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S Suster

Publications and source records attributed to S Suster.

At least 91 records · Page 5Linked to original sources

Mediastinal yolk sac tumors associated with prominent multilocular cystic changes of thymic epithelium: a clinicopathologic and immunohistochemical study of five cases.

Five cases of yolk sac tumor of the anterior mediastinum associated with prominent thymic cystic changes are presented. The patients were five men between the ages of 17 and 22 years (mean, 19.5 yr). Clinically, four patients presented with symptoms of chest pain and shortness of breath, whereas the fifth was asymptomatic: his tumor was discovered during a routine physical examination. None of the patients had a history of previous tumor elsewhere. Grossly, the tumors were described as cystic lesions measuring between 8 and 12 cm in greatest dimension. Histologically, the tumors showed prominent cystic changes reminiscent of those observed in acquired multilocular thymic cysts, i.e., the presence of cystic cavities lined by squamous epithelium with prominent inflammatory changes, lymphoid hyperplasia, cholesterol cleft granulomas, and remnants of thymic tissue in the walls of the cysts. In addition, there was a neoplastic cellular proliferation composed of small cells with scant cytoplasm, round nuclei, and inconspicuous nucleoli adopting a reticular pattern of growth and, in some areas, embedded in abundant myxoid stroma. Numerous intra- and extracellular hyaline globules, as well as Schiller-Duval bodies, could also be identified. Immunohistochemical studies showed positive reaction with alpha-fetoprotein antibodies in the tumor cells. Follow-up information obtained in these patients showed that all had died of tumor 1 to 3 years after initial diagnosis. The present cases document another unusual but distinctive growth pattern of yolk sac tumor in the mediastinum and highlight the importance of proper sampling of cystic lesions in this location.

Adolescent↗

The down-regulated in adenoma (DRA) gene encodes an intestine-specific membrane glycoprotein.

The protein product of the DRA gene, a gene whose expression is down-regulated in colon adenomas and adenocarcinomas, is a membrane glycoprotein and a member of a family of sulfate transporters. It is expressed in the intestinal tract (duodenum, ileum, cecum, distal colon), but not in the esophagus or stomach. DRA mRNA expression is restricted to the mucosal epithelium, and DRA protein expression is further limited to the columnar epithelial cells, particularly to the brush border. Consistent with its expression in the differentiated columnar epithelium of the adult human colon, DRA is first expressed in the midgut of developing mouse embryos at day 16.5, corresponding with the time of differentiation of the epithelium of the small intestine. A model for the structure of the DRA protein is proposed and its possible role in colon tumorigenesis is discussed.

Animals↗

Primary mediastinal and testicular seminomas: a comparison of K-ras-2 gene sequence and p53 immunoperoxidase analysis of 26 cases.

Primary mediastinal seminomas (MS) are rare tumors. Histologically, they are similar to their counterpart in the gonads. The survival rate has varied from 50% to 85% in different series. However, large series of these tumors primarily in the mediastinum are lacking. At the molecular level, a few reports document K-ras mutations in up to 40% of testicular seminomas (TS), localized predominantly to codon 12. Reports on TS p53 immunohistochemistry (IHC) range from negative to overexpression approaching 90% of cases, and by sequence analysis one small series showed a 23% mutation rate. To date, no analyses have been performed for either K-ras mutations or p53 immunohistochemical expression in primary MS. The authors studied 13 cases each of primary MS and TS from archival formalin-fixed, paraffin-embedded sections in which adequate tumor sampling and clinical history, including serological studies, and histological, histochemical, and IHC staining, were performed to confirm the diagnosis. p53 immunoperoxidase staining using citrate buffer/microwave antigen retrieval was performed. Topographic genotyping was performed on 5-microns-thick tissue sections up to 17 years old, in which the neoplastic cell population was sampled. Additionally, multiple sites within a given cases were sampled to determine clonality of the tumor cell population. Polymerase chain reaction and subsequent sequence analysis of the K-ras-2 exon-1 gene was used for mutation analysis. Focal weak staining with p53 IHC was observed in 4 of 13 (31%) MS and 10 of 13 (77%) TS cases, with all remaining cases being negative (P < .05). Only one MS case (8%) showed K-ras mutation (codon 13 GGC > GAC; glycine > aspartate), which is in contrast to 2 of the TS cases (15%), showing codon 12 mutations. All the remaining cases were wild type. Therefore, primary mediastinal seminomas appear to be different in their K-ras sequence and p53 immunostain profile from TS. Codon mutation type may be useful in determining primary versus metastatic origin of a mediastinal seminoma.

