Large Necrotic Mass Research Articles

Primary Adrenal Neuroblastoma in an Adult: A Rare Case Report

ABSTRACT Neuroblastoma (NB), an embryonal malignancy originating from neural crest cells, is predominantly a pediatric disease, making adult cases exceedingly rare. This case report documents the presentation and diagnostic process of a 23-year-old female diagnosed with adrenal NB, a condition scarcely encountered in adults. The patient presented with abdominal pain and a palpable mass. Imaging through computed tomography scan revealed a large necrotic mass in the left adrenal region. Ultrasound-guided fine-needle aspiration cytology (FNAC) showed round cells with scant cytoplasm and characteristic nuclear features, suggesting a round cell tumor, potentially NB. Surgical excision confirmed the diagnosis of poorly differentiated NB with low mitotic-karyorrhectic index. Adult NB, with an incidence of 1 in 10 million per year, has a notably worse prognosis compared to pediatric cases. The literature lacks consensus on optimal treatment protocols for adult patients, though a multimodal approach including surgery, chemotherapy, and possibly radioactive iodine therapy is often employed. This case underscores the utility of FNAC in preliminary diagnosis and highlights the clinical challenges posed by adult NB, emphasizing the need for more comprehensive studies to develop standardized treatment guidelines.

Urogenital schistosomiasis, a leading cause of bladder squamous cell carcinoma

Abstract Introduction/Objective Schistosoma, genus of trematodes, commonly known as blood flukes are parasitic flatworms responsible for a group of infections in humans termed schistosomiasis and hundreds of millions of people are infected worldwide. In areas where Shistosoma is not endemic to the region most cases of bladder malignancy are of urothelial origin. In areas where Shistosoma is endemic bladder malignancy is more commonly squamous cell carcinoma. However, with increased travel, migration and tourism it is important to potentially consider Shistosoma infection in areas that are not typically considered endemic regions. Methods/Case Report A 62-year-old male presented to our hospital and revealed through an interpreter he has had 1.5 years hematuria with “pieces of meat or brown dirt”. Cystoscopy was performed and showed a large necrotic mass. CT scan showed an 8.2 cm exophytic bladder dome mass. TURBT was performed and a diagnosis of invasive high-grade urothelial carcinoma with squamous differentiation and with multiple Schistosoma organisms identified.The patient received Schistosoma treatment and was scheduled for radical cystectomy and ileal conduct. A few days before the scheduled procedure the patient presented to our emergency department with acute abdominal pain. He was subsequently diagnosed with progressive bladder cancer with new left malignant ureteral obstruction, which lead to hydronephrosis, DVT, abdominopelvic ascites and intraperitoneal bladder perforation. Abdominal fluid cytology showed atypical keratinizing squamous cells suspicous for malignancy and marked acute inflammation. Patient underwent cystectomy with ileal conduit and the specimen was sent to pathology for examination. Results (if a Case Study enter NA) NA Conclusion Gross examination showed an irregular perforated bladder. Microscopic examination showed a high grade urothelial carcinoma with squamous and sarcomatoid differentiation with detrusor muscle invasion. Additionally, Schistosoma organisms with a terminal spine and calcified eggs were identified. Some of the calcified egg material was engulfed by giant cells. The organisms are surrounded by a prominent eosinophilic infiltrate. Lymphovascular invasion is also noted. Schistosomiasis is associated with the development of squamous cell carcinoma of the bladder. It is important to maintain high oncological alertness for squamous cell carcinoma, particularly for patients arriving from endemic areas. Such as in our case, a patient from Somalia living in Kansas City.

Abstract PO4-20-12: Case Report: Should core needle biopsy showing primary leiomyosarcoma of the breast warrant a second look for accurate diagnosis and management?

Abstract A 68-year-old female with unknown medical history presented to the emergency department with an exceptionally large necrotic mass of the right breast. Initial core needle biopsy showed morphology consistent with the exceedingly rare leiomyosarcoma of the breast. This patient’s necrotic, fungating tumor with abscess warranted urgent surgical excision. The pathology report that followed revealed malignant phyllodes tumor as her final diagnosis. The patient did well after surgery with no sign of metastasis and continued to follow-up with oncology. Lack of clinical guidance for breast leiomyosarcoma begs the question as to when further workup is indicated since it is known to imitate the histological and radiological features of other breast lesions. We discuss the need for updated guidance from prospective future studies to change national guidelines. Citation Format: Amanda Ritter, Sergey Shimunov. Case Report: Should core needle biopsy showing primary leiomyosarcoma of the breast warrant a second look for accurate diagnosis and management? [abstract]. In: Proceedings of the 2023 San Antonio Breast Cancer Symposium; 2023 Dec 5-9; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2024;84(9 Suppl):Abstract nr PO4-20-12.

