Epithelial Membrane Antigen Staining Research Articles

Sinonasal undifferentiated carcinoma: immunohistochemical profile and lack of EBV association.

The role of Epstein-Barr virus (EBV) in the development of sinonasal undifferentiated carcinoma (SNUC) remains unresolved. Reports of EBV-positivity in SNUC may reflect inclusion of lymphoepithelioma-like carcinomas within this group. In addition, SNUC have been incompletely characterized immunohistochemically, and their undifferentiated appearance often requires such ancillary studies to aid in their distinction from other high-grade neoplasms. To address these two issues, 25 cases of SNUC diagnosed between the years 1983 and 1999 were selected from our files. EBER in situ hybridization (ISH) was performed on the paraffin-embedded tissue by using 3H-labeled EBER-1 RNA probes. Neoplasms with sufficient tissue (22 of 25) were evaluated immunohistochemically for Ki-67, p53, chromogranin, synaptophysin, placental alkaline phosphatase (PLAP), CD99, carcinoembryonic antigen (CEA), epithelial membrane antigen (EMA), neuron-specific enolase (NSE), and latent membrane protein-1 (LMP-1). The median patient age was 58 years (range, 20-81 years), with a male-to-female ratio of approximately 3:1. The most common tumor location was the nasal cavity (18 cases), followed by the ethmoid and maxillary sinuses. Median survival was 18 months. All 25 tumors were negative for EBER-I by ISH. Ki-67 was negative in one case, 1+ in nine, 2+ in six, 3+ in five, and 4+ in one. P53 was negative in nine, 1+ in five, 2+ in two, 3+ in none, and 4+ in six. CD99 expression was strongly positive in 3 of 22 (14%) and completely negative in the remainder. Variably intense focal staining for EMA was present in 4 of 22 (18%). NSE faintly stained 4 of 22 (18%). Chromogranin, synaptophysin, PLAP, CEA, and LMP-1 were negative (0 of 22). Our results suggest that EBV does not play a role in the development of SNUC. Strict histologic criteria are necessary to avoid confusion with lymphoepithelioma-like carcinoma or other high-grade malignancies in this region. The finding of occasional CD99-positive cases adds SNUC to the growing list of CD99-positive neoplasms.

EMA: a differentiation antigen related to node metastatic capacity of breast carcinomas.

Epithelial membrane antigen (EMA), also known as MUC1, is a mucinous glycoprotein fixed to the luminal domain of the epithelial cell membrane of normal breast ducts. However, in breast cancer cells, it is usually dispersed in the cytoplasm. EMA staining patterns of 330 breast carcinomas were examined, and three groups formed: lineal (16%), cytoplasmic (75%), and negative (9%). Although these patterns were somewhat related to histological cancer types, this was not statistically significant. However, EMA showed statistically significant univariate relationships to tumor grade, tumor size, estrogen and progesterone receptors, and nodal stage. Logistic regression analysis showed that among these variables, all of which were univariately related to node metastasis, only tumor size and EMA were independent nodal stage predictors. A combined analysis of these two factors revealed that the statistical probability of a tumor metastasizing to four or more nodes increased in each tumor size group from 0.9% to 12% for pT1, from 2% to 29% for pT2 and from 10% to 63% for pT3, depending on the EMA staining. The tumors showing a lineal pattern were the least metastasizing, while the EMA-negative tumors were the most. After recognizing these relationships between EMA staining patterns and other well-known differentiation markers and the lymph node metastatic capacity of carcinomas, and considering the results obtained by others on survival, one might conclude that EMA is both a differentiation marker and a histological prognostic factor.

Perineurial cell differentiation in neurofibromas. Report of eight cases including a case with composite perineurioma-neurofibroma features.