Adolescent↗

Microglandular carcinoma of the pancreas: immunohistochemical and ultrastructural study of an unusual variant of pancreatic carcinoma that may closely resemble a neuroendocrine neoplasm.

Two cases are described of an unusual form of primary adenocarcinoma of the pancreas characterized histologically by their striking resemblance with a neuroendocrine neoplasm. The tumors were composed of a population of relatively small, uniform cells arranged in sheets admixed with small microglandular structures resulting in a cribriform pattern of growth. The tumor cells displayed scant cytoplasm with indistinct cell borders and round to oval nuclei with irregular clumping of chromatin and small, inconspicuous nucleoli. Immunohistochemical studies in both cases showed positivity of the neoplastic cells with CAM 5.2 antibodies and negative staining with a battery of neuroendocrine-related markers including chromogranin, NSE and synaptophysin, as well as with a variety of peptide hormones including insulin, glucagon, vasoactive intestinal polypeptide, gastrin and serotonin. Ultrastructural examination revealed a cohesive population of cells forming abortive glandular lumens lined by imperfectly formed microvilli and showing well-developed junctional complexes. No dense core neurosecretory granules or zymogen granules could be identified in any of the cells, supporting a ductal type of differentiation for these tumors. The main importance of recognizing this rare variant of pancreatic adenocarcinoma lies in avoiding misdiagnosis with other primary and metastatic neuroendocrine neoplasms of this organ. Immunohistochemical and ultrastructural examination will be of value in such cases for differential diagnosis.

Adenocarcinoma↗

Benign cutaneous adnexal tumors with combined folliculosebaceous, apocrine, and eccrine differentiation. Clinicopathologic and immunohistochemical study of eight cases.

Benign cutaneous adnexal tumors displaying divergent differentiation are rare, with very few well-documented cases reported in the literature. We describe eight cases of benign adnexal tumors showing a variable combination of eccrine, apocrine, and folliculosebaceous differentiation. Clinically, all tumors presented as solitary, slowly enlarging dermal or subcutaneous nodules located in the head and neck and the extremities. Histologically, they were characterized by well-circumscribed, unencapsulated nodules composed of a lobular proliferation of epithelial cells displaying a spectrum of trichogenic, sebaceous, apocrine, and eccrine differentiation. The histological spectrum included lobules and trabeculae of basaloid cells with glandular and ductal elements, well-formed folliculosebaceous units, primitive follicles, and foci of tricholemmal keratinization. Immunohistochemical evaluation in four cases showed similar cytokeratin, carcinoembryonic antigen, and epithelial membrane antigen staining profiles as those reported for sweat gland adenomas; in addition, focal S-100 protein positivity and GCDFP-15 positivity could also be demonstrated, suggesting eccrine-apocrine differentiation. The tumors were most frequently confused histologically with other adnexal neoplasms, including sebaceoma, sebaceous adenoma, basal cell carcinoma, chondroid syringoma, and trichoepithelioma. The present series highlights the capability.

Adenoma↗

Pleomorphic large cell lymphomas of the mediastinum.

