Intranodal Palisaded Myofibroblastoma: A Diagnostic Differential for Inguinal Lymphadenopathy.

BACKGROUND Benign tumors of the lymph nodes are rare and are not usually considered in the differential diagnosis in cases of lymphadenopathy because reactive hyperplasia, lymphoma, and metastatic carcinoma are the most likely causes of enlarged nodes. Intranodal palisaded myofibroblastoma (IPM) is a very rare benign mesenchymal tumor of the lymph nodes most often affecting but not limited to the inguinal region, with up to 92 cases reported in the English literature. The cell of origin is the intranodal differentiated smooth muscle cell or myofibroblast. Although the pathophysiology of IPM remains unclear, theories about viral oncogenesis and mutational changes in the ß-catenin gene with subsequent abnormal expression of ß-catenin and cyclin D1 have been raised. CASE REPORT We report a case of IPM in a 48-year-old man who presented with a mass in the left groin, with inconclusive imaging. The typical histologic findings of smooth muscle actin, cyclin D1, and ß-catenin positive intranodal spindle cell proliferation with characteristic palisades, amianthoid fibers, collagenous bodies, lack of atypia, and very low mitotic count, together with characteristic profile on ancillary testing, confirmed the diagnosis. In addition to staining with smooth muscle actin, cyclin D1 and ß-catenin, immunohistochemical studies showed focal positivity with desmin, a finding previously reported in 2 of the published cases. Surgical excision is usually curative, with a 6% recurrence rate and no reported cases of locally aggressive disease or malignant transformation. CONCLUSIONS Although rare, IPM should be included in the differential diagnosis of isolated lymphadenopathy.

Fournier gangrene in pregnancy.

BACKGROUND: Fournier gangrene is a rare and rapidly progressive necrosis of the perineal and genitourinary region. Although predominantly affecting men, we describe an unusual presentation in pregnancy. CASE: A 30-year old primigravid woman in the third trimester of pregnancy presented with a history of low back pain. Subsequently, a diagnosis of perianal sepsis and associated Fournier gangrene was established. The patient underwent an emergency cesarean delivery followed by radical surgical debridement and colostomy. CONCLUSION: Expedited delivery, timely diagnosis of gangrene, urgent, extensive debridement, and broad-spectrum antibiotic coverage contributed largely to effective management of this rare presentation in pregnancy.