Vaginal Leiomyoma Presenting as Pelvic Organ Prolapse in Pregnancy.

Vaginal leiomyomas are extremely rare in pregnancy. These can present in a variety of ways. They can cause mechanical obstruction leading to maternal and perinatal morbidity. Herein, we describe a patient with a vaginal leiomyoma misdiagnosed and operated as pelvic organ prolapse during the second trimester of pregnancy. She presented with a huge vaginal mass, foul-smelling vaginal discharge, difficulty in urination and defecation and septicemia. On examination, she had a large foul-smelling and necrotic mass protruding from the vagina with urethra lying outside the vagina, and sutures visible just above the mass on the anterior vaginal wall. She was admitted and a lower segment caesarian section was done followed by transvaginal resection of the mass, and cystoscopy was done. Vaginal leiomyomas require surgical removal and it is usually curative and recommended as the initial treatment of choice, especially when the tumour is large enough to cause a potential obstacle to normal labour. Key Words: Vaginal leiomyoma, Pelvic organ prolapse, Pregnancy.

Uterine Leiomyosarcoma with a Novel RAB2A-PLAG1 Gene Fusion

Abstract Introduction/Objective Uterine leiomyosarcoma is a malignant neoplasm with three distinct histologic subtypes: spindle, epithelioid, and myxoid. Distinguishing leiomyosarcoma from mesenchymal uterine neoplasms such as endometrial stromal sarcoma can be challenging as they may exhibit a similar histology and immunohistochemical profile. In such cases, molecular studies can help achieve the definitive diagnosis. It has been shown that ~25% of the uterine myxoid leiomyosarcomas harbor TRPS1-PLAG1 and RAD51B-PLAG1 gene fusions. To our knowledge, no uterine leiomyosarcoma with RAB2A-PLAG1 gene fusion has been previously reported in the English literature. Methods/Case Report An 80-year-old woman presented with abnormal uterine bleeding. A large necrotic uterine mass with no evident lymphadenopathy was detected on imaging. Endometrial curettage demonstrated a malignant mesenchymal neoplasm that was positive for CD10, desmin, and vimentin and lacked expression of SMA, SMMH, synaptophysin, and chromogranin. AE1/AE3 highlighted rare foci. The patient underwent total hysterectomy with bilateral salpingo-oophorectomy. The hysterectomy specimen was notable for a 10.8 cm rubbery focally calcified tan- white mass histologically composed of high-grade spindle and epithelioid cells with hypocellular and hyalinized areas. The mitotic activity was >25/10HPF. Immunohistochemically, the neoplastic cells expressed CD10, caldesmon, desmin (patchy), SMA (weak focal), and CAM5.2 (patchy) while they were negative for ER, PR, HMB45, AE1/AE3, WT1, cyclin D1, BCOR, myogenin, pan-NTRK, and ALK. P53 staining showed a wild type expression pattern. Histology and immunohistochemistry could not definitively differentiate between leiomyosarcoma and high grade endometrial stromal sarcoma. RNA sequencing identified a RAB2A-PLAG1 gene fusion. In light of the histology and ancillary studies, a diagnosis of leiomyosarcoma was rendered. Results (if a Case Study enter NA) NA Conclusion This is the first case of a uterine leiomyosarcoma with RAB2A-PLAG1 gene fusion. Pathologists should be cognizant of the extensive morphologic and immunophenotypic overlap between various uterine mesenchymal neoplasms. In challenging cases, molecular analysis is crucial in allowing the pathologist to arrive at the final diagnosis.

Variable presentations of radiation-associated angiosarcoma in patients treated for breast cancer

Introduction: Radiation-associated angiosarcoma is a rare and late complication of radiotherapy in breast cancer. Its incident is reported in the range of 0.03–0.3%. It carries a poor prognosis with a 5-year survival rate of 43–54%. Due to its non-specific presentation, diagnosis is often delayed. Case Report Case 1: A 60-year-old female presented six years after her breast cancer treatment with a history of bruising over the breast following trauma. Initial imaging and punch biopsy only showed inflammatory change. Her breast symptoms progressed and repeat biopsy revealed angiosarcoma. She was treated with chemotherapy followed by mastectomy. Case 2: A 60-year-old female treated for breast cancer, presented two and a half years later with subtle erythema over the breast. Imaging was normal. Punch biopsy showed angiosarcoma and she underwent mastectomy. Case 3: A 90-year-old female presented eight years after treatment for breast cancer. Her symptoms included blisters over the breast that bled at times. Punch biopsy showed angiosarcoma. There was rapid disease progression with convergence of the blisters into a large necrotic mass. She underwent a mastectomy for local control. The angiosarcoma recurred a year later and she died within three months of recurrence. Conclusion: Radiation-associated angiosarcoma is rare but carries a poor prognosis. Due to its non-specific presentation, diagnosis is often delayed. A high level of suspicion is needed in patients presenting with unusual breast symptoms and changes post-treatment.

