Solitary Fibrous Tumor of the Adrenal Gland.

OBJECTIVES: Solitary fibrous tumor (SFT) is a mesenchymal neoplasm that can arise at various anatomic locations. It is characterized by inv12(q13q13)-derived NAB2::STAT6 fusion, resulting in the nuclear expression of STAT6. Primary SFT of the adrenal gland is rare. We launched a multi-institutional collaboration to comprehend the overarching demographics, clinical and follow-up, macroscopic, microscopic, IHC, and FISH features of 9 patients with SFT of the adrenal gland. METHODS: We added a series of 9 patients to the collection of adrenal SFTs where the clinicopathologic parameters, including clinical presentation, imaging, histopathology, IHC, molecular profiles, and management and follow-up data, were analyzed comprehensively. A modified 4-variable risk stratification model, including age, tumor size, and necrosis, was applied. RESULTS: Our series consisted of 6 male and 3 female patients, ranging in age from 19 to 64 years (mean, 49.3 years). Abdominal pain (4) and fever with abdominal pain (1) were the presenting symptoms in 5 patients. In the remaining 4 patients, the tumors were detected by abdominal imaging for hypertension and diabetes. The size of the tumor ranged from 2 cm to 10.5 cm in maximum dimension. All tumors exhibited the morphology of a spindle cell SFT with a patternless architecture; 3 had a focal storiform arrangement. STAT6 positivity was observed in all tumors, and 7 were positive for CD34. Surgical resection was the primary modality of treatment. No adjuvant therapy was administered. Follow-up ranging from 7 months to 23 months was available for 7 patients. All were alive without disease recurrence or metastasis. Risk stratification placed 8 (88.9%) patients into a low-risk category and 1 into an intermediate-risk category. CONCLUSIONS: This series is the largest of adrenal SFTs to date. These tumors of the adrenal gland are predominantly spindle cell neoplasms with indolent behavior, with a wide age distribution and a slight male preponderance. Combining our cohort with the previously published cases, the majority of tumors fall into the low-risk category for the propensity to develop metastases. Owing to the rarity and age distribution associated with these tumors, the differential diagnosis is wide and requires a systematic approach for ruling out key differential diagnoses aided by STAT6 IHC.

Solitary fibrous tumour of lacrimal sac masquerading as lacrimal sac mucocele: a diagnostic and surgical dilemma.

Swellings in medial canthal area in eye most often involve the lacrimal sac, the commonest aetiology being chronic dacryocystitis. Very rarely, there is a deviation in the routine diagnosis when the pathology comes out to be a lacrimal sac tumour. We report the case of a young man who presented with a medial canthal mass not extending above the medial canthal ligament. The clinical presentation was typical for a lacrimal sac mucocele, however on histopathological evaluation was diagnosed as a rare case of solitary fibrous tumour of lacrimal sac. We recommend radiological investigation in medial canthal masses where there is negative regurgitation on pressure over lacrimal sac area to avoid misdiagnoses.

Portal Vein Thrombosis: A Rare Finding in a Noncirrhotic Patient.

Portal vein thrombosis (PVT) is a rare cause of abdominal pain, typically associated with cirrhosis or thrombophilia. A 18-year-old female presented with abdominal pain for 10 weeks. The diagnosis was confirmed with contrast-enhanced computed tomography (CECT) abdomen after an ultrasound showed dilated and obstructed portal vein. This unexpected finding prompted investigation for intrinsic hepatic disease and potential hypercoagulable disorders. Laboratory analysis revealed an elevated serum homocysteine level, an identified risk factor for venous thrombosis. Current literature describes the following factors as indications for anticoagulation: acute thrombus, lack of cavernous transformation, absence of esophageal varices and mesenteric venous thrombosis. PVT is an uncommon cause of abdominal pain, and in the absence of hepatic disease should raise the index of suspicion for an underlying thrombophilia. How to cite this article: Sahoo AK, Rauta S, Mohapatra SC. Portal Vein Thrombosis: A Rare Finding in a Noncirrhotic Patient. Euroasian J Hepato-Gastroenterol 2014;4(1):55-57.