Nine cases of primary non-lymphoblastic, non-Hodgkin's large cell lymphomas of the mediastinum characterized by a highly pleomorphic histologic appearance are described. The patients, four women and five men, were aged 30 to 65 years. All patients presented with symptoms referable to their tumors, including cough, chest pain, dyspnea, pleural effusion, and superior vena cava syndrome. Clinical and pathologic staging in all patients showed that the bulk of the tumor was confined to the chest cavity at the time of initial diagnosis, with local infiltration into the neck, lung hilum, and surrounding mediastinal structures. Three different histological growth patterns were observed: one composed of a diffuse proliferation of pleomorphic, highly atypical cells with bizarre nuclear features that closely resembled a high grade sarcoma; another one composed of sheets of large, epithelial-appearing atypical cells suggestive of anaplastic carcinoma; and another pattern characterized by a pleomorphic proliferation of large lymphoid cells admixed with numerous scattered Reed-Sternberg-like cells reminiscent of the lymphocyte-depleted variant of Hodgkin's disease. Immunohistochemical studies on paraffin-embedded tissue sections in all cases showed positive staining of the tumor cells with CD20 and CD45 antibodies and negative staining with a large panel of markers, including broad-spectrum keratin, CAM 5.2, carcinoembryonic antigen, epithelial membrane antigen, vimentin, actin, desmin, HMB 45, S-100 protein, CD3, CD15, CD30, and CD45RO. Because of their location restricted to the anterior mediastinum, frequent lack of recognizable lymph node architecture, and bizarre cytologic features, the present group of lesions posed difficulties for diagnosis, their correct identification was achieved through the application of a panel of immunohistochemical markers. An awareness of these unusual histologic appearances of primary large cell lymphoma in the mediastinum and inclusion of a broad panel of lymphoid markers are therefore recommended for the evaluation of pleomorphic, undifferentiated malignant neoplasms of this anatomic region.

Adult↗

Primary thymic epithelial neoplasms showing combined features of thymoma and thymic carcinoma. A clinicopathologic study of 22 cases.

Thymic epithelial neoplasms are unusual tumors that may span the gamut from clinically benign, well-differentiated lesions (encapsulated thymoma) to overtly malignant, poorly differentiated neoplasms (thymic carcinoma). It has been commonly believed that lesions displaying obvious cytologic features of malignancy (i.e., thymic carcinoma) represent a unique and separate group that is histogenetically distinct from thymoma. We have studied 22 cases of thymic epithelial neoplasms characterized by the admixture of areas displaying conventional features of thymoma with areas showing features of thymic carcinoma. The tumors occurred in six women and 16 men whose ages ranged from 23 to 83 years (median, 53). The lesions presented in eight patients with symptoms of chest discomfort resulting from the involvement of surrounding structures; in 14 patients, they were asymptomatic and discovered incidentally on routine chest radiographs. Histologically, most tumors showed a combination of conventional thymomatous elements with well-differentiated squamous-cell carcinoma (10 cases), followed by thymoma and poorly-differentiated squamous carcinoma (seven cases) and spindle-cell thymoma with poorly-differentiated squamous carcinoma (five cases). Areas of transition between the two different components could be identified in most cases. In five cases, areas showing the features of clear-cell carcinoma could be seen either arising from squamous carcinomatous elements or within the thymomatous component, and in one case transitions between lymphoepithelioma-like carcinoma and anaplastic carcinoma could be observed. Two patients had a history of myasthenia gravis with biopsy-proven thymomas in whom the tumors had been monitored without treatment for 10 and 14 years before the sudden enlargement of the mass. The resected specimens in both patients showed the emergence of a carcinoma arising from a thymoma. The present cases appear to support the existence of a continuum in the spectrum of differentiation between thymoma and thymic carcinoma, suggesting a close histogenetic relationship between these two conditions. Such findings are important not only for our understanding of these tumors but may also play a significant role in the assessment of the biologic behavior and management of these lesions.

Adult↗

Gastrointestinal stromal tumors with prominent signet-ring cell features.

Three cases of gastrointestinal stromal tumors characterized by a population of cells displaying prominent signet-ring cell features are described. The tumors occurred in women aged 45 to 74 years and presented as small (< 2.5 cm), well-circumscribed, serosal nodules located within the wall of the stomach, small bowel, and rectum. Histologically, the lesions were characterized by a proliferation of large, round-to-oval cells containing abundant clear cytoplasm with displacement of the nuclei toward the periphery, which imparted to them a striking signet-ring cell configuration. In some areas, the signet-ring cells were seen to merge with short fascicles of spindle cells, whereas in other areas, the tumor cells were associated with prominent deposition of myxoid matrix. Histochemical studies showed glycogen granules within the cytoplasm of the tumor cells in two cases. Immunohistochemical studies showed a heterogeneous pattern of staining, with strong positivity for actin, vimentin, and CD34 in one case; strong vimentin and S-100 protein and weak, focal positivity for actin and CD34 in another; and vimentin positivity alone in the third. The present cases illustrate an unusual morphologic appearance of gastrointestinal stromal tumors that should be distinguished from primary and metastatic mucin-secreting carcinoma and other types of neoplasms with clear cell or signet-ring cell features.

