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.

Unclassified sex cord/gonadal stromal testis tumor with a "pure" spindle cell component: a case report.

Unclassified sex cord/gonadal stromal tumors (SCSTs) composed predominantly of spindle cells are rare. Very few cases have been documented to date. Here, we report a case of "pure" spindle cell tumor of the left testis in a 83-year old man whose morphological and immunohistochemical findings were consistent with a diagnosis of unclassified SCST and review the literature. Owing to the spindle cell pattern, the differential diagnosis with other benign and malignant spindle cell lesions is discussed.

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.

Olfactory neuroblastoma with focal ganglioneuroblastic differentiation: a case report with literature review.

Olfactory neuroblastoma (ONB) is a rare malignant neuroectodermal tumour, with clearly defined histologic and immunohistochemical features, that typically arises in the superior nasal cavity. Although the classical clinicopathological features leave little room for misinterpretation, the wide variability in this tumour, including occasional divergent differentiation, may cause diagnostic difficulty. Herein, an unusual case of ONB with focal ganglioneuroblastic differentiation in an 81-year-old woman arising from the anterior ethmoid, filling the upper portion of the left nasal cavity and sparing the sinus cavities, is described. Histologically, the tumour was composed of atypical monotonous round cells that were positive for NSE, CD56, chromogranin, synaptophysin, neurofilament and calretinin and exhibited an irregular lobulated and nested growth pattern and sparse mitotic figures (3 to 4 mitoses per 10 HPF). Focally, the histology changed to ganglioneuroblastic differentiation consisting of large ganglion and spindle cells, positively staining for S-100, GFAP, CD99, neurofilament, calretinin, chromogranin and synaptophysin. Neuroblastomas, occurring in the nasal cavity, in analogy to other sites, tend to have an aggressive biologic behaviour and can histologically mimic other undifferentiated malignant neoplasms of the sinonasal tract. Differential diagnostic problems are discussed; a comprehensive review of the literature has also been performed with a focus on survival.

Uterine endometrioid adenocarcinoma with extensive pilomatrixoma-like areas. A case report.

Shadow cells are typical features of pilomatrixoma, although they have been described in other benign cutaneous tumours with characteristics of differentiation toward the hair matrix. The finding of extensive shadow cell differentiation in visceral carcinomas is otherwise unusual. We report herein a case of uterine adenocarcinoma with extensive pilomatrixoma-like areas in a 74-year-old woman. The endometrial tumour showed an invasive poorly differentiated growth with squamous differentiation deeply extending into the myometrium intermixed with lobules of empty squamoid polyhedral cells with clear shadow like nuclei, focally exhibiting a 'ghost' appearance. The cervix, salpinges, ovaries and pelvic lymph nodes were free of disease and, taking all evidence into account, the tumour was diagnosed as poorly differentiated endometrial endometrioid adenocarcinoma (FIGO stage IB). The recognition of an extensive pilomatrixoma-like component in a high- grade endometrioid adenocarcinoma may be important to avoid diagnostic misinterpretation with uterine metastases of malignant cutaneous pilomatrical tumours, such as pilomatrix carcinomas.

Subscapular elastofibrolipoma: a new variant type among elastofibromas or lipomas? A case report.

Elastofibroma is a rare soft tissue benign fibrous proliferation that characteristically occurs in periscapular soft tissues of the elderly, particularly in females, with typical morphological features consisting of an admixture of excessive collagen and abnormal elastic fibers displaying a beaded or globular appearance. Here we report an unusual, recently described histological variant with unclear origin, showing the presence of abundant mature fat tissue, named 'elastofibrolipoma', which could lead to confusion with other adipose and mesenchymal cell tumour proliferations. The issue as to whether elastofibrolipoma is reactive or neoplastic, variant of elastofibroma or lipoma, remains controversial. Differential diagnostic problems and histogenetic considerations are provided.

Cystic sebaceous lymphadenoma of the parotid gland: case report and review of the literature.

Sebaceous lymphadenoma (SL) of the salivary glands is a very rare, benign tumour with distinct histopathologic characteristics and uncertain histogenesis. It is almost exclusively confined to the parotid gland where it occurs generally as a slowly growing mass in elderly patients. Several imaging techniques and fine-needle aspiration cytology (FNAC) can be used for diagnosis, but definitive diagnosis is usually established only by histopathological examination. Such lesions are composed of a varying admixture of islands, duct-like structures and cysts covered with squamous or cuboid and columnar epithelium intermingled with a background of lymphoid stroma, sometimes with lymph follicles. Accurate diagnosis of SL is important since many salivary masses in adults are malignant. We report a case of cystic sebaceous lymphadenoma of the left parotid gland surgically resected in a 70-year-old woman. Fifteen months after surgery, the patient is well, with no evidence of recurrence. Differential diagnostic problems and histogenetic considerations are provided; a comprehensive review of the literature has also been performed.

