Primary pseudomyogenic hemangioendothelioma of bone: case report and review of the literature.

Pseudomyogenic hemangioendothelioma (PMH) represents a multicentric recently characterized tumor type, generally presenting in young adults, of postulated vascular origin and intermediate malignancy. This entity tends to arise in the deep-seated dermal-subcutaneous locations, preferentially limited to one anatomic site, and may extend secondary to bone. PMH restricted to the skeletal system is rare. To our knowledge, only 19 cases with description of both histologic and clinical findings have been reported to date. We report the clinicopathological features of a further intraosseous PMH occurring in a 46-year-old woman involving the right patella. Histologic examination showed an infiltrating growth composed of sheets and fascicles of spindled to epithelioid large cells, with ample eosinophilic cytoplasm, large vesicular nuclei and prominent nucleoli, sometimes resembling rhabdomyoblastic tumor cells, without morphologic signs of vascular differentiation. At immunohistochemical examination, neoplastic cells stained diffusely for AE1/AE3 keratins, vimentin, ERG, FLI-1, INI-1, FOSB with only focal CD31 expression.The morphologic clues leading to the correct diagnosis of intraosseous PMH have been correlated with the data of the literature, and a special emphasis has been given to the differential diagnosis with other neoplasms, particularly epithelioid sarcoma, in order to avoid unnecessary radical surgery and to optimise possible treatment protocols.

Tall cell variant of papillary breast carcinoma: an additional case with review of the literature.

Papillary lesions of the breast can be one of the most challenging aspects of mammary pathology because of a wide morphologic spectrum that may be encountered in these lesions. An unusual breast tumor has been first classified as "breast tumor resembling the tall cell variant of papillary thyroid carcinoma" and subsequently renamed "tall cell variant of papillary breast carcinoma". To our knowledge, only 13 cases of this neoplasm have been reported so far. Metastasis to the breast is not an uncommon event and about 5% of all such cases are of the thyroid origin. We report the clinico-pathological and immunohistochemical features, together with a molecular screening for BRAF mutations, of an additional case of tall cell variant of papillary breast carcinoma occurring in a 65-year-old woman. The immunohistochemical and molecular clues leading to the correct diagnosis have been correlated with the data of the literature. Tall cell variant of papillary breast carcinoma represents a unique histologic subtype of mammary carcinoma of probably low malignant potential which has to be recognized to avoid misdiagnosis as metastatic carcinoma from the thyroid.

[Biliary ileum and surgical emergency]. / Ileo biliare ed urgenza chirurgica.

The Authors report a case of intestinal occlusion due to a gallstone migrated in jejunum and, with a review of the literature, stress the difficult decision making for surgical timing in a critical patient.

[Appendiceal carcinoid: report of 3 clinical cases and review of the literature]. / Carcinoide appendicolare: descrizione di tre casi clinici e revisione della letteratura.

The Authors report three cases of appendiceal carcinoids, found during appendectomy for acute appendicitis during the last 15 years. Appendiceal carcinoid is a rare neoplasm, more frequent in females age ranging between 20 and 29 years. Lack of symptoms explains the occasional diagnosis of this disease. The prognosis is related mostly to the size of the tumor, local and lymphatic invasion, presence of metastases, localization inside the appendix, histological type and age of the patient. Therapy is mainly surgical and consists of appendectomy and right colectomy according to different criteria which are being discussed in this article.

[Acute abdomen caused by colo-colic invagination: a case of invagination of a colonic lipoma]. / Addome acuto da invaginazione colo-colica: un caso di invaginazione da lipoma del colon.

The Authors report a case of intestinal occlusion due to a colo-colonic intussusception arising from a lipoma of trasverse colon and, by a review of the cases in the Literature, they discuss diagnostic and therapeutic procedures in emergency.

[Familial adenomatous polyposis: review of the literature and report of 3 cases]. / La poliposi adenomatosa familiare: revisione della letteratura e descrizione di tre casi clinici.

The Authors describe three cases of Familial Adenomatous Polyposis, (FAP), in patients of the same family, mother and two daughters, with different stages of the disease. Familial adenomatous polyposis is a mendelian dominant inherited syndrome with an incidence of 1:11,000, caused by an alteration of APC gene, which causes multiple disorders of the development ecto-, endo- and mesoderma. The syndrome is characterized by the presence of adenomatous polyps in the gastroenteric tract, mostly in colon-rectum and duodenum with demonstrated adenoma-carcinoma sequence. In the family here reported a case of familial adenomatous polyposis at the adenomatous stage and two of cancer of colon-rectum are registered. In the first case surgery had a preventive aim, and ileo-rectal anastomosis was performed; in the other two cases the treatment was Miles operation with radical intention.

[MALT lymphoma of the gastrointestinal tract: description of clinical cases and review of the literature]. / Linfomi MALT del tratto gastroenterico: descrizione di casi clinici e revisione della letteratura.

