Rare Presentation of Limb-Body Wall Complex in a Neonate: Case Report and Review of Literature.

The limb-body wall complex (LBWC) aka body stalk syndrome is an uncommon congenital disorder characterized by severe malformations of limb, thorax, and abdomen, characterized by the presence of thoracoschisis, abdominoschisis, limb defects, and exencephaly. This condition is extremely rare with an incidence of 1 per 14,000 and 1 per 31,000 pregnancies in large epidemiologic studies. Majority of these malformed fetuses end up with spontaneous abortions. We present this rare case with occurrence in a preterm infant of 35 weeks' gestation. Our report highlights majority of the clinical presentations as reported in previous literature, but the significant pathological findings of absent genitalia and malformed genitourinary as well as anorectal malformations make this case presentation an even more rare occurrence. Infant karyotyping was normal male and there is no specific underlying genetic correlation in this condition which has a fatal prognosis.

How natural disasters change natural patterns: coccidioidomycosis imported to New Orleans.

OBJECTIVE: To identify cases of coccidioidomycosis in the New Orleans area following Hurricane Katrina. METHODS: We performed clinical surveillance across multiple medical disciplines and among three major teaching hospitals in the greater NOLA area in the posthurricane period. Each case involved a detailed history, physical examination, laboratory evaluations, and, in the two fatal cases, a comprehensive postmortem examination. RESULTS: We identified four cases of coccidioidomycosis during the posthurricane period: three with disseminated disease and one with disease limited to the respiratory tract. Two patients were co-infected with HIV and died during hospitalization; one was only diagnosed at autopsy. The two immunocompetent patients responded well to antimicrobial therapy. CONCLUSIONS: A heightened awareness of non-endemic disease is warranted in the practice of postdisaster clinical medicine and public health, as demonstrated by the appearance of coccidioidomycosis in the traditionally nonendemic NOLA area following Hurricane Katrina.

Mediastinal pleomorphic sarcoma in an immunodeficient patient: case report and review of the literature.

Pleomorphic sarcoma, widely known as malignant fibrous histiocytoma (MFH), is a soft tissue sarcoma. The occurrence of this malignancy in the mediastinum is rare. To our knowledge, only 13 cases of MFH of the mediastinum have been previously reported. Furthermore, only three cases of MFH in patients infected with human immunodeficiency virus (HIV) have been previously described. Here we present a 44-year-old African-American male who complained of epigastric pain radiating to the right chest. On admission, a chest radiograph revealed a widened mediastinum, and chest computerized tomography (CT) identified a large mass in the posterior mediastinum. Histologic diagnosis revealed a high-grade MFH. He was also incidentally diagnosed with HIV infection. The rarity of this malignancy and uncommon site of presentation in association with an immunodeficient state makes this case unique. This is the first report in the literature of an HIV-infected patient presenting with this uncommon tumor in the mediastinum.

Myxofibrosarcoma with metastasis to the lungs, pleura, and mediastinum: a case report and review of literature.

Myxofibrosarcoma is a common connective tissue neoplasm of malignant fibrocytes in a myxoid matrix favoring the extremities. Like many other tumors of connective tissue, soft tissue sarcoma exhibits high recurrence rates but is rarely known to metastasize. We present a patient with myxofibrosarcoma of the hand with metastases to the lungs, pleura, and mediastinum. The mediastinal metastasis presented clinically with gastrointestinal symptoms due to compression of the gastroesophageal junction. To our knowledge, this is the first report of metastatic myxofibrosarcoma of the hand and also the first report of metastatic myxofibrosarcoma to mediastinal lymph nodes at the level of the gastroesophageal junction. We also performed a comprehensive literature review of metastatic myxofibrosarcoma.