Primary soft-tissue neoplasms of the foot: the clinicopathologic features of 401 cases.

Neoplasms of the soft tissue range from benign, indolent tumors to some of the most aggressive malignancies known to man. Despite exhaustive studies detailing their occurrence elsewhere within the body, there has been relatively little detailing such neoplasms arising within the soft tissues of the foot. Due to the relative infrequency with which malignant soft-tissue tumors occur in the foot, large series pertaining to this location have been difficult to assemble. To better elucidate the relative frequency of various benign and malignant soft-tissue tumors of the foot, the clinicopathologic features of 401 such neoplasms are described. All cases were retrieved from the files of a major medical center which specializes in the treatment of cancer. Of the 401 cases identified, 149 were benign, while 252 cases were of a malignant nature. The clinicopathologic features are summarized and descriptive statistics are applied. Special focus is given to the discussion of the malignant and rare tumors within this series. Recent concepts regarding diagnosis, prognosis, and therapy are similarly discussed.

Recurrent umbilical cord torsion leading to fetal death in 3 subsequent pregnancies: a case report and review of the literature.

During a span of 3.5 years, a 30-year-old, gravida 9, para 3 woman experienced 3 pregnancies complicated by umbilical cord torsion and constriction. In each case, the complication resulted in acute vascular compromise and intrauterine fetal demise. Gross examination disclosed cord constriction and torsion at the fetal end of the cord in each instance. Histologic sections from the cord torsion sites demonstrated fibrosis and deficiencies in Wharton's jelly in each case. Cytogenetic studies prepared using fetal villous tissue demonstrated normal karyotypes in fetal cells from the first 2 pregnancies (46,XX and 46,XY, respectively). The karyotype from the third pregnancy showed a 46,XX,del(X)(q24) mutation in 3 of 15 cultured cells, while 12 of 15 cells possessed a normal 46,XX karyotype. This cytogenetic abnormality was not believed to represent the cause of fetal demise in this case. To our knowledge, this is the first report of umbilical cord torsion in 3 pregnancies within one family. The familial clustering observed in this report suggests that a genetic predisposition for umbilical cord torsion may exist in some cases.

Isolated Langerhans cell histiocytosis of the thyroid: a report of two cases with nuclear imaging-pathologic correlation.

We describe two cases of isolated langerhans cell histiocytosis (LCH) of the thyroid gland, one of which was found in conjunction with an incidental papillary carcinoma. The first case was that of a 43-year-old man who presented with a 1- to 2-cm nodule within the left lobe of the thyroid. Fine-needle aspiration cytology revealed atypical cells with convoluted nuclei in a background of eosinophils and lymphocytes. The findings prompted a recommendation for excision secondary to the high suspicion of a hematologic malignancy. Histologic sections demonstrated LCH in association with a small focus of papillary carcinoma. The second case involved a 43-year-old woman who presented with a 1.8-cm nodule within the right lobe of the thyroid. Fine-needle aspiration in this case demonstrated abundant hemosiderin-laden macrophages, occasional lymphocytes, and a single benign sheet of follicular cells. No eosinophils were seen; however, a single group of atypical histiocytic cells with cleaved nuclei was noted. The nodule was subsequently resected. Histologic examination demonstrated LCH in association with follicular nodular hyperplasia with cystic degeneration. Immunohistochemical studies were performed in both cases, revealing CD1a and S100 immunoreactivity in the Langerhans' cells. Although LCH may occur as a manifestation of systemic disease, its occurrence as an isolated finding in the thyroid is rare. Its occurrence in association with papillary carcinoma of the thyroid is even more uncommon. We present two cases of isolated LCH of the thyroid, one of which was found in association with papillary carcinoma of the thyroid. The cytologic, histologic, immunohistochemical, and radiologic features are described in each case. The ultrastructural findings from the first case are also presented.

Aggressive epithelioid hemangioendothelioma of the lower extremity: a case report and review of the literature.

A case of epithelioid hemangioendothelioma (EHE) of the left lower extremity in a 50-year-old male is discussed. The neoplasm presented as a tan-purple, painful mass involving the fourth digit of the left foot. Following an excisional biopsy and partial amputation of the fourth digit, the lesion recurred with a multifocal distribution. A physical exam following the recurrence demonstrated multiple palpable lymph nodes in the left inguinal region. A complete amputation of the fourth digit was performed in conjunction with local excisions of the remaining lesions. Additionally, an inguinal lymphadenectomy was performed for staging purposes. Histologically, the tumor was predominantly composed of epithelioid endothelial cells forming nests, cords, and sheets and occasionally forming irregular, primitive vascular structures. Less commonly, nests of tumor cells assumed a spindled morphology. Patches of necrosis and hemorrhage were apparent throughout the neoplasm. There was minimal atypia and mitoses were not identified. Immunohistochemical stains for CD34, CD31, and Ulex europaeus antigens were positive in tumor cells. This case illustrates the potential aggressive behavior of this rare neoplasm and argues against the designation "tumor of low-grade malignancy" with which it is commonly attributed.

