Carmela Emmanuele

Solitary fibrous tumour of the kidney with sarcomatous overgrowth

Solitary fibrous tumour (SFT) rarely occurs in the kidney, with only one case exhibiting malignant behaviour. We report the case of a typical SFT of the kidney with sarcomatous overgrowth in a 34‐year‐old woman. This malignant component, grossly apparent as a nodular area arising in the context of the main tumour mass, consisted of CD34+ mitotically active atypical plump spindle‐ to epithelioid‐shaped cells, including pleomorphic multinucleated giant cells. A novel immunohistochemical finding was diffuse and strong S‐100 protein expression by sarcomatous cells. This should be kept in mind by pathologists to avoid confusion with other S‐100 protein‐positive malignant neoplasms.

Myofibroblastoma of the Breast with Hemangiopericytoma-like Pattern and Pleomorphic Lipoma-like areas : Report of a Case with Diagnostic and Histogenetic Considerations

Myofibroblastoma (MFB) of the breast is an uncommon benign spindle cell tumor which may exhibit a wide spectrum of histological features. We report an unusual case of MFB of the male breast, showing cellular areas with a hemangiopericytoma-like pattern similar to that observed in solitary fibrous tumor (SFT) and extensive fibromyxoid areas containing numerous atypical stromal cells. The association of these atypical cells with mature adipocytes and microcystic and/or myxoid degenerative changes resembled pleomorphic lipoma-like and myxoid liposarcoma-like features, respectively. To our knowledge, these peculiar morphological findings have not been previously reported in MFB of the breast. They should be recognized to avoid confusion with other mesenchymal tumors, especially with hemangiopericytoma, pleomorphic lipoma (PL), spindle-cell lipoma (SCL) and myxoid liposarcoma. A case of MFB of the breast showing morphological features also commonly seen in SFT and PL/SCL is further morphological evidence in support of the speculation that the mesenchymal tumors of the breast, also known under the terms benign spindle cell tumors, fibromas, SFTs, SCLs and MFBs, are histogenetically related lesions.

Correlation of α2ß1 Integrin Expression with Histological Type and Hormonal Receptor Status in Breast Carcinomas

Summary Interactions between cells and extracellular matrix are mediated in part by a family of αs heterodimeric molecules known as integrins. Immunohistochemical studies have shown that benign hyperplastic/neoplastic mammary epithelium expressed high levels of α2sa2#1 collagen/laminin receptor. In contrast, malignant cells of breast carcinoma exhibited marked diminuition or loss of the α2sa2#1 integrin. A correlation has been suggested between the loss of the α2sa2#1 expression and the increased invasiveness of neoplastic cells. This study investigated the expression of α2sa2#1 integrin and its extracellular ligand collagen IV by using monoclonal antibodies on the cryostat section of 124 invasive mammary carcinomas. Two patterns of α2sa2#1 immunoreactivity, i. e. pericellular and basolateral, were identified in breast carcinomas and correlated with their histological type. In most invasive ductal carcinomas of no special type (NOS), integrin staining tended to decrease in both pericellular and basolateral aspects. Loss of basolateral staining for α2sa2#1 integrin corresponded closely to the loss of immunoreactivity for collagen IV Mucinous and medullary carcinomas showed strongly α2sa2#1 pericellular staining, but no basolateral reactivity or collagen IV expression. Only two of the infiltrating lobular carcinomas expressed strong pericellular reactivity. In 82 ductal carcinomas NOS, the abnormally low expressionlabsence of α2sa2#1 integrin correlated with estrogen and progesterone receptor negativity (p The results of the present study suggest that changes in α2sa2#1 expression correlate with the histological type and hormonal receptor status in breast carcinomas. The clinical implications of these findings remain to be elucidated.