Among 99 cases of neurofibroma (NF), eight tumors (8%) contained epithelial membrane antigen (EMA)-positive perineurial cells inside the lesions. These cells were numerous and represented a significant part of the tumor cell population. In case 7, EMA-positive cells represented approximately half of the tumor. These patients' ages ranged from 23 to 73 years (average 51 years). Six patients were females and two were males. Neurofibromatosis type 1 was present in one case. The histological types of neurofibromas with EMA-positive cells were as follows: cutaneous, well-circumscribed, localized type in four cases; cutaneous diffuse type in one case; subcutaneous, well-circumscribed type in two cases; and subcutaneous plexiform type in one case. Perineurial cell differentiation was suspected by examining routinely stained sections in four cases; it was unsuspected in four cases. The perineurial cells were arranged in a pattern similar to that of "pure" perineurioma. They possessed thin bipolar processes and were arranged in laminar fascicles and whorls. In cellular areas, the cytoplasm was more eosinophilic and cell borders were poorly visible. In contrast, when the stroma was myxoid, it accentuated the shape of individual cells. As these morphological features of perineurial cell differentiation in NF are not entirely specific, EMA staining is recommended to prove the perineurial cell differentiation in neurofibromas. An additional immunohistochemical result of this study is the presence of numerous CD34-positive cells in all neurofibromas, which is similar to previous studies.

Chondrosarcoma of the base of the skull: a clinicopathologic study of 200 cases with emphasis on its distinction from chordoma.

Conventional chondrosarcoma (CSA) of the skull base is an uncommon neoplasm that can resemble chordoma, and indeed it is misdiagnosed frequently as such. This has important clinical implications, because when treated with similar aggressive treatment strategies, CSA has a much better prognosis than chordoma. In an effort to identify those morphologic and immunohistochemical features that help to identify conventional skull base CSA correctly and to understand its prognosis better, particularly compared with chordoma, when treated with surgery and proton beam irradiation, the authors performed a clinicopathologic analysis of 200 CSAs. The patients ranged in age from 10 to 79 years (mean, 39 years), 87 patients were male and 113 patients were female, and most presented with symptoms related to the central nervous system. Approximately 6% of the tumors arose in the sphenoethmoid complex, 28% originated in the clivus, and 66% developed in the temperooccipital junction. Histologically, 15 tumors (7.5%) were classified as hyaline CSA, 59 (29.5%) as myxoid CSA, and 126 (63%) as mixed hyaline and myxoid CSA. A total of 101 (50.5%) tumors were grade 1, 57 (28.5%) had areas of grades 1 and 2, and 42 (21%) were pure grade 2 neoplasms. The vast majority of patients originated from referring hospitals, and the diagnosis was changed prospectively at our institution to CSA from chordoma in 74 patients (37%). Of the tumors studied immunohistochemically, 96 of 97 (98.9%) stained for S-100 protein, 0 of 97 (0%) stained for keratin, and faint staining for epithelial membrane antigen was seen in 7 of 88 tumors (7.95%). All patients underwent high-dose postoperative fractionated precision conformal radiation therapy with a dose that ranged from 64.2 to 79.6 Cobalt-Gray-equivalents (median, 72.1 Cobalt-Gray-equivalents, given in 38 fractions. The 200 patients had a median follow-up of 63 months (range, 2.1 mos - 18.5 yrs). Tumor control was defined as lack of progression by clinical and radiographic assessment. Based on this definition, there were three local recurrences, and two of these patients died of tumor-related complications. The 5- and 10-year local control rates were 99% and 98% respectively, and the 5- and 10-year disease-specific survival rates were both 99%. In contrast to CSA, the 5- and 10-year survival rates of chordoma have been reported to be approximately 51 % and 35% respectively, and in our institution intensive treatment has resulted in 5- and 10-year progression-free survival rates of 70% and 45% respectively. CSA of the skull base can be distinguished reliably from chordoma, and this distinction is important because skull base CSA has an excellent prognosis when treated with surgery and proton beam irradiation, whereas chordomas have a substantially poorer clinical course despite similar aggressive management.

The use of histological and immunohistochemical markers to distinguish pleural malignant mesothelioma and in situ mesothelioma from reactive mesothelial hyperplasia and reactive pleural fibrosis.