Combined use of the GlideScope and flexible fiberoptic bronchoscope after several failed intubation attempts for a retrosternal thyroid mass: a case report

A GlideScope® and a flexible fiberoptic bronchoscope (FOB) were successfully used in combination, to intubate a 73-year-old female patient in which repeated previous intubation attempts with conventional laryngoscope had failed. GlideScope®, and FOB were used sequentially. She had been scheduled for an elective total thyroidectomy to remove a huge retrosternal goiter. A computed tomography (CT) scan revealed a large necrotic mass (measuring 10 x 16 cm) with heterogeneous enhancement involving the thyroid gland and extending to the anterior mediastinum, causing significant tracheal compression and left deviation. Endotracheal intubation was successfully performed while she was awake using a combination of GlideScope and FOB. The operation lasted for two hours and was uneventful. The patient was then transferred to the intensive care unit (ICU) and kept intubated, sedated, and mechanically ventilated overnight. She was awakened and extubated the following day with no airway complications.
 Key words: Adult; Failed intubation; Female; Fiberoptic bronchoscope; GlideScope; Humans; Intubation, Intratracheal; Retrosternal thyroid mass
 Citation: Abaalkhayl MS. Combined use of the GlideScope and flexible fiberoptic bronchoscope after several failed intubation attempts for a retrosternal thyroid mass: a case report. Anaesth. pain intensive care 2022;26(4):554-558; DOI: 10.35975/apic.v26i4.1965
 Received: July 05, 2022; Reviewed: July 14, 2022; Accepted: July 16, 2022

INTERESTING RADIOGRAPHIC FINDING OF SVC SYNDROME DUE TO PRIMARY LARGE B-CELL LYMPHOMA

INTERESTING RADIOGRAPHIC FINDING OF SVC SYNDROME DUE TO PRIMARY LARGE B-CELL LYMPHOMA

S2076 Not an Ordinary Intussusception: A Sigmoid Diverticula With an Extra-Colonic Mass

Introduction: Intussusception is defined as telescoping of a segment of the gastrointestinal tract into an adjacent lumen frequently found in childhood and are rarely found in adults. Etiologies of intussusception include benign or malignant tumors and idiopathic causes. Diverticula are rare findings for intussusception. This is a case of a patient found to have an intussusception due to a sigmoid diverticula and found to have a pelvic mass isolated outside of the diverticula. Case Description/Methods: An 80-year-old White female presented for worsening abdominal pain, nausea, and vomiting. History otherwise was unremarkable. CT of the abdomen and pelvis demonstrated a small bowel obstruction with a transition point in the left lower abdomen and a large 12.7x10.4 cm necrotic right pelvic mass involving the sigmoid colon (Figure 1A). The patient underwent a flexible sigmoidoscopy and endoscopic ultrasound. Endoscopic findings demonstrated an intusscepting large diverticulum at 40 cm and a peri-sigmoid heterogenous vascular mass on endosonography (Figure 1B and 1C). The patient underwent surgical resection of the mass, which upon resection demonstrated a mass arising from the sigmoid diverticulum isolated off the true colonic wall. Pathology demonstrated a 10.5 cm smooth muscle tumor concerning for a leiomyoma of deep soft tissue which did not involve the muscularis mucosae; staining was positive for muscle markers, no significant mitotic activity on Ki-67 mitotic proliferation markers, CD117, DOG1 were negative. Post operatively the patient did well. Discussion: Adult Intussusception are commonly due to colonic masses and are rarely associated with diverticula. This case highlights a patient who was found to have a sigmoid diverticulum as the etiology for intussusception and found to have an associated colonic mass as an extension from the diverticula isolated from the colonic wall. This diverticulum was likely a result of the chronic mass creating tethering of the bowel to the area. Masses arising from diverticula are rare and the majority of mases are found upon evaluation of other symptoms (pain and bleeding). In this patient, pathology demonstrated a leiomyoma which are rare benign tumors that are generally asymptomatic and found incidentally. During endoscopy, outside of a diverticula there was no obvious mass until endoscopic ultrasound was done. Therefore, this case highlights how critical it is for endoscopists to have a low threshold for further work up despite an unrevealing endoscopy.Figure 1.: A (Top Left), CT Scan of abdomen and pelvis with large necrotic mass. B (Top Right), Endoscopic view of diverticula. C (Bottom Left), Endoscopic ultrasound of necrotic mass.