Primary synovial sarcoma of the kidney. A case report with pathologic appraisal investigation and literature review.

Synovial sarcoma (SS) is a soft tissue neoplasm with clearly defined histologic, immunohistochemical and molecular features that usually arises in the extremities of young adults. The occurrence of these tumors in the kidney is extremely rare and have been prevalently described in case reports. The objectives of this work were to evaluate the frequency of primary renal synovial sarcomas and the pathologic progression in recognition of this possibly under-diagnosed entity. A comprehensive review of the literature has also been performed with a focus on survival. We report the clinico-pathological features of an intrarenal SS occurring in a 67-year-old man. The tumour, measuring 4 cm in its greatest diameter, completely replaced the cortex and the medulla of the inferior region of the left kidney compressing the iliopsoas muscle. Radiological imaging was consistent with a renal cell carcinoma. Histologically, the tumour was composed of atypical monotonous vimentin+, CD99+, bcl-2+ spindle cells exhibiting a haphazard fascicular growth pattern and a high mitotic rate (3 to 5 mitoses per HPF). The diagnosis was supported by reverse transcription-polymerase chain reaction which demonstrated SYT-SSX2 gene fusion. The patient was alive with local recurrence of disease 24 months after surgery. Synovial sarcomas occurring in the kidney, in analogy to other sites, tend to have an aggressive biologic behaviour. Despite being extremely uncommon, with only 44 cases reported to date, they should be included in the differential diagnosis of benign and malignant spindle cell tumours of the kidney. This study also emphasizes the importance of a correct pathologic diagnosis for prognostic and therapeutic implications.

Pleomorphic hyalinizing angiectatic tumor of the male breast: a heretofore unreported occurrence.

Pleomorphic hyalinizing angiectatic tumor (PHAT) is considered an unusual mesenchymal tumor of intermediate malignant potential that is distinguished by a prominent angiectatic vasculature with clusters of thin-walled, fibrin-lined vessels surrounded by sheets of spindled and pleomorphic cells. This lesion occurs mostly in the subcutaneous soft tissues of the lower extremities. In this paper, the authors report the first case of PHAT involving the breast parenchyma of a 75-year-old man. The patient was referred to the hospital with a history of a painless left breast mass that had been growing for several years. The lesion did not recur after surgery. The tumor exhibited histopathologic and immunohistochemical features identical to those of a PHAT of other sites. The purpose of this report is to add a rare tumor to the differential diagnosis of mammary spindle cell neoplasms.

Ossifying fibromyxoid tumor with atypical histological features: a case report.

Ossifying fibromyxoid tumor of soft tissues (OFMT) is considered a rare mesenchymal neoplasm. Its main histological features are sheets and ill-defined lobules of rounded bland cells within a fibromyxoid background and a thick collagenous capsule with an incomplete rim of lamellar bone. This lesion occurs mostly in the soft tissues of the lower extremities and limb girdles. In this paper, we describe a mesenchymal tumor removed from the right thigh of a 41 year-old-woman. The neoplasm differed histologically from typical forms of OFMT for areas of moderate cellularity and atypia, nuclear enlargement and small nucleoli. Focally, stromal tongues of osteoid were centrally and irregularly located within the lesion with evident spindling of tumor cells around them. The mitotic activity was low (up to 19 per 50 HPF) and atypical figures were rarely seen. The tumor was positive to S-100 protein, vimentin, CD10, CD56, CD99, ASMA, calponin and collagen IV. Rare elements were positive for cytokeratin AE1/AE3. To the best of our knowledge, this is the first case of atypical OFMT reported to be positive for calponin. The patient is currently alive and well with no evidence of disease at 96 months following surgery. In spite of low-grade histology, OFMT has high local recurrence rate and low metastatic potential, primarily in the lungs, even several years after surgical removal. The recognition of this entity is important. In this report the authors address differential diagnosis and enigmatic histogenesis of this neoplasm.

[Cutaneous angiolipoleiomyoma: a case report and literature review]. / Angiolipoleiomioma cutaneo: segnalazione di un caso e revisione della letteratura.