Distinguishing malignant mesothelioma from reactive mesothelial hyperplasia and reactive fibrosis can be a diagnostic problem in small pleural biopsies, made more difficult by the recent recognition of mesothelioma-in-situ. Antibodies to epithelial membrane antigen (EMA), p53, and bcl-2 have all been advocated for differentiating reactive from neoplastic conditions, but reports are inconsistent. These antibodies have therefore been applied to 31 cases of malignant mesothelioma, 34 cases of reactive pleural disease (20 reactive mesothelial hyperplasia and 14 reactive pleural fibrosis) and four small biopsies that were initially coded as suspicious, from patients who later developed frank mesothelioma. Thirty out of 31 (97 per cent) cases of mesothelioma showed positive nuclear staining for p53, with a higher incidence of positivity in epithelioid than in sarcomatoid elements and 30/31 (97 per cent) showed diffuse linear membrane staining for EMA, again more intense in the epithelioid elements. No mesothelioma was positive for bcl-2. In seven cases that contained both in situ and invasive mesothelioma, the in situ elements showed similar staining patterns to the invasive epithelioid elements. Thirteen out of 20 (65 per cent) cases of reactive mesothelial hyperplasia showed occasional nuclear positivity for p53 and 5/20 (25 per cent) cases showed focal weak membrane staining for EMA. Three out of 14 (21 per cent) cases of reactive pleural fibrosis showed positive nuclear staining for p53 and 6/14 (43 per cent) cases showed focal membrane staining with EMA. No reactive cases stained for bcl-2. All four suspicious cases showed diffuse linear staining with EMA and three showed focal staining for p53. It is concluded that strong diffuse linear staining for EMA is a good marker of malignancy when differentiating epithelioid malignant mesothelioma and mesothelioma-in-situ from reactive mesothelial hyperplasia, although weak focal staining may occur in reactive conditions. Nuclear staining for p53 is also suggestive of epithelioid mesothelioma, but should be regarded as no more than suspicious. The antibodies used in this investigation are less helpful in differentiating sarcomatoid mesothelioma from reactive pleural fibrosis.

Immunohistochemical staining for androgen receptors: a sensitive marker of sebaceous differentiation.

Androgen receptors (AR) are present in normal skin being localized to the basal and differentiating cells of the sebaceous gland, and as such, sebaceous glands are androgen sensitive tissue. Androgen receptor expression was examined in 43 sebaceous neoplasms including 8 sebaceous carcinomas, 22 sebaceous adenomas, 12 specimens showing sebaceous hyperplasia, and 1 sebaceous epithelioma, as well as in 14 squamous cell carcinomas, 2 clear cell acanthomas, and 35 basal cell carcinomas. Epithelial membrane antigen (EMA) expression was also examined in all of the sebaceous neoplasms. All specimens were fixed in formalin and embedded in paraffin. Diffuse positive nuclear androgen receptor antibody immunohistochemical staining was observed in all samples of sebaceous neoplasms, whereas approximately 60% of basal cell carcinomas showed only focal positivity for nuclear androgen receptor immunoreactivity. Clear cell acanthomas and squamous cell carcinomas were uniformly negative. Whereas all sebaceous neoplasms exhibited immunoreactivity for androgen receptors, the staining pattern was more marked in the nuclei of seboblasts and differentiating sebocytes in the adenomatous, hyperplastic, and epitheliomatous lesions than in the nuclei of the less differentiated sebaceous carcinoma cells. All the sebaceous neoplasms except for sebaceous carcinomas exhibited immunoreactivity for EMA. In the sebaceous carcinomas, EMA staining was absent in the most poorly differentiated specimen, but with increasing differentiation, the carcinomas became immunoreactive to EMA. We have shown that the nuclei of sebaceous neoplasms, including sebaceous gland carcinomas, show immunoreactivity for androgen receptors (AR), that immunohistochemical staining for the presence of AR may be a reliable marker of sebaceous differentiation, and that the AR may be a better marker of sebaceous differentiation than EMA, particularly in poorly differentiated sebaceous carcinomas.