S2896 A Rare Case of Necrotic Testicular Seminoma Presenting as a Large Retroperitoneal Mass Diagnosed With EUS and Laparotomy

Introduction: Retroperitoneal seminomas are a rare form of cancer that accounts for approximately 2% of all seminomas. Seminomas commonly present with fever, weight loss, abdominal or back pain, and a painless testicular nodule. The differential diagnosis for a retroperitoneal mass is broad, including abscess, lymphoma, sarcoma, metastatic lymph nodes, and extragonadal tumors. The pathogenesis of germ cell tumors is unclear, but the failure of primordial germ cell migration during embryonal development has been suggested. We report an atypical case of a large necrotic retroperitoneal mass diagnosed as a primary retroperitoneal seminoma after EUS and laparotomy. Case Description/Methods: A 33-year-old male with no medical history presented to the ED for new-onset flank pain and night sweats. On admission, the patient was febrile and tachycardic. The testicular exam and US of the testes were normal. CT abdomen and pelvis revealed a well-circumscribed, round mass posterior to the pancreatic head. EUS with FNA was performed, noting a round heterogenous 7.1cm x 6.2cm mass with well-defined borders and a necrotic center located posterior to the pancreatic head. An intact interface was seen between the mass and the duodenum, IVC, pancreas, and right kidney, suggesting a lack of invasion. The FNA yielded 0.5ml of blood-tinged, thick, and purulent fluid with cytology significant for malignant tumors with extensive necrosis. An exploratory laparotomy with excisional biopsy established the diagnosis of extragonadal germ cell tumor with extensive necrosis, consistent with seminoma. The patient completed four cycles of Etoposide followed by a right-sided orchiectomy for residual lesions found on the right testicle. A post-surgical PET scan did not demonstrate recurrence. Discussion: Primary retroperitoneal seminoma is a rare entity that must be considered when evaluating a retroperitoneal mass. GCTs are the most common neoplasm arising from the testes, with < 2% originating in other locations, termed primary extragonadal GCTs. Studies suggest the remission rate with chemotherapy and resection of residual masses was 92%, and the five-year overall survival rate was 88% for primary retroperitoneal tumors. Early identification of primary retroperitoneal seminomas may expand therapeutic possibilities and help improve outcomes for patients with a favorable prognosis. EUS-FNA was attempted in this case to obtain the diagnosis; however, an excisional biopsy was required to confirm the diagnosis.

S2096 Development of Achalasia in Chronic Intestinal Pseudo-Obstruction (CIPO)

Introduction: Neuroendocrine carcinoma (NEC) of the esophagus is a rare malignancy and represents approximately 0.4-2% of all esophageal cancer. Histologically, two types of NEC of the esophagus have been described: small cell NEC and large cell NEC. Small cell NEC is the more common type, comprising almost 95% of all NEC of the esophagus. Case Description/Methods: We present a 52-year-old Hispanic male with 30 pack year smoking history and chronic alcohol abuse presented with sore throat and worsening dysphagia to both liquids and solids for 3 weeks. Physical examination revealed anterior cervical swelling which was tender to palpation. The oropharynx looked moist with mild tonsillar erythema. Complete blood chemistry showed mild leucocytosis. COVID-19 PCR and streptococcal Ag test came back negative. The computer tomography (CT) of the neck with contrast revealed an esophageal mass at the level of T1- T2 with paratracheal extension associated with enlarged paratracheal lymph nodes. The endoscopic evaluation revealed a large bloody, exophytic, friable, and necrotic mass in the oropharynx which was partially obstructing the airway. A large, circumferential, ulcerating and partially obstructing mass was found in the proximal esophagus. Immunohistochemistry was positive for synaptophysin and chromogranin and negative for CK5/6 and p63. KI-67 was greater than 90%. The biopsies were consistent with a neuroendocrine malignancy. The decision was made to start him on cisplatin, etoposide with the addition of Durvalumab and radiation therapy. Discussion: Neuroendocrine tumors of the esophagus have an aggressive nature, with no current standard treatment guidelines. From the limited literature available, The treatment strategy is dictated by the extent of disease with surgery being the preference in limited disease and a combination of chemotherapy, immunotherapy, and radiation therapy for the extended disease. The most commonly used chemotherapy is cisplatin with etoposide and the addition of immunotherapy, durvalumab. An alternative chemotherapy regimen that may be used if patients exhibit intolerance to cisplatin includes carboplatin with etoposide and the addition of durvalumab. In our case, we used cisplatin, Etoposide, Durvalumab and radiotherapy which is protocol for small cell carcinoma of lung.Although many recent advances were made in NEC management, there are no definitive guidelines. As of now, despite all aggressive measures, NEC of the esophagus has a poor overall prognosis.