The Authors describe a rare case of cutaneous angiolipoleiomyoma in an acral location together with a brief literature review. A 62-year-old male presented with a slow-growing asymptomatic nodule, 2.2 cm in diameter, located in the subcutaneous tissue of the left calf. Ten months after surgical excision, the patient is alive and free of disease. Histologically, the lesion was well-circumscribed and contained three components: areas of mature fat tissue were intermingled with cellular areas of spindle eosinophilic cells, reminiscent of smooth muscle cells, and a complex mixture of vessels of different types and sizes. Immunohistochemically, the cellular spindle component was positive for vimentin and smooth muscle actin, and negative for S-100, HMB-45, MART-1 and oestrogen and progesterone receptors. The Authors discuss differential diagnosis with other benign lesions such as angioleiomyoma and subcutaneous angiomyolipoma.

[Myopericytoma-type perivascular myoma located in the soft tissue of the foot: a case report and review of the literature]. / Mioma perivascolare di tipo miopericitoma dei tessuti molli del piede: descrizione di un caso e revisione della letteratura.

We describe a rare case of myopericytoma-type perivascular myoma (MTPM) which arose in acral location and the literature on this field is briefly reviewed. The patient, a 68-year-old man, presented with an enlarging painful nodule, 1.4 cm across, located in the subcutaneous tissue of the right foot. The patient is alive and well 17 months after surgical excision of the nodule. Microscopically, the lesion was well-circumscribed and characterized by a biphasic pattern with a concentric perivascular spindle and ovoid cell proliferation and an extensive hemangiopericytomatous growth component. Immunohistochemically, the neoplastic cells were positive for vimentin, smooth muscle actin, desmin and calponin, negative for S-100 protein, CD34, CD31 and cytokeratins (AE1/AE3, Cam 5.2). Electron microscopy showed electrondense cell bodies suggestive for myopericytic differentiation. The clinico-pathological features of the present case are similar to those previously reported in the literature and we also discuss herein the main histological criteria for the differential diagnosis with other spindle cell and vascular lesions of soft tissues.

[Galanin immunoreactivity in a laryngeal paraganglioma: case report and literature review]. / Paraganglioma laringeo. Descrizione di un caso con espressione immunoistochimica di galanina e revisione della letteratura.

The authors describe a case of laryngeal paraganglioma (LP) occurring in a 57-year-old-woman. To date, 70 cases have been described in the literature. It is benign and recurrences are infrequent. The differential diagnosis with typical and atypical carcinoids, hemangiopericytomas, alveolar soft-part sarcomas, medullary thyroid carcinomas, malignant melanomas and metastatic renal cell carcinomas is supported by immunohistochemistry. Moreover, this tumor shows the immunohistochemical expression of galanin, a variably expressed marker of paragangliomas that it is not expressed in carcinoid tumors. Nevertheless, our observations militate against its role as a solitary marker but advocate its use in conjunction with other antibodies for the differential diagnosis of neuroendocrine neoplasms of larynx.

[Ganglioneuroma of parapharyngeal space: case report and review of the literature]. / Ganglioneuroma dello spazio parafaringeo: descrizione di un caso e revisione della letteratura.

A case of ganglioneuroma of parapharyngeal space is described and the literature on this field is briefly reviewed. The patient, a 7-year-old female, presented with a left parapharyngeal space mass causing medial displacement of the left lateral pharyngeal wall. The lesion sized 4.4 cm in the largest diameter. Histologically, the tumour showed the coexistence of two distinct cells types--ganglion cells and Schwann cells--within a loose stroma. Immunohistochemically, both cell types were immunoreactive for S-100 protein and negative for cytokeratins.

[Lymphoepithelioma-like carcinoma of the esophagus: description of a case]. / Carcinoma simil linfoepitelioma (LELC) dell'esofago: descrizione di un caso.

The authors describe a case of lymphoepithelioma-like carcinoma (LELC) of the esophagus occurring in a 77-year-old man. To date, 13 cases have been described in the literature, all in natives of Japan. Histological features, immunohistochemical findings and differential diagnosis are discussed. In our case epithelial neoplastic cells were completely negative for the EBV genome.

[Lympho-epithelial carcinoma (malignant lympho-epithelial lesions) of the parotid gland. Description of a case with detection of EBV by in situ hybridization]. / Carcinoma linfoepiteliale (lesione linfoepiteliale maligna) della ghiandola parotide. Descrizione di un caso con rilevazione dell'EBV mediante ibridazione in situ.

The authors describe a case of malignant lymphoepithelial lesion (MLEL), commonly referred to as lymphoepithelial carcinoma of parotid gland, that is a very rare tumour. There is a relatively high incidence in Eskimos of Alaska and Greenland, but some cases are described in natives of south China. The immunophenotypic profile and histopathological aspect of this neoplasm are discussed, and the differential diagnosis in regard to other primitive or metastatic tumours of parotid is also considered. In our case a diffuse positivity of epithelial neoplastic cells for EBV genome was found using in situ hybridization. The possible role of EBV in the ethiopathogenesis of this rare lesion is herein discussed.