Primary anaplastic large cell lymphoma of the central nervous system

Central nervous system (CNS) involvement is extremely rare in anaplastic large cell lymphoma (ALCL), and in children only isolated cases have been reported, mainly as secondary CNS involvement. A case of fatal primary ALCL of the brain in a 13-year-old white boy is reported. Magnetic resonance imaging of the brain showed decreased absorption in T1- and T2-weighted image showed a hyperintense signal in the right parietal lobe and 2 masses in the right frontal lobe. A frontal lobe biopsy showed a pleomorphic neoplasm diffusely infiltrating the brain parenchyma and composed of large cells with bizarre, often polylobated or horseshoe-shaped nuclei. Immunohistochemical stains showed diffuse strong positivity for CD30, anaplastic lymphoma kinase protein (ALK-1), p80, leucocyte common antigen, CD45RO (UCHL1), and focal staining for epithelial membrane antigen. Immunostainings for cytokeratins, monocyte-macrophage, and B-cell markers were negative. Epstein-Barr virus latent membrane protein was not detected. To the best of our knowledge, there is only 1 case of primary ALCL of the brain in childhood previously reported in the literature. Before the biopsy, both cases were clinically misdiagnosed as mycobacterial CNS infection. Therefore, primary ALCL should also be included in the differential diagnosis when a mycobacterial CNS infection is suspected in pediatric patients; a careful cytological evaluation of the cerebrospinal fluid or cerebral biopsy are essential for an accurate diagnosis.

‘Sclerosing’ Perineurioma: A Tumor Variant?

We describe two examples of soft tissue perineurioma, a hard tumor, 1.7 cm in diameter, involving the dermis and superficial subcutaneous tissue of the left thumb, and the other a massive, lobulated lesion of the pelvis. Both occurred in 49-year-old men. Both were mobile and well demarcated but unencapsulated. Their cut surfaces were white and collagenous. Microscopically, each consisted of spindle-shaped or epithelioid tumor cells in a dense stroma of haphazardly oriented collagen bundles. Spindle-shaped cells were individually distributed, whereas epithelioid cells were often arranged in cords or clusters. Cytologic atypia was conspicuous in the massive tumor, but no mitotic activity was observed. Immunostains showed diffuse reactivity for vimentin, membrane pattern staining for epithelial membrane antigen and type IV collagen, as well as patchy CD34 reactivity. Stains for S-100 protein, Leu-7, smooth muscle actin, desmin, and cytokeratin were negative. Ultrastructural studies showed perineurial cell characteristics, including slender cytoplasmic processes, numerous pinocytotic vesicles, occasional tight junctions, and discontinuous surface basement membrane. Perineuriomas featuring marked stromal sclerosis often occur in the hands of young adults, as has recently been reported; the authors considered it a clinicopathologic variant. Herein we report two sclerotic perineuriomas, one a small tumor of the thumb and the other a centrally situated, massive pelvic tumor with sclerosis and degenerative cytologic atypia. We believe that exaggerated collagen deposition in perineuriomas, occasionally associated with degenerative nuclear changes, is not a manifestation of a unique, clinically relevant tumor variant but rather a manifestation of chronicity akin to that seen in “ancient schwannoma” and ‘atypical neurofibroma.’

Chordoid glioma of the third ventricle: Confirmatory report of a new entity

The term “chordoid glioma” was recently introduced to denote a circumscribed, apparently low-grade neoplasm arising in or preferentially involving the third ventricle of middle-aged women. We report biopsy and postmortem findings in a 60-year-old woman with symptoms of forgetfulness, headache, and lethargy. Neuroimaging showed a contrast-enhancing third ventricular mass with obstructive hydrocephalus. The tumor was subtotally resected. Microscopically, it consisted of clusters and strands of epithelioid cells in a mucoid matrix. Its margins were remarkably discrete and showed little tendency to infiltrate surrounding brain parenchyma. The majority of neoplastic cells were glial fibrillary acidic protein (GFAP) and vimentin positive, whereas S100 protein labeled only individual cells. Stains for epithelial membrane antigen (EMA) and cytokeratin were nonreactive. There was no evidence of neuroendocrine differentiation or expression of estrogen and progesteron receptors. Lymphoplasmacellular infiltrates were noted throughout the lesion and at the tumor-brain interface. The MIB-1 labeling index averaged 1.5%. At present, chordoid glioma is considered a glial neoplasm of uncertain histogenesis with distinct clinicopathologic features.