Imaging findings of granulocyte colony-stimulating factor-producing tumors: a case series and review of the literature

Granulocyte colony-stimulating factor (G-CSF)-producing tumors have an aggressive clinical course. Here, we report five cases of G-CSF-producing tumors and review the literature, focusing on imaging findings related to tumor-produced G-CSF. In addition to our cases, we identified 30 previous reports of G-CSF-producing tumors on which 18F-fluorodeoxyglucose positron emission tomography (FDG-PET)/CT, bone scintigraphy, or evaluation of bone marrow MR findings was performed. White blood cell count, serum C-reactive protein, and serum interleukin-6 were elevated in all cases for which these parameters were measured. G-CSF-producing tumors presented large necrotic masses (mean diameter 83.2 mm, range 17–195 mm) with marked FDG uptake (mean maximum standardized uptake value: 20.09). Diffuse FDG uptake into the bone marrow was shown in 28 of the 31 cases in which FDG-PET/CT was performed. The signal intensity of bone marrow suggested marrow reconversion in all seven MRI-assessable cases. Bone scintigraphy demonstrated no significant uptake, except in two cases with bone metastases. Splenic FDG uptake was increased in 8 of 10 cases in which it was evaluated. These imaging findings may reflect the effects of tumor-produced G-CSF. The presence of G-CSF-producing tumors should be considered in patients with cancer who show these imaging findings and marked inflammatory features of unknown origin.

Giant Adrenal Pheochromocytoma Presenting With Stroke

Background: Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumours arising from the adrenal medulla or from paravertebral ganglia of the sympathetic chain(1). The tumours commonly produce one or more catecholamines: epinephrine, norepinephrine, and dopamine leading to a classic triad of presentation with pounding headache, profuse sweating, and palpitations occurs in spells; However, one in 10 patients may be completely asymptomatic and the diagnosis of PPGL is frequently missed(2). Care Report: A 29 years old African female presented with one-month history of throbbing headache, palpitations, profuse sweating, and unintentional weight loss. Her previous medical history and family history was unremarkable. She was found to have accelerated hypertension and a small lacunar infarct and some suspected subarachnoid hemorrhage on MRI head but was clinically silent. Investigation for secondary causes of hypertension revealed high metanephrine in urine and her imaging showed giant adrenal mass suggestive of pheochromocytoma.CT Abdomen of large left adrenal necrotic solid mass lesion with heterogenous enhancement of its solid components measuring 6.5 x 6.1 x 7 cm in maximum AP, TR and CC dimensions respectively.She was scheduled for open resection and left adrenalectomy after approximatly10 day and was treated with high-sodium diet, alpha blockers, and beta blockers perioperatively. Histopathology examination revealed Pheochromocytoma measuring 8 x 7 x 5 cm with diffuse growth >10% of tumor volume with central necrosis; PASS =4, which has malignant potential. She recovered well post operatively and required no further antihypertensive therapy or hormonal replacement, follow up hormonal profile and imaging with Iodine-123 metaiodobenzylguanidine (MIBG) scintigraphy were negative for remnants or metastasis,further evaluation with gallium DOTATATE PET CT and molecular genetic testing was considered, but it was not available. Conclusion: Pheochromocytoma is a rare cause of secondary hypertension with a variable clinical presentation. Episodes of tumoral catecholamine release, are thought to be responsible for the high prevalence of cardiovascular emergencies, such as myocardial infarction, heart failure, and stroke as a complication of hypertensive crisis. Timely diagnosis and treatment are crucial to prevent life-threatening complications.

Inflammatory pseudotumor of fallopian tube: A case report with review of literature

Inflammatory pseudotumor of fallopian tube is an uncommon benign lesion. We report a case of an inflammatory pseudotumor of left fallopian tube in a 29 year-old woman. She presented with pain in lower abdomen, urinary frequency and dysuria. Contrast enhanced computerized tomography revealed a large necrotic mass in left adnexa abutting superior wall of urinary bladder and lower anterior wall of uterus. Left ovary was seen adherent to the posterior part of the mass. Serum CA-125 was within normal limit. The patient underwent tru-cut biopsy and subsequent excision of left adnexal mass. Histopathology revealed an inflammatory pseudotumor. We reviewed the clinical, imaging and pathological features of the inflammatory pseudotumor of fallopian tube and discussed its differential diagnosis. Keywords: Inflammatory, Pseudotumour, Fallopian Tube, Diagnosis, and Immunohistochemistry.

