcomes to lobectomies in terms of recurrence-free survival, notably in tumors under 3 cm. CONCLUSIONS: In patients with a history of MPLC associated with smoking, physicians should be cognizant of additional malignancies different from the original lesion subtype. Biopsy and histopathology are the gold standard for diagnosis. Surgery is the standard treatment in MPLC, but clinicians should assess for sufficient pulmonary reserve to ensure viability. Reference #1: Chen C, Huang X, Peng M, Liu W, Yu F, Wang X. Multiple primary lung cancer: a rising challenge. Journal of thoracic disease. 2019; 11(Suppl 4):S523-S536. Reference #2: Chen TF, Xie CY, Rao BY, et al. Surgical treatment to multiple primary lung cancer patients: a systematic review and meta-analysis. BMC surgery. 2019; 19(1):185. Reference #3: Jiang L, He J, Shi X, et al. Prognosis of synchronous and metachronous multiple primary lung cancers: systematic review and meta-analysis. Lung cancer (Amsterdam, Netherlands). 2015; 87(3):303-10. DISCLOSURES: No relevant relationships by jayanth keshavamurthy, source=Web Response No relevant relationships by Diana Kozman, source=Web Response No relevant relationships by Samantha Mattox, source=Web Response No relevant relationships by Rohit Munagala, source=Web Response No relevant relationships by Nikhil Patel, source=Web Response No relevant relationships by Kanwar Singh, source=Web Response No relevant relationships by Asad Ullah, source=Web Response

SUN-939 Case Report: Malignant Pheochromocytoma

Introduction: The concept of malignancy for pheochromocytoma is complex and the best definition is the presence of metastases, according to WHO. Anatomopathological scoring systems are not effective in predicting metastases. Malignancy should be considered when tumors larger than 8cm (> 80g), paragangliomas (especially retroperitoneal), dopamine / methoxythyramine increase, Ki67> 6% and SDHB mutation. At 5 years, survival ranges from 50-69%. Metastases may appear 20-40 years after initial treatment of pheochromocytoma. We describe a case that metastasis was identified 33 years after pheochromocytoma excision Case report: A 57-year-old female patient with a postoperative history of 33 years of right adrenal pheochromocytoma was discharged from the endocrinologist after 10 years of follow-up. At diagnosis 33 years ago, she had symptoms of hypertension with paroxysms and weight loss that disappeared after tumor removal. 2 years investigating weight loss with general practitioner without another celebratory. On physical examination, orthostatic hypotension was highlighted. Plasma methanephrine 0.8 nmol / L (VR <0.5) and plasma normetanephrine 1.8 nmol / L (VR <0.9), chromogranin A 5.7 nmol / L (VR <3 nmol / L) and clonidine test with 36.6% suppression of metanephrines, suggesting tumor recurrence. MRI localized recurrence of the adrenals and MIBG scintigraphy with I131 that showed, respectively, in the topography next to the paracaval and retroportal right diaphragmatic crura, isointense T1 and slightly hyperintense T2 at 1.8 cm and radiopharmaceutical hypercaptation in right adrenal topography. Genetic panel by NGS did not identify germline mutation in 22 pheochromocytoma-related genes. FDG PETCT was consistent with MRI and MIBG images. Gallium PETCT68 DOTATOC detected the lesions already described, in addition to a lytic lesion in the left femoral intertrochanteric medulla. Anatomopathological approached abdominal lesion confirming pheochromocytoma metastasis in lymph node conglomerate. Currently has a negative methanephrine plasma, however chromogranin A 142 ng / mL (VR <93), and was chosen by the observant approach. Conclusion: The case of the patient illustrates that pheochromocytoma should be followed indefinitely, as metastases may appear many years later and may present different aggressiveness potentials.

Active surveillance after complete spontaneous regression of a metastatic Merkel cell carcinoma of the head

Complete spontaneous regression (CSR) of Merkel cell carcinoma (MCC) is a known phenomenon. There are no reports of an active decision to avoid resection or adjuvant treatment and keep surveillance after CSR of MCC in the literature. An 84-year-old Caucasian male patient with a 6×5 cm MCC on the left forehead, with evidence of satellite nodules and a lymph node conglomerate in the left parotid region compatible with metastasis and confirmed by 18F-FDG PET-CT, presented CSR of the neoplasm. The use of PET-CT and non-operative active surveillance strategy can assist with monitoring response, thus avoiding unnecessary resections.

