Cutaneous angiosarcoma arising in massive localized lymphedema of the morbidly obese: a report of five cases and review of the literature.

A significant proportion of angiosarcoma arises in the setting of chronic lymphedema, often following surgery for breast carcinoma (Stewart-Treves syndrome). Massive localized lymphedema (MLL), a relatively recently described and rare pseudotumor most often occurring in morbidly obese patients, is thought to be the result of derangements in lymphatic drainage secondary to excessive adipose tissue. Only a very small number of cases of angiosarcoma have been reported in patients with MLL. We reviewed the clinical and pathological features of five patients with angiosarcoma arising in this unusual clinical setting. The tumors arose in three women and two men, who were in the age range from 36 to 81 years (median: 52 years). All patients were or had recently been morbidly obese (mean body mass index = 47.2 kg/cm(2) ; range:37.6-68.5 kg/cm(2) ) and had long-standing soft tissue changes of MLL. The tumors arose in the thigh (two cases), lower leg (two cases) and abdomen/proximal thigh (one case) and ranged from 6 to 28.5 cm in size (mean: 15.9 cm). All tumors were conventional high-grade angiosarcomas and microscopically exhibited an admixture of vasoformative, spindled and epithelioid features. Microscopic changes of MLL, including dermal fibrosis, expansion of interlobular fibrous septa and lymphangiectasia, were identified in all cases. Clinical follow-up (four cases; mean: 13.8 months; range: 2-32 months) showed one patient to be dead of disease, one patient to be dead from therapy-related complications and two patients to be alive without disease. One case was too recent for meaningful clinical follow-up. We conclude that lymphedema secondary to obesity should be recognized as a significant risk factor for the development of angiosarcoma. The clinical and pathological features of angiosarcoma arising in this distinctive clinical setting appear to be identical to those of other lymphedema-associated angiosarcomas. With the continuing epidemic of obesity in Western societies, the incidence of obesity-related angiosarcoma is probably to increase. Consequently, it is critical that lymphedematous areas in obese patients be carefully examined for clinical evidence of early angiosarcoma, with the hope that timely diagnosis may lead to improved patient outcome.

Sulfa-induced acute eosinophilic pneumonia.

Acute eosinophilic pneumonia (AEP) is an infrequently seen interstitial lung disease secondary to medications. We report a series of 3 case of severe AEP which developed as a result of sulfa medication. 2 patients had received treatment with sulfamethoxazole for acne and 1 was treated with sulfasalazine for colitis. Patients were on sulfa medication for 1-3 weeks prior to presentation. All patients presented with fever, acute onset bilateral pulmonary infiltrates as well as marked peripheral eosinophilia. Mean eosinophil count was 2.21 × 109/L. There was a lack of response to steroids. One patient required extracorporeal membrane oxygenation and prolonged mechanical ventilation via tracheostomy. 2 patients underwent successful lung transplantation (1 bilateral living-related lobar lung transplant and 1 orthotropic cardiopulmonary allotransplantation). In all cases lung biopsy and explants showed acute and organizing diffuse alveolar damage with increased interstitial and airspace eosinophils. To our knowledge, our series is the first to show the clinical features of sulfa induced AEP in an adolescent population.

Albumin In Situ Hybridization Can Be Positive in Adenocarcinomas and Other Tumors From Diverse Sites.

OBJECTIVES: Albumin messenger RNA (mRNA) expression is a marker of hepatocellular differentiation. Most published data are from review of tissue microarrays, and albumin in situ hybridization (ISH) expression across several tumor types is incompletely characterized. METHODS: Sections from 221 tumors were evaluated for albumin mRNA. Immunohistochemistry was used to confirm diagnoses. Albumin ISH was performed according to manufacturer-provided instructions. Fifty-nine cases were evaluated with both commercial ISH assays. RESULTS: Albumin mRNA was detected in all hepatocellular carcinomas (HCCs) and 81% of intrahepatic cholangiocarcinomas. Lung (20%), gallbladder (39%), hepatoid pancreatic (n = 1 of 1) adenocarcinoma, breast invasive ductal carcinoma (18%), yolk sac tumor (25%), and acinar cell carcinoma (29%) showed expression. Both assays were concordant in 93% of cases. CONCLUSIONS: Albumin ISH was expressed in all HCCs studied. It was also positive in intrahepatic cholangiocarcinoma and patchy positive in gallbladder adenocarcinoma and a subset of other neoplasms, which can be a potential pitfall.