A MISCARRIAGE THAT METASTASIZES: A CASE OF CHORIOCARCINOMA IN THE ICU

SESSION TITLE: Fellows Critical Care Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Gestational trophoblastic neoplasia (GTN) is a rare oncologic emergency that requires rapid treatment. Complications can be numerous based on sites of metastasis. We present a young patient with significant disease burden and numerous complications requiring multiple aspects of critical care. CASE PRESENTATION: A 19-year-old female presented to the emergency department with 3 weeks of cough and dyspnea. 4 months prior she had a spontaneous abortion with 4 weeks of abnormal uterine bleeding. At an outside hospital she was noted to have multiple metastatic lesions with a β-HCG of 1.6 million and was transferred to our hospital for evaluation by gynecologic oncology. On presentation she was tachycardic and noted to have abdominal distension. Labwork showed sodium 131, hemoglobin 8.7, and β -HCG >850,000. Outside hospital CT chest/abdomen/pelvis showed innumerable pulmonary lesions, large necrotic mediastinal mass, pericardial effusion without tamponade, metastases in the liver, spleen, kidneys, and massive ovaries. CT of the head with lesions in the right parietal and frontal lobes with edema. She was admitted to gynecologic oncology and started chemotherapy and whole brain radiation. On hospital day 4 she was intubated for altered mental status and hypoxia. Prior to intubation she reported a headache, and afterwards had fixed and dilated pupils. CT head showed hemorrhage of brain lesions and she had emergent hemicraniectomy. Post-procedure she required multiple vasopressors; bedside echocardiogram showed known pericardial effusion without tamponade. She developed significant metabolic acidosis with renal failure and started urgent renal replacement therapy. On ICU day 4 her left hand was cool with nonpalpable pulses. CT angiography showed radial artery occlusion and she underwent decompressive surgery and radial artery dissection by surgery. After surgery, she remained intubated for an additional 7 days before being extubated. She was discharged to rehab after 49 days of hospitalization, eventually completing chemotherapy as an outpatient. DISCUSSION: GTN is a rare malignancy with the most common subtype being choriocarcinoma, which is known for rapid spread and progression. It is hallmarked by extremely elevated beta-HCG with diffuse metastatic disease. Symptoms of GTN are dependent on organs affected. It is considered an oncologic emergency requiring urgent treatment to avoid permanent injury. Classically GTN was a terminal diagnosis but is now considered a highly curable. Our patient had multiple sequelae from the metastatic spread of her disease but was successfully extubated and completed chemotherapy. CONCLUSIONS: This case details the course of a patient with a primary diagnosis that is rarely encountered in critical care medicine. She had multiple complications that required aggressive critical care in a timely fashion and was able to be discharged home with an overall good outcome. Reference #1: Smith, H.O. (2003) 'Gestational trophoblastic disease epidemiology and trends', Clinical Obstetrics and Gynecology, 46(3), pp. 541. Reference #2: Bakri, Y.N., Berkowitz, R.S., Khan, J., Goldstein, D.P., von Sinner, W., Jabbar, F.A. (1994) 'Pulmonary metastases of gestational trophoblastic tumor. Risk factors for early respiratory failure', Journal of Reproductive Medicine, 39(3), pp. 175. Reference #3: Onal, O., Salman E., Yetisir, F., Kilic, M. (2015) 'Hand ischaemia after radial artery cannulation', BMJ Case Report, bcr2015211145 DISCLOSURES: No relevant relationships by Morium Akthar, source=Web Response No relevant relationships by Alfred Lardizabal, source=Web Response No relevant relationships by Timothy Roedder, source=Web Response