Immunohistochemical distinction of ocular sebaceous carcinoma from basal cell and squamous cell carcinoma.

Diagnosis of sebaceous carcinoma of the periorbital region is often delayed. Clinically, this lesion can mimic several inflammatory disorders. Histopathologically, it can mimic either squamous cell or basal cell carcinoma. To identify an immunohistochemical approach to assist in the diagnosis of periorbital sebaceous carcinoma. The immunohistochemical profiles of several cases of periorbital sebaceous, basal cell, and squamous cell carcinoma were examined. Although at least focal epithelial membrane antigen (EMA) staining can effectively distinguish sebaceous carcinoma (10 of 11 were positive) from basal cell carcinoma (1 of 16 were positive), most squamous cell carcinomas examined were also focally EMA positive (11 of 14). However, Cam 5.2 reactivity was seen in most sebaceous carcinomas (8 of 11) but no squamous cell carcinomas (0 of 14). In addition, at least focal BRST-1 reactivity was also seen in most sebaceous carcinomas (7 of 11) but no basal cell carcinomas (0 of 16). Periorbital sebaceous, basal cell, and squamous cell carcinomas have different immunohistochemical staining profiles; a panel of commonly available antibodies, including anti-EMA, BRST-1, and Cam 5.2, may help distinguish these diseases from each other when that distinction cannot be clearly made by light microscopy alone.

Microcalcifications in the anterior pituitary gland of the fetus and the newborn: A histochemical and immunohistochemical study

Calcified concretions are a normal and constant finding in the anterior pituitary gland of fetuses and newborns. Their light and electron microscopic characteristics have been recently reported by the authors. In this study, undecalcified and decalcified sections from 20 neonatal and 60 fetal anterior pituitary glands were studied by histochemical and immunohistochemical methods to further clarify their nature and mechanism of formation. All the glands revealed homogeneous and/or laminar calcifications located either within the interstitium or follicular structures. They were composed of a diastase-resistant periodic acid-Schiff—positive carbohydrate-rich matrix. The Feulgen method for DNA was negative. Their core frequently reacted to Alcian blue and epithelial membrane antigen (EMA). EMA also stained the apical membranes of adjacent epithelial cells. Other immunostains (vimentin, keratin, and pituitary hormones) were negative. The positive staining for Alcian blue and EMA and the negative staining with the Feulgen method for DNA suggest that the core of the calcifications consists of acidic mucosubstances and EMA-positive proteinaceous material previously secreted by viable pituitary cells. The EMA-negative periphery of the concretions probably develops from further extracellular peripheral mineralization that leads to larger, sometimes laminated psammoma bodies. The occurrence of pituitary calcifications in states of adult physiological and pathological hyperprolactinemia suggests that the marked proliferation of lactotrophs occurring during the fetal life play an important role in the pathogenesis of the fetal and neonatal concretions.

UNILATERAL ADRENAL HETEROTOPIA WITH RENAL-ADRENAL FUSION

An 83-year-old white woman presented for evaluation of diffuse upper abdominal pain and weight loss. Physical examination demonstrated no tenderness or abdominal masses. Blood pressure was normal. Computerized tomography of the abdomen and pelvis revealed a 2.9 X 2.2 cm. solid mass in the upper pole of the right kidney. Laboratory studies, including renal function, hormonal evaluation and hematological parameters, were normal, and urinalysis was negative. Upper pole partial nephrectomy was performed en bloc with the adrenal gland through a flank incision. Histological examination of the specimen was remarkable for fusion of the adrenal gland with the kidney and adrenal cortical clear cells admixed with renal parenchyma (part A of figure). Renal cystic changes were noted at the junction of the fusion. Immunohistochemical stains for epithelial membrane antigen (part B of figure), pan cytokeratin and cytokeratin 7 were positive in the renal tissue, and negative in the extrarenal and intrarenal adrenal tissue, confirming heterotopia. The patient did well postoperatively. DISCUSSION