Immunohistochemical distribution of S-100 protein and type IV collagen in human embryonic and fetal sympathetic neuroblasts

SummaryThe expression and distribution of S-100 protein and type IV collagen was studied immunohistochemically in sympathetic neuroblasts from the paravertebral region to the adrenal glands in human embryos and fetuses ranging from 7 to 12 weeks gestational age. Prom 7 weeks gestational age, S-100 protein was detected in round or oval cells mingling with sympathetic neuroblasts, and in spindle-shaped cells forming a continuous layer around them. The latter S-100 protein-positive cells were found in contact with the Schwann cells of nerve fibres entering the groups of sympathetic neuroblasts. Staining for type IV collagen showed that all groups of sympathetic neuroblasts were surrounded by a continuous basement membrane. By examining serial sections stained for type IV collagen and S-100 protein, a continuous basement membrane was found along the distribution pattern of the peripheral S-100 protein-positive spindle cells. The morphology of these cells, and their relationships with Schwann cells and with the basement membrane of the sympathetic neuroblasts, indicated that they were Schwann-like cells probably capable of synthesizing a continuous basement membrane separating the neuroblasts from the adjacent tissues. In contrast, the round or oval S-100 protein-positive cells, in contact with the sympathetic neuroblasts and not associated with nerve fibres, were considered as sustentacular or sustentacular precursor cells. At week 7 gestational age, the peri-adrenal sympathetic neuroblasts and their sustentacular and Schwann-like cells started to invade the adrenal glands and mingled with the adrenal cortical cells. These findings suggest the extra-adrenal origin of the sustentacular cells in embryonic and fetal adrenal glands.

Correlated expression of BCL-2 protein, estrogen receptor, cathepsin D and low growth fraction (PCNA) in intracystic papillary breast carcinoma.

The expression of BCL-2 protein was evaluated immunohistochemically in 23 intracystic papillary carcinomas (IPCs) of the breast. Twenty-two patients were female and one male, aged 49-90 years (median 72). Twenty-one cases had a benign behaviour, while two cases developed local recurrence. Of the 23 tumours, 19 (82%) were immunoreactive for BCL-2, the majority of positive carcinomas showing intense cytoplasmic staining of more than 50% neoplastic cells. The intensity of BCL-2 expression was significantly correlated with prognostic markers such as estrogen receptor (ER) positivity (p = 0.001), cathepsin D (CD) reactivity in the neoplastic cells (p = 0.001) and low growth fraction, evaluated by proliferating cell nuclear antigen (PCNA) immunostaining (p = 0.008). An inverse relationship was also found between BCL-2 and p53 protein (p = 0.001). Three cases of high grade (G3) IPC expressed p53, high PCNA index, and CD (the latter only in the stromal cells), but no immunostaining for BCL-2 and ER. Thus, absence of BCL-2 expression in high grade IPC was associated with ER-negative, rapidly proliferating and p53-positive immunophenotype. All high grade tumours showed invasion of the cystic wall. Local recurrence developed in one of these. The authors conclude that BCL-2 immunoreactivity in IPC is related with tumour grade and with a range of molecular markers of favourable prognosis such as ER positive status, CD expression in the neoplastic cells, and low PCNA index. These findings are consistent with the indolent clinical course and the very favourable prognosis of IPC of the breast.

Distribution of extracellular matrix glycoproteins in the human mesonephros

We analyzed the expression and distribution of collagen types IV and VI, laminin and fibronectin during the development and regression of the mesonephros in human embryos and fetuses ranging from 6 to 12 weeks of gestation by indirect immunoperoxidase methods. Type IV collagen, laminin and fibronectin were detected along the glomerular, tubular and capsular basement membranes of developing and mature nephrons. Only type IV collagen and fibronectin were found in the mesangium. Type VI collagen formed a delicate interstitial fibrillar network and a continuous basement membrane-like structure along the mesonephric nephrons. Basement membranes (GBM) of developing and mature glomeruli showed a distinct continuous staining for this collagen. The mesangial matrix was rich in type VI collagen. Mesonephric involution started during the 8th week of gestation and coincided with a moderate expansion of mesangial matrix and progressive collapse of the capillary walls, while the tubules became thinner and shorter. Staining for all extracellular matrix glycoproteins studied showed GBM wrinkling, gradual disintegration of some capillary loops and glomerulosclerosis. The sclerotic glomeruli were strongly positive for type IV collagen and less positive for type VI collagen and fibronectin. Laminin was absent. Our results indicate that collagen types IV, VI, laminin and fibronectin may be involved in the development and regression of the human mesonephros.