PRIMARY MEDIASTINAL CHORIOCARCINOMA (PMC) IN A YOUNG WOMAN WITH HEMOPTYSIS AND A LUNG MASS

SESSION TITLE: Medical Student/Resident Lung Cancer Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Primary mediastinal choriocarcinoma (PMC) is an extra gonadal, non-gestational, angioinvasive rare germ cell neoplasm that mostly arises along the midline structures. PMC contains syncytiotrophoblastic cells which secrete human chorionic gonadotropin hormone (hCG). An elevated hCG is considered an important serologic tumor marker in the diagnosis of mediastinal germ cell tumor, in the absence of gestation and with no previous gynecologic malignancy. CASE PRESENTATION: A 39 year-old woman with no past medical history was admitted to the hospital with severe right sided chest pain associated with hemoptysis that started 2 weeks prior to admission. Physical exam revealed decreased breath sounds on the right hemithorax with dullness to percussion. Bloodwork was significant for β-HCG of 6,566 mlU/ml with negative alpha fetoprotein, and normal lactate dehydrogenase. A CT scan of the chest revealed a large necrotic solid mass with numerus enlarged vessels located in the anterior mediastinum (Figure 1). PET scan was negative for any metastatic lesions. Transvaginal ultrasound was negative for intrauterine or ectopic pregnancy. Transthoracic core biopsy showed poorly differentiated choriocarcinoma. Immunohistochemistry staining was positive for β-HCG, placental alkaline phosphatase, and pancytokeratin AE1/AE3 (Figure 2). The tumor was not amenable to surgical resection due to its large size and high vascularity. The patient was started on cisplatin and etoposide followed by etoposide, MTX, Act-D, cyclophosphamide, and vincristine (EMA-CO). After the first course of chemotherapy, the levels of β-HCG markedly decreased. DISCUSSION: PMC is a rare malignancy in both genders, more prevalent in men than in women. The natural process of PMC has been poorly understood. Possible theories include: (1) trophoblastic embolus related to a gestational event after a long period of latency; (2) metastases from a primary gonadal choriocarcinoma; (3) retained primordial germ cells that migrated during embryogenesis. An extensive workup is required to exclude other β-HCG-positive tumors from PMC, such as primary poorly differentiated non-small cell carcinoma of the lung, and gestational metastatic choriocarcinoma. Our patient was found to have a large highly vascular mass in the presence of elevated β-HCG, and absence of primary genital tumor. The histological features showed extensive areas of hemorrhage and necrosis and biphasic cellular population including cytotrophoblastic and syncytiotrophoblastic cells. In contrast to gestational choriocarcinoma, PMC is rapidly progressive and fatal in the great majority of cases with a dismal 5 years survival of less than 5%. CONCLUSIONS: Due to the rarity of PMC, currently there is no consensus regarding treatment guidelines. Thus, case reports such as this contribute to the scant literature to raise the profile of this rare and devastating disease. Reference #1: Ruan Z, Wang S, Wang Z, Jing Y. A rare case of bilateral massive hemothorax from spontaneous rupture of a primary mediastinal mixed germ cell tumor. Ann Thorac Surg. 2012;93:664–666 Reference #2: Bachmann J, Ernestus K, Werner T, et al. Detection of primary choriocarcinoma in the mediastinum by F-18 FDG positron emission tomography. Clin Nucl Med. 2007;32:663–665. Reference #3: Serno J, Zeppernick F, Jakel J, Primary pulmonary choriocarcinoma; cas report and the review of the literature, Gynecologic nad obstetric investigation, vol. 74, no. 2, pp. 171-176,2012 DISCLOSURES: No relevant relationships by Marwah Abduljabbar, source=Web Response No relevant relationships by Sara Mehrsefat, source=Web Response No relevant relationships by jorge otoya, source=Web Response No relevant relationships by Noman Subhani, source=Web Response no disclosure on file for Maria Wallis-Crespo

Imaging features and clinical course of undifferentiated round cell sarcomas with CIC-DUX4 and BCOR-CCNB3 translocations.

To describe the pre-treatment imaging features and clinical course of undifferentiated round cell sarcomas with CIC-DUX4 and BCOR-CCNB3 translocations. In this retrospective study, several pre-treatment imaging features (tumor location, size, enhancement pattern, necrosis, flow voids, calcification, and FDG avidity) and the clinical course of patients were evaluated. In 12 patients with CIC-DUX4 sarcomas (median age, 24 years; range, 12-75), sarcomas were located in the soft tissue (n = 10), bone (n = 1), and lungs (n = 1). On MRI, all 10 CIC-DUX4 sarcomas presented as a large necrotic mass (mean size 6.7 cm, range 2.3-11.3) with 100% demonstrating contrast enhancement, 60% showing flow voids, and 20% demonstrating fluid-fluid levels. On PET, the mean SUVmax was 13.2 (range, 8.5-18.1). Among 12 patients with follow-up, 3 died within a year of diagnosis. The most common site of metastases was the lungs (5/12). In 5 patients with BCOR-CCNB3 sarcomas (median age, 14 years; range, 2-17), sarcomas were located in the spine (n = 2), femur (n = 1), tibia (n = 1), and pelvis (n = 1). On radiograph or CT, 2 were lytic, 3 were sclerotic. Soft tissue calcifications occurred in 40% of BCOR-CCNB3 sarcomas. On MRI, all 3 BCOR-CCNB3 tumors enhanced with 33% demonstrating flow voids and 66% exhibiting necrosis. On PET, the mean SUVmax was 6.3 (range 5.7-6.9). CIC-DUX4 sarcomas often present as necrotic and hypermetabolic soft tissue masses while sarcomas with BCOR-CCNB3 translocations are vascular bone lesions with necrosis at imaging. CIC-DUX4 sarcomas are clinically more aggressive than BCOR-CCNB3 sarcomas.