Adenocarcinoma↗

Clear cell tumors of the skin.

Clear cell tumors of the skin represent a heterogeneous group of lesions of diverse histogenesis that share as their common denominator the presence of cells containing abundant clear cytoplasm. The phenomenon of cytoplasmic clearing may be observed focally or represent the dominant feature in a variety of tumors originating from the epidermis or in tumors within the dermis showing well-defined features of eccrine, sebaceous, and follicular lines of differentiation. In addition to the aforementioned conditions, melanocytic lesions derived from the epidermis characterized by a prominent or predominant clear cell component as well as metastases to the skin from clear cell tumors of internal organs may enter the differential diagnosis and will also be discussed. Because of the superficial resemblance imparted by this distinctive cytologic feature, these lesions may occasionally pose difficulties for diagnosis. A review of the various entities that comprise this group of lesions is presented, with emphasis on their distinguishing histopathologic features and differential diagnosis.

Adenocarcinoma, Clear Cell↗

Gastrointestinal stromal tumors.

Stromal tumors of the gastrointestinal tract represent relatively rare lesions that are thought to arise from connective tissue elements located along the entire length of the gut. For many years these tumors have been the subject of much controversy and debate in the literature regarding their histogenesis, criteria for diagnosis, prognostic features, and nomenclature. Only a minority of these lesions, mainly those confined to the esophagus and rectum, have been shown to correspond to mature, well-differentiated types of neoplasms such as leiomyoma or leiomyosarcoma of the conventional type. The majority of stromal tumors of the gastrointestinal tract correspond to a heterogeneous group of lesions that have as their common denominator an immature proliferation of epithelioid or spindle cells arising from its muscle layer and showing partial or incomplete myoid, neural, ganglionic, or mixed features of differentiation. This review will attempt to summarize our current knowledge and understanding of these lesions based on review of the literature and the author's personal experience, with an analysis of the criteria for distinguishing between benign and malignant tumors, and a proposal for a working classification for these lesions.

Gastrointestinal Neoplasms↗

Primary thymic carcinomas.

Primary thymic epithelial neoplasms are unusual tumors that have been the subject of much interest and debate, and their natural behavior has been difficult to determine. In this chapter the authors review current concepts on the classification and criteria for diagnosis of thymic carcinoma.

Carcinoma↗

Nevoid melanoma: a clinicopathological study of seven cases of malignant melanoma mimicking spindle and epithelioid cell nevus and verrucous dermal nevus.

We report seven cases of a distinctive type of malignant melanoma characterized by a deceptively benign histological appearance with an architecture resembling that of benign melanocytic nevi on scanning magnification. Two predominant architectural patterns were observed: a dome-shaped pattern (two specimens) and a verrucoid pattern (five specimens). The specimens with a dome-shaped pattern of growth were characterized by a smooth epidermal surface and a proliferation of epithelioid melanoma cells with an inconspicuous intraepidermal component resembling spindle and epithelioid cell nevi (Spitz nevi). Gradual diminution in the size of dermal nests toward the bases of the lesions simulating the maturation phenomenon of benign nevi was observed; however, the dermal organization in cords and strands of melanoma cells and the persistence of cellular atypia extending to the bases of the tumors allowed their recognition as malignant melanomas. On the other hand, the specimens with a verrucoid growth pattern consisted of broad, exophytic tumors with a verrucous epidermal surface resembling that of papillomatous dermal nevi but distinguished from them by the presence of a continuous proliferation of melanocytes along the dermal-epidermal junction and by confluent sheets of melanoma cells in the dermis without evidence of true maturation. Clinical follow-up showed local recurrence in three patients after intervals ranging from 5 months to 5 years and regional metastasis in one patient after 2 years. The lesions described here may constitute a serious pitfall for diagnosis because of their innocent silhouette on scanning magnification and their superficial resemblance to spindle and/or epithelioid cell nevi and benign verrucous melanocytic nevi. Proper attention to cytological detail and subtle architectural features will aid in recognizing this unusual variant of malignant melanoma.

Adult↗

Mediastinal hemangiomas: a study of 18 cases with emphasis on the spectrum of morphological features.

