Nodal and extranodal soft tissue polymorphous hemangioendothelioma: a case report and review of the literature.

BACKGROUND: Polymorphous hemangioendothelioma is a rare vascular tumor of borderline malignant potential and only 10 cases have been described in the literature so far. METHODS AND RESULTS: We report a case of nodal and extranodal polymorphous hemangioendothelioma and review the literature. A 66-year-old man with an unremarkable past medical history was admitted for a left submandibular mass. The main nodule was composed of angiosarcoma-like areas mixed with angiomatous features; some vascular spaces with hobnail endothelium were seen. The tumor involved two adjacent lymph nodes. Immunohistochemically, the neoplastic cells were strongly positive for CD31 and vimentin. After a few months the tumor recurred and the patient was treated with radiation therapy. CONCLUSIONS: Polymorphous hemangioendothelioma is a distinct entity in the hemangioendothelioma group with its own clinical and histological features.

Primary peritoneal clear cell carcinoma versus ovarian carcinoma versus malignant transformation of endometriosis: a vexing issue.

Peritoneum is a site for both primary and secondary tumors. Primary peritoneal tumors are fairly rare. The most common primary tumors of the peritoneum are malignant mesothelioma and serous papillary adenocarcinoma. Clear cell carcinoma of the peritoneum is extremely rare and often misdiagnosed as mesothelioma, serous carcinoma, or metastatic adenocarcinoma, so it represents a diagnostic challenge for both clinicians and pathologists. Up to date, to the best of our knowledge, only 11 cases of primary peritoneal clear cell carcinoma have been reported in the English literature. Distinguishing this tumor of the peritoneum versus ovarian carcinoma can be problematic. Herein, we report a rare case of primary peritoneal clear cell carcinoma occurring in a 49-year-old woman, along with a review of the literature.

An unusual association of malignant gastrointestinal neuroectodermal tumor (clear cell sarcoma-like) and Ewing sarcoma.

Very recently a new designation of "Malignant Neuroectodermal Gastrointestinal Tumor" has been proposed for an aggressive form of neuroectodermal tumor with features similar to that of Clear Cell Sarcoma of Soft Tissue, however without a melanocytic differentiation. Also known as "clear cell sarcoma-like tumors of the gastrointestinal tract", these tumors show some features strongly suggesting an origin from a gastrointestinal neuroectodermal precursor cell unable to differentiate along the melanocytic lineage. They occur mainly in young and middle-aged adults, and have a poor prognosis with a high rate of liver and lymphnode metastases. Histologically they are composed of epithelioid or oval-to spindle cells with a sheet-like or nested pattern of growth, strongly positive for neural markers (S-100, SOX10, and vimentin) and negative for the melanocytic ones. EWSR1 gene rearrangements including EWSR1-ATF1 or EWSR1-CREB1 GENE fusions are typically assessed in these tumors. Here we report a case of malignant neuroectodermal gastrointestinal tumor which immunophenotypically unusually expressed FLI-1, occurring in a 29-year-old man with a previous medical history of Ewing sarcoma. We finally suggest that this case might be a further evidence of a link between these two entities.

Cystic variant of endometrial stromal sarcoma: report of two cases.

Endometrial stromal sarcoma is an uncommon tumor representing 0.2% of all uterine neoplasm and 15% to 26% of uterine sarcomas. Endometrial stromal sarcoma has a gross appearance as single nodule, multiple masses, or a poorly demarcated lesion with occasional cystic degeneration; rarely, it shows a cystic multilocular feature. We report 2 cases of endometrial stromal sarcoma with a prominent cystic appearance, forming multilocular cystic mass, detected by ultrasonography. The differential diagnosis among multicystic endometrial stromal sarcoma and cystic uterine tumors might be very difficult.

Disseminated Cryptococcus neoformans infection and Crohn's disease in an immunocompetent patient.

Cryptococcus neoformans is a human pathogen ubiquitously present in the environment. It primarily affects immunocompromised patients, but individuals with no underlying disease or immunodeficiency can also be affected. We herein describe the case of a patient found to have Crohn's disease and disseminated cryptococcosis simultaneously. She had no predisposing underlying cause for impaired immunity. Our patient showed signs that would have make it hard to discriminate between an inflammatory bowel disease and an infection if bowel only would have been involved. The patient underwent surgical intervention; medical therapy was effective against Cryptococcus. She is at now being followed-up for Crohn's disease. When dealing with patient affected with inflammatory bowel diseases, careful history taking, objective and instrumental examination are demandable in order not to overlook associated conditions or infectious diseases. Diagnosis and therapy of cryptococcosis infection in patient with Crohn's disease are herein discussed.

Unexpected autoptic finding in a sudden death: gossypiboma.

Gossypiboma, i.e. a retained surgical sponge, is a serious and rare complication in surgical practice, most commonly occurring in abdominal procedures. Migration of retained surgical sponge is an unusual sequelae, particularly if occurring in tracheo-bronchial tree. Herein, we report a 35-year-old man who had had a retained surgical sponge forgotten during a radical thyroidectomy, and subsequently a trans-luminal migration of the gossypiboma which went through the trachea causing a sudden death of the patient. All the operators of the surgical team should keep in mind this terrible complication to avoid unpleasant consequences to patient and themselves.