Ewing sarcoma is a malignant tumor that more commonly affects the long bones. Primary Ewing sarcoma of the adrenal gland is a rare diagnosis. We report an unusual case of primary Ewing sarcoma of the adrenal gland in a 34-year-old man who initially presented with abdominal as well as flank pain and abdominal mass. Computed tomography and magnetic resonance imaging showed a heterogeneous, hemorrhagic right adrenal mass with inferior vena cava extension and thrombosis. Thus, a major open surgery including right adrenalectomy, right nephrectomy, inferior vena cava thrombectomy with resection, and a retroperitoneal lymph node dissection was necessitated. Early recognition with radiological imaging and treatment of extraosseous Ewing sarcoma of the adrenal gland with a multimodality approach reduces morbidity and mortality.
Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor most commonly seen in young adults and children. We report a case with morphology and immunohistochemistry supporting the diagnosis of synovial sarcoma. On core biopsy, the tumor had spindle cell and epithelioid morphology with a myxoid background. Staining for transducin-like enhancer of split 1 and CD99 were positive; however, subsequent fluorescence in situ hybridization for SYT (SS18, nBAF chromatin remodeling complex subunit) break apart returned negative. Further study showed fluorescence in situ hybridization for EWSR1 (EWS RNA binding protein 1) gene rearrangement, supporting the diagnosis of AFH. The resected specimen showed a predominant spindle cell proliferation along with histiocytoid areas supporting a diagnosis of AFH. This case report highlights the fact that synovial sarcoma and AFH can overlap morphologically and immunohistochemically. When approaching a biopsy specimen with spindle cell morphology, and transducin-like enhancer of split 1, CD99, and epithelial membrane antigen positivity it is important to include AFH in the differential diagnosis.
INTRODUCTION: Malakoplakia is a rare chronic granulomatous disease that may affect many organs. The gastrointestinal tract is the second most common site of involvement (after the urinary tract). The most common sites of colonic involvement are the rectum, sigmoid, and right colon, in descending order of frequency. The pathogenesis of malakoplakia is unknown. The treatment of malakoplakia ranges from antibiotic therapy to surgical resection. Here we describe a case of a 33-year-old man who presented with hematochezia and was noted to have malakoplakia on rectal mass biopsy during colonoscopy. CASE DESCRIPTION/METHODS: A 33-year-old man presented to our clinic due recurrent episodes of painless hematochezia in the setting of warfarin for recent lower extremity deep venous thrombosis. He is a resident of a nursing home due to a complex neurosurgical history which includes resection of a right temporal glioblastoma multiforme tumor followed by chemo and radiation therapy. He also had subsequent craniotomy and ventriculo-peritoneal shunt placement and used oral dexamethasone for recurrent episodes of cerebral edema related to residual brain tumor. Despite stopping warfarin he continued to have rectal bleeding. Colonoscopy showed a frond-like/villous, fungating and polypoid non-obstructing circumferential mass in the rectum. Biopsies showed malakoplakia. After multidisciplinary discussions, a decision was made to continue dexamethasone to minimize cerebral edema and bowel resection was declined by patient’s parents. We opted to use a prolonged course of Ciprofloxacin, however, the patient developed severe urosepsis and unfortunately died. DISCUSSION: First reported by Michaelis and Gutmann in 1902, malakoplakia is derived from the Greek “malakos” (soft) and “plakos” (plaque) and reflects its usual endoscopic appearance as a friable, yellow mucosal lesion. Coliform bacteria in macrophage cytoplasm (von Hansemann histiocytes), and laminated intracytoplasmic inclusion bodies (Michaelis-Gutmann bodies) are characteristic. A decrease in macrophage cyclic GMP activity with resultant impaired bactericidal activity is hypothesized to be the cause of malakoplakia. Macrophage abnormalities and clinical symptoms resolve after discontinuation of glucocorticoids. Antibiotics such as ciprofloxacin have been successful treatments in few case reports. Unfortunately our patient did not have the opportunity to utilize any of these antibiotics prior to his passing.
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