A study of 18 cases of mediastinal hemangiomas is presented. The patients were eight women and 10 men between the ages of newborn to 74 years. Clinically, the most common symptoms included dyspnea, cough, and chest pain. In one patient the mediastinal hemangioma was associated with another hemangioma in the gastrointestinal tract and presented initially with gastrointestinal bleeding. Four patients were asymptomatic. Anatomically, 14 tumors were located in the anterior mediastinum and four in the posterior mediastinum. Surgical resection was the treatment of choice for all patients. Grossly, the lesions were described as well circumscribed, cystic, hemorrhagic tumors that ranged in size from 2 cm to 20 cm in diameter. Histologically, the tumors were divided into two groups, one showing the features of capillary hemangioma characterized by a lobular and solid growth pattern featuring dilated small vessels and a solid proliferation of endothelial cells and another group showing the features of cavernous hemangioma characterized by large dilated vascular spaces with areas showing interstitial inflammatory changes, fibrosis, and smooth muscle proliferation. A few tumors were characterized by prominent regressive changes, including stromal hyalinization, dystrophic ossification, cystic and perivascular myxoid changes, and extensive fatty overgrowth. Some of the tumors showed extreme cellularity with virtual obliteration of vascular lumina due to compression by tumor cells and focal mitotic activity similar to that observed in cutaneous juvenile hemangiomas. Despite their large size, often infiltrating appearance, and sporadic mitotic activity, follow-up ranging from 1 month to 4 years in 11 patients showed all to be alive and well, thus demonstrating the benign nature of these neoplasms. A discussion of the differential diagnosis and a review of the literature on these rare tumors are presented.

Adolescent↗

Primary pulmonary sarcomas with features of monophasic synovial sarcoma: a clinicopathological, immunohistochemical, and ultrastructural study of 25 cases.

We present 25 cases of a primary pulmonary sarcoma bearing histological, immunohistochemical, and ultrastructural features indistinguishable from those of monophasic synovial sarcoma of soft tissue. The patients were 11 men and 14 women between the ages of 16 and 77 years. Clinically, the most common symptoms were chest pain, cough, shortness of breath, and hemoptysis. The lesions involved all lung segments. Grossly, they varied in size from 0.6 to 20 cm and were described as soft to rubbery tumors with areas of necrosis and hemorrhage, some with cystic changes. Two lesions involved the bronchial wall and in one case the tumor was described as encircling the bronchial tree. Histologically, all of the lesions were characterized by an atypical spindle cell proliferation with a solid growth pattern. Areas of myxoid, neural, hemangiopericytic, and epithelial-like growth pattern were observed. Mitoses, necrosis, and hemorrhage were seen in all lesions in varying proportions. Immunohistochemical studies for epithelial membrane antigen (EMA) and keratin showed strong focal positivity in 25 of 25 and 23 of 25 lesions, respectively. Immunohistochemical study for vimentin showed diffuse strong positivity in all lesions. Other immunostains, including desmin, smooth muscle actin, and S-100 protein, were negative. Electron microscopy in three cases showed spindle cells with elongated nuclei containing abundant cytoplasmic rough endoplasmic reticulum and well developed desmosome type intercellular junctions. Follow-up information ranging from 2 to 20 years was obtained in 18 patients. Six patients died of their tumors, whereas four patients died of unrelated causes without evidence of recurrence or metastases. Eight patients were alive with disease (recurrence and/or metastases) from 1 to 7 years after diagnosis. Four patients were alive and well without evidence of recurrence or metastases from 2 to 20 years (mean follow-up, 12.5 years). The present group of lesions appears to constitute a distinctive and as yet previously undescribed primary sarcoma of the lung, which probably represents the visceral counterpart of monophasic synovial sarcoma of soft tissue in a pulmonary location. Because of their distinctive biology these lesions should be distinguished from a variety of primary and metastatic malignancies of the lung.

Adolescent↗

Chordomas of the mediastinum: clinicopathologic, immunohistochemical, and ultrastructural study of six cases presenting as posterior mediastinal masses.