MALT-lymphomas are tumors of the mucosa associated lymphoid tissue, without primitive involvement of lympho nodes and spleen. They are located preferentially in the gastroenteric tract specially in the stomach (27%), but they may occur also in the respiratory and urinary tracts. There is a very well known link between MALT-omas and Hp infection, not only in the gastroenteric tract, but also in other sites. Moreover eradication of Helicobacter pylori can, in some cases, contribute to achieve the complete remission of the tumor. In this article the Authors report three cases of MALT-lymphomas: two of the stomach and one of the jejunum-ileum. Diagnosis is based on instrumental tests according to the involved tract and endoscopy of the upper digestive tract associated with biopsies more specific in case of gastric involvement; yet bioptic samples show a more significative incidence of false negative due to the extramucosal site of tumor. In these cases, a correct diagnosis will be obtained only through histologic examination of the surgical specimen. Surgery obtains good results in early stages, playing also a central role in palliation in association with radio- and chemotherapy. In case of low-grade B MALT-lymphomas of the stomach and in IE stages, eradication of Helicobacter pylori can, in 70-90% of cases, reduce the extension of the lymphocytic infiltration.

[Primary adenocarcinoma of the appendix. Case report and review of the literature]. / Adenocarcinoma primitivo dell'appendice. Descrizione di un caso clinico e revisione della letteratura.

A rare case of primary adenocarcinoma of the vermiform appendix (less than 250 cases described in the literature) in a 36 year-old female patient presenting signs and symptoms of an acute appendicitis is reported. Adeno-carcinoma of the vermiform appendix is a rare neoplasm of the gastrointestinal tract with an incidence of about 0,01-0,2%. Usually the diagnosis of adenocarcinoma of the vermiform appendix is difficult because symptoms and signs are not pathognomonic. In the case described, the diagnosis was intra and postoperative and confirmed by the pathological examination of the surgical specimens. At laparotomy, performed under suspicion of an acute appendicitis, disseminated disease was discovered, characterized by the involvement of the two ovaries, the left colon, the cecum, the vermiform appendix, with a peritoneal carcinosis and a hydroureteronephrosis. According to the dissemination of the disease, the surgical treatment was right hemicolectomy, anterior resection of left colon, bilateral oophorectomy and omentectomy. The post-operative course was regular. Adjuvant therapy was performed for 6 cycles, with 5FU and oxaliplatinum. The patient is still alive after 6 months and there is no sign of progression of the disease. A mild left hydroureteronephrosis is persistent.

[Primary gastric lymphoma: a case report]. / Linfoma gastrico primitivo: descrizione di un caso clinico.

In this case report the Authors describe a case of primary gastric lymphoma in a 62 years old patient who presented with dyspepsia and weigh loss. Primary gastric lymphoma is a rare neoplasm which of 1-10% of the malignant gastric neoplasms in the gastroenteric tract. The clinic presentation is usually aspecific. The infection by H. pylori is a factor of predisposition for this kind of disease. The diagnostic pathway consists in x-ray examination of the gastrointestinal tract, the endoscopy with biopsies, the computerized tomography and the echo-endoscopy. However obtaining a preoperative diagnosis is often difficult because of the submucosal localization of the lymphoma. There is not a common strategy among the Authors for the treatment of the disease, which can be surgical, radiotherapic or chemotherapic.

[Retroperitoneal tumors: a case of liposarcoma]. / I tumori del retroperitoneo: un caso di liposarcoma.

The Authors report a case of retroperitoneal sarcoma, in the histological subtype "liposarcoma", occasionally diagnosed in a 57 year old woman, who presented aspecific symptoms, characterized by dry cough, vomiting and chest pain. Chest TC showed a neoplasm of the retroperitoneum, close to liver, right colon, duodenum and right kidney. The arteriography of the right renal artery showed a neovascolarization inside the mass. A surgical resection was performed. The histological examination confirmed the diagnosis of liposarcoma. Liposarcomas account for 30% of the retroperitoneal soft tissue sarcomas; symptoms and diagnosis are late and the surgical resection of primary neoplasm, metastases and local recurrences representing the only therapeutic choice.

[Gastrointestinal stromal tumors: report of a clinical case and review of the literature]. / Tumori stromali gastrointestinali: descrizione di un caso clinico e revisione della letteratura.

In this case report, the Authors describe a case of stromal gastric tumour, in a male 65 years old, who presented gastrointestinal bleeding. Gastro-Intestinal Stromal Tumors (GISTs) are neoplasm with an incidence of 1-3 per cent of the digestive tract malignant neoplasms. The rarity of this disease, its visceral wall localization, the histopathological characteristics make the diagnosis difficult. Moreover there is no correlation between the behaviour of these neoplasms and the histologic features. Surgery represents the main treatment for GISTs based on complete resection, followed by a long-term follow-up. Chemotherapy and radiotherapy don't seem to play a crucial role in the treatment of these neoplasms. A new treatment with inhibitors of the tyrosinekinase is under discussion. Follow-up represents the only way to evaluate the effective behaviour of the disease, due to the lack of classic prognostic factors impact.