T-cell rich, Ki-1-positive post-transplant lymphoproliferative disorder: A previously undescribed variant following liver transplant

Post-transplant lymphoproliferative disorders (PTLD) are a consequence of the immunosuppressive therapy following organ transplant. We describe a patient who developed PTLD seven years after liver transplant and while receiving cyclosporine and prednisone. Magnetic resonance imaging demonstrated a paraspinal mass extending from T11 to L1. Microscopically, this was composed of a diffuse infiltrate of small to intermediate sized T-lymphocytes with clusters of large anaplastic tumor cells with amphophilic cytoplasm, large irregular nuclei and prominent nucleoli. A high mitotic rate and atypical mitotic figures were noted in the clusters of large cells. Flow cytometric and immunohistochemical analysis failed identify either a monoclonal B-cell population or a T-cell population with aberrant expression of the T-cell surface markers. Strong positivity for CD30 and focal staining for epithelial membrane antigen (EMA) of the large cells was seen. Leukocyte common antigen (LCA), cytokeratin, vimentin, monocyte/macrophage and B- and T-markers were negative. The small lymphoid cells were positive for CD3, MT-1 and UCHL-1. Based on the immunophenotypic and morphological evaluation, this was characterized as a T-cell rich PTLD. PCR analysis identified a monoclonal population of B-cells. This unusual case emphasizes the morphological and immunophenotypic diversity of PTLD. The utility of PCR analysis in the evaluation of PTLD is also demonstrated.

Thymoma with pseudosarcomatous stroma: report of an unusual histologic variant of thymic epithelial neoplasm that may simulate carcinosarcoma.

Six cases are described of an unusual type of primary thymic epithelial neoplasm characterized by a biphasic epithelial/spindle cell morphology that closely resembled a carcinosarcoma. The patients were two women and four men 28-70 years of age. The tumors presented clinically as asymptomatic anterior mediastinal masses found incidentally on routine chest radiographs. All patients were treated by complete surgical excision. Grossly, the tumors consisted of well-circumscribed, encapsulated masses that measured 6-14 cm in greatest diameter and showed a gray-white, homogeneous, rubbery cut surface. Histologically, the lesions were composed of anastomosing islands and cords of oval to polygonal epithelial cells displaying large nuclei with occasional prominent nucleoli and rare mitotic figures, separated by areas containing a highly cellular spindle cell proliferation without nuclear atypia. Thymic remnants could be identified in the periphery of the lesions in four cases. Immunohistochemical stains showed diffuse strong positivity for keratin and focally for epithelial membrane antigen (EMA) in the epithelial cell component, and strong positivity for vimentin and focally for actin in the spindle cell stromal component. Stains for keratin, EMA, desmin, S-100 protein, and CD34 were negative in the spindle stromal cells in all cases except one, in which EMA positivity was present; CD5 stains were negative in the epithelial cells in all cases examined. Electron microscopic examination in one case showed well-formed desmosomes and tonofilaments in the epithelial elements, as well as features indicative of fibroblastic differentiation in the spindle stromal cells. Because of the unusually florid spindle cell stromal component and the focally atypical features of the epithelial cells, some of these tumors initially were misinterpreted as examples of carcinosarcoma. Clinical follow-up in five cases showed that the patients were alive and without evidence of disease over a period of 5-20 years (mean follow-up 10 years), suggesting a benign or very low grade malignant biologic behavior. The present cases appear to represent an unusual, previously undescribed morphologic variant of thymoma characterized by a prominent pseudosarcomatous stromal component. Because of the distinctive histologic appearance and indolent clinical behavior, these lesions should be distinguished from other more aggressive anterior mediastinal neoplasms displaying a biphasic morphology.

Fine needle aspiration cytodiagnosis of primary and metastatic squamous cell carcinoma of the breast.