Six cases of chordomas presenting as primary posterior mediastinal tumors are described. Three patients were female, and three were male between the ages of 8 and 65 years (mean, 40.6 years). In all cases, the tumors presented radiographically as relatively well-circumscribed, encapsulated soft tissue masses that did not seem to be related to the thoracic or dorsal spine. Only in one case, focal infiltration of bone at the level of T6-T7 was observed at the time of surgery. Histologically, the lesions showed a spectrum of features that ranged from sheets and cords of large cells with abundant vacuolated cytoplasm to small, stellate cells embedded within an abundant mucoid matrix. In one case, the cell population showed more pronounced nuclear atypia with loss of cytoplasmic vacuolization, frequent mitotic figures, necrosis, and solid areas characterized by a perivascular distribution of atypical spindle cells set against a myxoid stroma. Another case showed features of chondroid chordoma, with an immature chondroid-appearing matrix surrounding the atypical tumor cells. Immunohistochemical studies in all cases showed positive staining of the tumor cells with CAM 5.2 and broad-spectrum keratin, epithelial membrane antigen (EMA) and vimentin, and, to a lesser extent, with S-100 protein. Stains for muscle actin, carcinoembryonic antigen (CEA), and desmin were negative. Ultrastructural examination in two cases showed a spectrum of features that varied from large cells with abundant cytoplasm containing scattered ribosomes, glycogen granules, Golgi apparatti, abundant intermediate filaments, and small lumen formation with immature microvilli to smaller cells with elongated cytoplasmic processes, fewer intermediate filaments, rare desmosome type intercellular junctions, and complexes of mitochondria/rough endoplasmic reticulum. On clinical follow-up, two patients died with metastases to the lungs, chest wall, and liver from 1 to 3 years after diagnosis, and two patients are alive and well without evidence of disease after 3 and 16 years. Chordoma should be entertained in the differential diagnosis of posterior mediastinal tumors. Application of immunohistochemical stains or electron microscopy will be of aid in separating them from other conditions that may histologically closely resemble these lesions.

Adolescent↗

Ectopic poorly differentiated (insular) carcinoma of the thyroid. Report of a case presenting as an anterior mediastinal mass.

A case is presented of an anterior mediastinal mass arising in a 64-year-old woman that showed on histologic, immunopathologic, and ultra-structural examination features of a poorly differentiated (insular) thyroid carcinoma. The tumor in this patient most likely arose from ectopically displaced thyroid tissue on the basis of a developmental defect. The clinicopathologic features and differential diagnosis of the lesion in the setting of its mediastinal location are discussed. Ectopic poorly differentiated (insular) carcinoma of the thyroid should be added to the list of mediastinal tumors showing a solid or insular growth pattern.

Carcinoma↗

Mucoepidermoid carcinomas of the thymus. A clinicopathologic study of six cases.

Six cases of mucoepidermoid carcinoma of the thymus are presented. The patients were two men and four women aged 17 to 66 years (median age, 34.5). Clinically, three patients had symptoms of chest discomfort and three were asymptomatic. Grossly, the tumors in three patients were described as cystic structures varying in size from 7 to 8 cm in greatest dimension, with focal areas of induration within the walls of the cyst that averaged from 1.5 to 3 cm. The other three cases had grossly and radiographically well-circumscribed, homogeneous tumor masses. Histologically, the lesions showed a spectrum of features that ranged from those of well-differentiated, to moderately well-differentiated, to poorly differentiated mucoepidermoid carcinoma, with sheets and solid islands of squamoid cells admixed with mucin-secreting epithelium lining gland-like spaces. In four cases, the tumor was histologically seen in continuity with the epithelial lining of multilocular cystic structures; the nonneoplastic components of the cysts contained abundant inflammation and showed the features of otherwise conventional acquired multilocular thymic cysts. Clinical follow-up showed that the two patients with intermediate and high-grade tumors died within 2 and 7 months after initial diagnosis. One of these patients showed at autopsy residual tumor limited to the mediastinum, whereas the other patient died with metastases to pericardium and myocardium despite postoperative radiation therapy. Two patients with low-grade tumors were alive and well with no residual disease 2 and 3 years after surgery, and the other two were lost to follow-up. Mucoepidermoid carcinoma of the thymus should be included in the differential diagnosis of cystic neoplasms of the thymus. As with their counterparts at other sites, the biologic behavior of these tumors closely correlates with their degree of differentiation and amount of cytologic atypia.

Adolescent↗