59. Dedifferentiated solitary fibrous tumour arising from the bladder: A case report

Background: Solitary fibrous tumour (SFT) is a rare mesenchymal tumour of fibroblastic differentiation. They most commonly arise in the pleura, but have a wide anatomical distribution. Conventional SFTs are circumscribed, spindle cell neoplasms with characteristic histomorphology including patternless architecture, dense collagen and prominent haemangiopericytoma-like ‘staghorn’ vessels. Conventional SFTs express CD34, Bcl2 and STAT6, but are negative for S100, keratin and desmin. Molecular profiling has shown consistent 12q chromosomal abnormality resulting in NAB2-STAT6 fusion. The NAB2-STAT6 gene fusion product is reliably identified on STAT6 immunohistochemistry, which has proven both a highly sensitive and specific marker for SFTs. Most SFTs are clinically benign, however up to 15% may show features of malignancy including increased mitoses (>4 per 10 HPF), necrosis and infiltrative margins. In addition, areas of dedifferentiation may occur, characterised by sharply demarcated areas of hypercellularity and pleomorphism with loss of morphological features of conventional SFT. Dedifferentiated areas may lose the typical immunohistochemical profile of conventional SFT, including loss of CD34 and STAT6. Aim: To report a case of dedifferentiated SFT in the bladder and review the current literature. Method: A 39-year-old male presented with one month history of urinary frequency and abdominal pain. CT and MRI showed a large heterogenous necrotic mass in the posterior bladder wall. An en bloc tumour resection and cystoprostatectomy was performed. Results: Macroscopic examination showed a 15 cm circumscribed tumour arising from the superior bladder. Microscopy revealed a spindle cell tumour arising from the bladder muscularis propria with areas of conventional SFT adjacent to vast areas of dedifferentiation with malignant features including hypercellularity, pleomorphism, increased mitoses (40 per 10 HPF) and necrosis. Immunohistochemistry showed positive staining for STAT6, CD34 and Bcl2 in both conventional and dedifferentiated areas. Conclusion: This is a unique case of a dedifferentiated SFT of the bladder in a young patient.

2542 Emerging Use of Hemospray in Lower Gastrointestinal Bleeding: Primary Mucosal Melanoma of the Jejunum

INTRODUCTION: Small bowel (SB) primary melanomas (PM) are extremely rare; around 30 cases are reported to date. Diagnosis is challenging due to difficult endoscopic access and dealing with its complications such as bleeding is even more difficult. We describe in this report a case of jejunal primary melanoma presenting with lower gastrointestinal (GI) bleeding controlled with Hemospray. CASE DESCRIPTION/METHODS: A 70-year-old man with coronary artery disease on aspirin presented with symptomatic anemia. He endorsed recurrent melena associated with weight loss. Vital signs were stable. Physical exam remarkable for melena on rectal exam. Computed tomography scan of the abdomen revealed a jejunal mass. The patient underwent a push enteroscopy, which showed a large ulcerated necrotic mass in the mid-jejunum that was actively bleeding. The entire mass was oozing from multiple foci with a large surface area. It was invading the jejunal wall with larger vessels exposed. Hemospray was used to achieve hemostasis. Pathology revealed malignant melanoma; a thorough skin and eye exam was negative for PM. The patient denied any history of excision of pigmented skin lesions. The final diagnosis was primary melanoma of the jejunum. DISCUSSION: Malignant melanomas are mostly cutaneous. Only 3-4% originate in mucosal membranes as primaries. The diagnosis is challenging due to nonspecific manifestations and difficult pathologic access. Radiological studies are the first diagnostic approach. Balloon enteroscopy allows histopathological diagnosis and management of complications such as bleeding. In our case, the jejunal tumor was ulcerated with an extensive surface area and multiple foci of actively bleeding vessels. Due to the abundant lymphovascular supply of the intestinal mucosa, SB melanoma is a highly vascularized tumor. Hemospray was the preferred initial modality in this particular scenario and was successfully used for hemostasis. It is an inorganic powder that provides hemostasis by acting as mechanical tamponade and increasing the local concentration of clotting factors. It is easy to use with an excellent safety profile, does not require precise targeting, and is efficacious in large surfaces. Mucosal tissues do not absorb it; thus, it has no risk of systemic toxicity. It is FDA approved for hemostasis of non-variceal GI bleeding. It is contraindicated in varices and fistulas. It was also used in cancer-related bleeding with promising results. It can be applied repeatedly as a bridge to other definitive modalities.

Case-based discussion: a case of misdiagnosis of primary lung malignancy

A 69-year-old man was initially referred to urology outpatient clinic for investigation of frank haematuria. His medical history included transurethral resection of prostate in 2008, autoimmune hepatitis, hyperlipidaemia and hypertension....