Linfoma difuso de células B grandes metastásico a testículo y vejiga. Un reporte de caso

Resumen:&#x0D; Descripción del caso clínico: Presentamos el caso de un varón de 44 años, con diagnóstico de linfoma difuso de células B grandes, de tipo centro germinal, metastásico a testículo y vejiga. Se presentó con edema y dolor en miembros inferiores, retención urinaria e injuria renal aguda. Se encontró un conglomerado ganglionar retroperitoneal en la tomografía de ingreso, y alteración en los marcadores tumorales séricos. Se realizó una ecografía doppler testicular, a pesar de que el examen físico genital fue normal, encontrándose un tumor testicular. Se llevó a orquiectomía radical, y en el mismo tiempo quirúrgico, a derivación urinaria endoscópica con catéteres JJ, y se evidenció entonces, un compromiso neoplásico vesical. Los reportes histológicos de testículo y vejiga reportaron tumor maligno mal diferenciado de probable origen metastásico, y la inmuno-histoquímica reportó linfoma difuso de células B grandes de tipo centro germinal.&#x0D; Relevancia: Los linfomas no Hodgkin constituyen las principales causas de linfoma secundario de testículo y vejiga.&#x0D; Implicaciones clínicas: En pacientes hombres jóvenes con masas retroperitoneales, como en el caso de linfoma de células B grandes, es importante tomar en consideración el compromiso secundario de testículo y vejiga.&#x0D; Conclusión: el compromiso secundario de testículo y vejiga corresponde a una presentación inusual de este tipo de linfoma. Se reportó el caso con el objetivo de tomar en consideración el diagnóstico, en pacientes hombres jóvenes con masas retroperitoneales.

2697 Gastrointestinal Stromal Tumor Mimicking a Small Lymphocytic Lymphoma

INTRODUCTION: Gastrointestinal stromal tumors (GIST) have been associated with synchronous or metachronous secondary malignancies in up to 43% cases. The occurrence between GIST and hematological malignancies is approximately 7%. Here, we present a case of a GIST mimicking Small Lymphocytic Lymphoma (SLL). CASE DESCRIPTION/METHODS: A 70-year-old male presented with a one-month history of bilateral axillary lymphadenopathy. Physical exam revealed a conglomerate of lymph nodes of both axillary regions. CT of the chest-abdomen-pelvis showed diffuse axillary, mesenteric, retroperitoneal and inguinal lymphadenopathy. In addition, it showed a 40 mm mass in the lesser curvature of the stomach (Figure 1). FNA of the axillary region showed findings consistent with SLL. Upper endoscopy showed a large subepithelial mass in the stomach (Figure 2). Biopsies were taken with cold forceps showing reactive white blood cells, this was suspected to be lymphoma. The patient was diagnosed with Stage IV SLL and started on Ibrutinib. Three months after therapy was started, CT revealed significantly improved on adenopathy but an increase in the size of the gastric mass. A repeat upper endoscopy with endoscopic ultrasound (EUS) showed a hypoechoic, heterogeneous round mass with well-defined borders, originating from muscularis propria layer (Figure 3). Fine needle biopsy (FNB) was performed and showed clusters of neoplastic cells, positive for CD117 and DOG1, negative for CK7, CK20, and CDX2, consistent with a GIST. DISCUSSION: Little is known about the coexistence of GIST and other metachronous tumors of different anatomic location. Several theories have been proposed to explain this link. These theories include pro-oncogenic changes in the tissue microenvironment, carcinogenic effect by chemotherapy and desmoid-type fibromatosis after trauma or surgery. When located in the stomach, GISTs most commonly present as subepithelial lesions. Since mucosal biopsies have a false negative rate of almost 50%, additional evaluation with EUS and EUS guided tissue acquisition is of essential importance. Obtaining core samples by FNB is useful for tissue architecture evaluation and immunohistochemistry assessment of the sample. In this particular case, EUS was not performed initially; instead, mucosal biopsies were obtained leading to a false negative result. This case highlights the rare association between GISTs and other malignancies, as well as, the importance of EUS for the evaluation and accurate diagnosis of different subepithelial lesions.