Ameloblastoma of the Sinonasal Tract: Report of a Case with Clinicopathologic Considerations

Ameloblastomas are locally aggressive jaw tumours with a high propensity for recurrence and are believed to arise from remnants of dental lamina or odontogenic epithelium. Extragnathic ameloblastomas are unusual, and primary sinonasal tract origin is very uncommon with few cases reported in the literature. We herein report a case of primary sinonasal ameloblastoma presented in a 74-year-old male with nasal obstruction, rhinorrhoea, and sinusitis. Nasal endoscopy showed the right nasal cavity completely obstructed by a polypoid lesion attached to the lateral nasal wall. A preoperative CT scan was performed showing a solid lesion, measuring 2 cm in the maximum diameter, extending from the nasopharynx area with obstruction of the ostiomeatal unit and sphenoethmoidal recess into the lateral pharyngeal space, laterally to the parotid, without continuity with maxillary alveola and antrum. The tumour was completely excised endoscopically, and a final diagnosis of ameloblastoma was rendered. At the 12-month followup, there was no evidence of recurrence.

Primary laryngeal localization of multiple myeloma: A case report

Multiple myeloma is a lymphoproliferative disease that may involve the bone marrow as well as extramedullary soft tissues. However, laryngeal localization of multiple myeloma is extremely rare. We herein present the case of a 68-year-old male patient with a history of dyspnea, dysphonia and dysphagia. Laryngoscopic examination revealed a lesion involving the right glottis and right vestibular (false) vocal fold, with absence of ipsilateral laryngeal motility and constriction of the airway. Computed tomography and magnetic resonance imaging revealed a gross swelling infiltrating the right glottis and right false vocal fold, sized 33×19×33 mm, with sub-centimeter laterocervical lymph nodes bilaterally. Careful integration of the clinical manifestations with the radiological and pathological data led to the diagnosis of multiple myeloma. Given the rarity of this localization, the purpose of this study was to increase knowledge of this disease among ear, nose and throat specialists, in order to enable a more timely diagnosis.

Diagnostic Consideration for Sinonasal Wegener’s Granulomatosis Clinically Mistaken for Carcinoma

We report a case of Wegeners granulomatosis clinically mistaken for carcinoma in a 21-year-old girl presenting with an ulcerated mass of the nasopharynx associated with enlarged laterocervical nodes. The lesion was clinically suspected as malignant on the basis of clinical and radiological findings (namely, computed tomography scan and positron emission tomography). However, multiple biopsies were not conclusive for malignancy showing histological change suggestive of Wegeners granulomatosis. A serum determination of cANCA supported the diagnosis of Wegeners granulomatosis. Clinical findings and image studies suggested an erroneous diagnosis of malignancy whereas a definitive diagnosis of Wegeners granulomatosis was achieved only after repeated biopsies thus leading to a correct therapeutic approach. The Wegener granulomatosis must be added to the list of the differential diagnoses of the masses of the nasopharynx associated with or without enlarged laterocervical nodes.

Paratesticular Pleomorphic Rhabdomyosarcoma in the Elderly An Immunohistochemical, Ultrastructural Study and Review of the Literature

A case of paratesticular pleomorphic rhabdomyosarcoma, presenting as an ulcerated mass in the left hemiscrotum of an 83-year-old man, is reported. By light microscopy, this neoplasm could not be distinguished from other pleomorphic sarcomas, such as malignant fibrous histiocytoma, leiomyosarcoma, and liposarcoma. Immunohistochemistry showed that most tumor cells were strongly reactive with antibodies directed against myoglobin, desmin, a-sarcomeric actin, and myogenin, whereas x-smooth muscle actin expression was less extensive. Electron microscopic examination demonstrated thick and thin filaments with condensations of electron-dense material reminiscent of structures of Z-lines in the large pleomorphic cells. On the basis of these findings, the neoplasm was classified as a pleomorphic rhabdomyosarcoma. According to a review of the literature, this is the first well-documented example of paratesticular pleomorphic rhabdomyosarcoma in an elderly person.