To assess the diagnosis of squamous cell carcinoma involving the breast by fine needle aspiration cytology in four cases (two primary and two metastatic). All patients were women between the ages of 42 and 74 years. The aspiration findings were confirmed by cell blocks of the aspirates, biopsy and/or comparison of the tissue from the previous primary. The aspirate was obtained using multiple passes in the breast masses while maintaining negative pressure. The cytohistologic features in all cases were characterized by numerous malignant squamous cells with hyperchromatic, enlarged, dense nuclei; thickened nuclear membranes; coarse chromatin; abundant eosinophilic, keratinizing glassy cytoplasm; and a necrotizing background with keratin debris. The diagnostic value of immunocytochemical study was considered questionable despite immunopositivity for cytokeratin and epithelial membrane antigen and negative staining for carcinoembryonic antigen and B72.3 in all cases. The identification of malignant squamous cells in breast aspirates is an important observation. Such cells, when seen as the predominant ones in an aspirate, should prompt a search for other sources of a primary tumor before a diagnosis of squamous cell carcinoma of the breast is accepted.

Collecting duct carcinoma of the kidney: a report of three cases and review of the literature

Collecting duct carcinoma (CDC) of the kidney is a rare neoplasm arising from the medullary collecting ducts. The clinicopathological features of three cases are here presented to add to the 42 cases reported so far in the English language literature. Hematuria is the most common presenting symptom, although 10% of patients present with metastatic disease, often in neck lymph nodes. The tumor mass is typically centred on the medulla of the kidney and extends into the cortex. Radiological diagnosis may be difficult because the radiological features of CDC are poorly described. The histological appearances have been described as tubulo-papillary, tubular, solid and sarcomatoid. Intracytoplasmic mucin may be present. The typical immunohistochemical profile is positive staining with antibodies for low and high molecular weight keratin and epithelial membrane antigen (EMA) and positive staining with the lectin Ulex europaeus agglutinin. Literature reports of cytogenetic characterisation show loss of chromosomes resulting in monosomies. Prognosis is poor as more than half of the reported cases have developed metastases or died within two years of presentation.

Primary intrapulmonary meningiomas: A clinicopathologic and immunohistochemical study of ten cases

BACKGROUND Primary intrapulmonary meningiomas are rare, and their occurrence has been reported in the literature only sporadically. METHODS The clinical, pathologic, and immunohistochemical features of ten cases of primary intrapulmonary meningioma were reviewed. RESULTS The patients were 4 women and 6 men between the ages of 30 and 72 years (mean age: 51 years). In nine patients, the lung tumor was found during a routine chest radiographic study. The only patient with clinical symptomatology was a man who presented with a persistent cough of 45 days' duration. None of the patients had a previous history of central nervous system (CNS) meningioma or other tumor at the time of diagnosis. Macroscopically, the neoplasms were described as soft, white, well circumscribed lesions that ranged in size from 1.5 cm to 4 cm in greatest dimension. No predilection for any particular lobe or segment of lung was observed. Histologically, all the tumors showed the characteristic growth pattern of CNS meningiomas and were classified accordingly. Seven tumors were transitional meningiomas, and three were fibrous meningiomas. Psammoma bodies were observed in 50% of the tumors. Immunohistochemically, six cases showed positive staining for Epithelial Membrane Antigen (EMA) and vimentin, whereas two of these cases also showed focal positive staining for CD34. Clinical follow-up ranging from 1 month to 24 years was obtained for 6 patients. All the patients were alive and well except for 1 female who died 20 years after the initial diagnosis at age 92 of a cerebrovascular accident. CONCLUSIONS The clinical follow-up in this study suggests that these tumors are amenable to surgical resection, and when the tumor is resected in its entirety, the patient is cured. In addition, the immunohistochemical results suggest that, as in CNS meningiomas, EMA and vimentin are the most reliable immunologic markers for these tumors.Cancer 1996;78:2328-33.

