Lymphangioma circumscriptum: clinicopathological spectrum of 29 cases

To describe the clinicopathological spectrum of Lymphangioma Circumscriptum [LC]. Observational case series. Department of Pathology and Microbiology, AKUH, Karachi, from 2002 to 2012. All reported cases of LC were retrieved from medical record. Clinical and pathological features were noted. Frequency percentages were determined. There were 29 cases of LC predominantly males [62%]. The mean age was 27.17 +/- 15.5 years. The commonest sites was anal/perianal region [24%] followed by extremities [17%] and tongue, [14%]. Vulval LC was seen in 3 patients. Two cases were described on scrotum. The lesions were most commonly suspected as viral warts, mole or polyp [in anal Region]. Vesicles with erosions and bleeding and localized growth were the usual clinical presentations. Four of the patients presented with swelling since birth. All were treated with surgical excision. Microscopic examination revealed acanthotic squamous epithelium with papillomatosis. The subepithelial region had collections of lymphatic channels composed of ectatic dilated vessels with serum and inflammatory cells in their lumina. The lymphatic channels were seen in deeper layers along with lymphocytic aggregates. Lymphangioma circumscriptum is a malformation of abnormal lymphatic channels with feeding cisterns in subcutaneous tissue. It is a benign lesion usually occurring in anal/perianal region and confused with warts. Surgical excision is preferred mode of treatment

Sarcomatoid carcinoma of prostate involving the whole lower urinary tract and rectum

Sarcomatoid carcinoma of prostate is an extremely rare but aggressive neoplasm. It is generally associated with a poor prognosis. About 100 cases have so far been reported in the English literature. We report the case of a 64-year-old male with a very rapidly progressive disease that ultimately involved the whole lower urinary tract and rectum. The management of this case along with etio-pathogenesis and literature review is discussed

Juvenile dermatomyositis

Juvenile dermatomyositis [JDM] is an important subtype of dermatomyositis characterized by inflammation of muscle, skin and gastrointestinal tract. A 14-year-old girl, with a history of fever, joint pain, easy fatigability and a rash since the age of 3 years is described. Physical examination, laboratory evaluation, electromyography [EMG] and muscle biopsy were suggestive of a chronic inflammatory process involving the muscles, most likely dermatomyositis. The report highlights the importance of a muscle biopsy as the gold standard for diagnosing dermatomyositis

Hepatic angiosarcoma with metastasis to small intestine

Hepatic angiosarcomas are rare tumours with poor prognosis, with patients usually dying within 6 months. Metastases mainly occur in lymph nodes, spleen, lungs, bones and adrenals. Metastasis to small intestine is even rarer. Similarly, primary or metastatic angiosarcomas in small intestine are extremely rare, often present with recurrent gastrointestinal bleeding and anemia, and have an extremely poor prognosis. Both primary or metastatic intestinal angiosarcomas may exhibit epithelioid morphology. It may be very difficult to differentiate between primary and secondary cases in intestine and especially when the tumour exhibits epithelioid morphology

Metachronous primary cancers of colon and stomach

Synchronous or metachronous occurrence of esophageal, gastric and colonic cancers is a very rare occurrence, although there are several case reports in literature. A case of a 41 years old man with metachronous cancers of colon and stomach is reported

Minimally invasive papillary carcinoma of the extrahepatic bile ducts

Invasive papillary carcinomas of the Extrahepatic Bile Ducts [EBD] are uncommon [4-5%]. The course is less aggressive than conventional adenocarcinomas of the extrahepatic bile ducts. Non-invasive and minimally invasive papillary carcinomas are even rarer, behave as in-situ carcinomas and are associated with excellent long-term prognosis. A variety of lesions of the EBD that show papillary architecture should be distinguished from papillary carcinoma. Here, we report a case of papillary carcinoma of the common bile duct showing minimal invasion. Separation of invasive from non-invasive or minimally invasive papillary carcinoma is critical in estimating the patient outcome