[Hemangiopericytoma-like tumor (HPCLT) of the nasal cavity: morphological, immunohistochemical, and ultrastructural features]. / Tumore simil emangioperitocitoma (HPCLT) della cavità nasale: aspetti morfologici, immunoistochimici ed ultrastrutturali.

A case of hemangiopericytoma-like tumour (HPCLT) of the nasal cavity is described in a 81-year-old woman. The tumour, accompanied by symptoms of nasal obstruction and epistaxis, presented as a polypoid lesion filling the left middle meatus region. Histological, immunohistochemical and electron microscope studies revealed a uniform sheet-like proliferation of spindle to oval vimentin-positive cells growing around a rich vascular network. The favourable outcome sixteen months after endoscopic sphenoethmoidectomy would seem to confirm the diagnosis of HPCLT. The differential diagnosis of this rare lesion is discussed.

[Eradicating therapy of Helicobacter pylori in the duodenal ulcer]. / Terapia eradicante dell'Helicobacter pilory nell'ulcera duodenale.

Helicobacter pylori (HP) is the conclusive etiopathogenetic element of peptic lesion. The disappearance of the bacteria from the antral mucosa involves a rapid cicatrization of the ulcer with correlated recidivist, in direct manner, to reinfections or insufficient treatments. The leading problem lies in definitive bacterial eradication, made difficult by the notable resistance of the HP to the antimicrobic agent. The therapeutic solutions require the contemporary use of medicine with a different mechanism of action. A triple medicine scheme has been experimented, with non-contemporary administration of Azithromycin and subsequently of metronidazol for 3 days and a tablet/day of omeprazolo for 30 days. The research was carried out on a sample of 60 units, affected by duodenal ulcer in active phase, with endoscopic and hystologic controls at the beginning and follow up at 60 days. The results show a bacterial eradication which exceed 90%, with regression of the symptomatology in 48-72 hours and side effects of modest entity. The collaboration of the subjects, after informed consent, was total, also favoured by the brevity of the cycle, uniformity of the dosing and easy distribution/day. The data confirm the etiopathogenetic value of the HP in the gastric and duodenal ulcer.

[Bi- and tridimensional reconstruction with spiral computerized tomography in esophageal neoplasms. Preliminary clinical applications]. / Ricostruzioni bi e tridimensionali con Tomografia Computerizzata spirale nelle neoplasie dell'esofago. Applicazioni cliniche preliminari.

Spiral CT allows rapid data acquisition in an entire anatomical region during a single breath-hold and permits to obtain 2D and 3D reconstructions with good diagnostic accuracy. We investigated the capabilities of spiral CT reconstructions in demonstrating esophageal cancers. Spiral CT was performed in 10 patients with esophageal cancers and in 3 patients who underwent endoprostheses placement with surgical or interventional procedures. Bidimensional (MPR, CPR, MIP) and three-dimensional reconstructions with Shaded Surface Display (SSD) were obtained at the end of each exam. In the analysis of the results, CT reconstructions were compared with previous barium esophagography. Some of the main semiologic patterns were considered: tumor visualization, longitudinal extent of the neoplasm and its relationships with adjacent structures. MPRs were obtained in 9 patients and allowed to visualize the esophageal lumen with a filling defect, the neoplastic mass and its longitudinal and extraluminal extent. SSD reconstructions was obtained in 7 patients and allowed a good assessment of longitudinal neoplasm extent in 6 patients. Neoplastic mass visualization and extraluminal tumor extent were difficult to assess in all cases. MIP reconstructions were obtained in all patients and demonstrated longitudinal neoplasm extent with good accuracy. Neo-plastic tissue was visualized only indirectly through the filling defect of the esophageal lumen. MIP allowed to define, in 5 patients, the relationships of the neoplasms with adjacent vascular structures after the intravenous administration of contrast agent. In conclusion, we consider that Spiral CT reconstructions can be useful for a more accurate staging of esophageal cancers.

[Granular cell tumor of the male breast. Case report and review of the literature]. / Tumore a cellule granulari della mammella maschile. Descrizione di un caso e revisione della letteratura.

The authors report a case of granular cell tumor of the breast arising in a 38-year old man. Preoperative diagnosis was of breast carcinoma. However, frozen sections showed the benign nature of the tumour. It is important to identify this lesion as, in spite of a clinical and mammographic similarity with carcinoma, its behaviour is quite benign, and a simple tumour excision is the recommended treatment.