Primary intrapulmonary meningiomas: A clinicopathologic and immunohistochemical study of ten cases

Primary intrapulmonary meningiomas are rare, and their occurrence has been reported in the literature only sporadically. The clinical, pathologic, and immunohistochemical features of ten cases of primary intrapulmonary meningioma were reviewed. The patients were 4 women and 6 men between the ages of 30 and 72 years (mean age: 51 years). In nine patients, the lung tumor was found during a routine chest radiographic study. The only patient with clinical symptomatology was a man who presented with a persistent cough of 45 days' duration. None of the patients had a previous history of central nervous system (CNS) meningioma or other tumor at the time of diagnosis. Macroscopically, the neoplasms were described as soft, white, well circumscribed lesions that ranged in size from 1.5 cm to 4 cm in greatest dimension. No predilection for any particular lobe or segment of lung was observed. Histologically, all the tumors showed the characteristic growth pattern of CNS meningiomas and were classified accordingly. Seven tumors were transitional meningiomas, and three were fibrous meningiomas. Psammoma bodies were observed in 50% of the tumors. Immunohistochemically, six cases showed positive staining for Epithelial Membrane Antigen (EMA) and vimentin, whereas two of these cases also showed focal positive staining for CD34. Clinical follow-up ranging from 1 month to 24 years was obtained for 6 patients. All the patients were alive and well except for 1 female who died 20 years after the initial diagnosis at age 92 of a cerebrovascular accident. The clinical follow-up in this study suggests that these tumors are amenable to surgical resection, and when the tumor is resected in its entirety, the patient is cured. In addition, the immunohistochemical results suggest that, as in CNS meningiomas, EMA and vimentin are the most reliable immunologic markers for these tumors.

Surgical treatment of clival chordomas: the transsphenoidal approach revisited.

This is a report of 12 cases of clival chordomas that were surgically treated at the Catholic University Medical School, Rome, Italy, over a 7-year period. The study emphasizes the role of the transsphenoidal approach. The study group included seven men and five women whose ages ranged from 26 to 80 years (mean 49.8 years). Diplopia was the most common presenting symptom (eight cases). The tumor involved the upper and middle clivus in five cases, the middle clivus in five, and the lower clivus in two cases. One patient developed spinal metastasis. On histological examination, eight cases proved to be typical chordomas, three cases had a chondroid component, and one case of chordoma had atypical features. Immunohistological staining for vimentin and epithelial membrane antigen was positive in all cases. Follow-up periods ranged from 14 to 86 months (mean 40.2 months). The primary treatment consisted of surgery. Ten patients with chordomas of the upper and middle clivus underwent a total of 13 transsphenoidal procedures. Total tumor removal was achieved in seven cases, subtotal removal in two, and partial removal in one case. In the two cases of lower clival chordomas, total removal was accomplished in one and partial removal in the other. After total removal, no recurrence was noted at 14 to 86 months (mean 37.5 months). In the cases undergoing operation via a transsphenoidal approach, there was zero morbidity and one cerebrospinal fluid fistula that resolved without surgery. The tumor recurred in two patients after subtotal and partial removal, respectively. The authors opted to reoperate in cases of recurrence. Postoperative radiotherapy was administered in only two cases in which further surgery was not indicated because of medical reasons or because such a procedure was contrary to the patient's wishes. When mortality and morbidity rates of this group are compared to those of chordoma patients who were treated with extensive skull-base surgery, the results prompt a reappraisal of the transsphenoidal approach in the treatment of clival chordomas.

Ringlike structures in fine needle aspirates from the breast

Spherical ringlike structures of various sizes resembling Liesegang rings (LRs) are described in four fine needle aspirates (FNAs) from breast lesions over a period of 13 years. A characteristic finding in these structures was a distinct double layer outer wall with striations and an amorphous central nidus. Under polarized light they were non-refractile and no birefringence was noted in Congo red. Immunohistochemical stains for calcium, iron, mucus, glycogen, amyloid, cytokeratin and epithelial membrane antigen in all the cases were found to be negative. Since LRs can be mistaken for ova or parasites, their presence in aspirates of breast should be kept in mind to avoid misdiagnosis.