Myelodysplastic syndrome with atypical eosinophilia in association with ring chromosome 7. A case report.

The presence of morphologically abnormal eosinophils in the bone marrow and/or peripheral blood has been rarely reported as a prominent feature in myelodysplastic syndromes (MDS). Specific chromosomal aberrations have been observed in such cases. We report a case of a 76-year-old man who presented with chronic, transfusion-dependent anemia. Peripheral blood smear analysis revealed anisocytes, mild leukopenia, and occasional hypersegmented eosinophils. A subsequent bone marrow biopsy and aspiration disclosed hypercellularity, and morphologic abnormalities within the megakaryocyte, erythroid, and myeloid series. The myeloid population was predominantly comprised of eosinophils with varying degrees of dyspoiesis. The constellation of hematologic findings were without a precise categorization according to the FAB classification of myelodysplastic syndromes. Subsequent cytogenetic techniques demonstrated a ring chromosome 7 in all 20 metaphases analyzed in cultured bone marrow cells. Eighty-five-percent of the analyzed cells showed a ring chromosome composed of both the long and short arms: r(7)(p22q36). In the remaining metaphases, the ring was composed of only the short arm: r(7)(p22q10). To our knowledge, these uncommon cytogenetic abnormalities have not been previously reported in association with MDS with morphologically atypical bone marrow or peripheral eosinophilia.

Clear-cell epithelioid leiomyoma of the round ligament.

A case of clear-cell epithelioid leiomyoma of the round ligament in a 69-year-old woman is described. The neoplasm presented as a firm left inguinal mass. A preoperative computed tomography (CT) scan demonstrated an enhancing lesion extending extra-abdominally from the region of the external inguinal ring. The surgical resection specimen was tan-white, well-circumscribed, and measured 5.8 cm in maximum dimension. Microscopic examination revealed a well-demarcated neoplasm comprised of polygonal cells with abundant clear cytoplasm arranged in clusters and single files with abundant intervening hyalinized stroma. There was minimal nuclear atypia and mitotic figures were rare. Periodic acid-Schiff with diastase demonstrated intracytoplasmic glycogen. Immunohistochemical stains for pan-actin, smooth muscle actin, desmin, bcl-2, and vimentin were positive in the tumor cells, whereas stains for CD34, carcinoembryonic antigen, cytokeratin, epithelial membrane antigen, S100 protein, and neurofilaments were negative. Electron microscopy demonstrated features of smooth muscle differentiation including longitudinally oriented fine filaments with focal condensations, pinocytotic activity, and subplasmalemmal densities. This case illustrates the ubiquitous distribution of epithelioid smooth muscle neoplasms and highlights the potential pitfalls for diagnosis when they occur in an unusual location.

Pyloric gland adenoma of the main pancreatic duct.

A case of a pyloric gland type adenoma of the main pancreatic duct in a 69-year-old woman is reported. The tumor led to occlusion and cystic dilatation of the main duct in the pancreatic tail. The surgical resection specimen disclosed a polypoid, bilobed mass attached to the wall of the main pancreatic duct by a thin fibrous stalk. Light-microscopic examination revealed a well-demarcated nodule composed of closely packed tubular glands lined by columnar, mucin-secreting cells with abundant clear cytoplasm and basally oriented nuclei. Focal, mild cytologic atypia was seen. Pyloric metaplasia and focal papillary hyperplasia was present in the adjacent ductal epithelium. Periodic acid-Schiff reactions, with and without diastase predigestion, showed reactivity in the tubular glands, whereas alcian blue (pH 2.5) was negative. Immunohistochemical stains for chromogranin, serotonin, somatostatin, and gastrin failed to detect the respective antigens. Genetic analysis using polymerase chain reaction with mutant enrichment and allele specific oligonucleotide hybridization detected a single mutation at codon 12 of K-ras, which changed the wild-type glycine to arginine. This mutation is commonly found in invasive pancreatic ductal carcinomas. Although tumors with microscopic and immunohistochemical features consistent with pyloric gland adenoma have been described in the gallbladder, to our knowledge, this is the first reported case within the pancreatic ductal system. The finding of a K-ras, codon 12 mutation and the presence of focal dysplasia may denote neoplastic potential in association with this lesion.