Gallbladder sarcoma: a clinicopathological study of seven cases from the UK and Austria with emphasis on morphological subtypes.

BACKGROUND: Primary sarcoma of the gallbladder (PGBS) is rare, with only 40 cases reported in the literature. Most of these have been diagnosed as leiomyosarcoma. We aimed to evaluate the histological features of a case series of this rare tumor and correlate these with clinical features. DESIGN: Cases recorded as "gallbladder sarcoma" from different institutes were reviewed and the clinicopathological features of these cases were recorded. Only primary gallbladder wall mesenchymal tumors were included. Epithelial tumors, mixed tumors (carcinosarcoma or sarcomatoid carcinoma), and tumors extending into the gallbladder from the abdomen or sarcoma with other known primaries were specifically excluded. RESULT: PGBS occurred in one male and six females with a median age of 70 (range 64-82) years. Patients presented with acute or chronic cholecystitis, abdominal pain, weight loss, and pruritus. They were generally found to have elevated alkaline phosphatase and bilirubin, and leukocytosis. Tumors ranged from 1.1 to 4 cm with a median size of 3 cm. Most PGBS arose in the body but one arose in the fundus. All tumors were associated with ulcerated mucosa. Based on morphological and immunohistochemical features of the PGBS, there were three myxofibrosarcomas (malignant fibrous histiocytoma, MFH, storiform pleomorphic), one leiomyosarcoma (LMS), one angiosarcoma (AS), and two liposarcomas (LS). All patients received cholecystectomy and three received adjuvant chemotherapy. Follow-up revealed that six patients died of the disease 6 weeks to 2 years after diagnosis and one died of unrelated causes. CONCLUSION: PGBS are rare and mainly occur in the gallbladder body in middle-aged females. They generally present with acute cholecystitis and have a very poor prognosis. A variety of sarcoma types are found with MFH being the predominant variant.

Superficial acral fibromyxoma: a clinicopathological study of new 41 cases from the U.K.: should myxoma (NOS) and fibroma (NOS) continue as part of 21st-century reporting?

BACKGROUND: Superficial acral fibromyxoma (SAF) remains poorly recognized by general pathologists and dermatopathologists, partly attributable to its relatively uncommon occurrence and recent documentation. OBJECTIVES: To examine a series of SAF and document the U.K. experience with this new entity. METHODS: We reviewed 771 tumours reported between 1970 and 2006 in seven different U.K. hospitals and coded as myxoma, not otherwise specified (NOS), fibroma (NOS) or dermatofibroma (NOS) presenting at acral sites. Forty-one cases of SAF were studied. RESULTS: The patients comprised 27 men and 14 women, age range 19-91 years (mean 50, median 47), presenting with a solitary mass or nodule with a mean size of 1.92 cm. The common clinical sites were the toes (n=29) and fingers (n=11) as well as the palm (n=1), with more than 75% of cases close to or involving the nail bed. All cases presented with a painless mass except for four cases where pain was the presenting complaint. A history of trauma was reported in only two cases. Histologically, all cases presented as proliferation of spindle-shaped and/or stellate cells with a storiform and fascicular pattern embedded in a fibromyxoid/collagenous stroma with conspicuous mast cells. Multinucleated cells were observed (n=22), increased number of blood vessels in the stroma and extravasation of red blood cells (n=4). The characteristic immunophenotype was CD34+, CD99+/-, epithelial membrane antigen+ focally/-, S100-, desmin-, smooth muscle actin-, HMB45- and cytokeratin-. CONCLUSIONS: We describe a large series of 41 cases of SAF showing that it is a distinct entity with typical clinical, histological and immunohistochemical features. Follow-up was available only in 12 patients, precluding a firm comment on recurrence. However, complete excision and follow-up review is recommended.