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A Beham

Publications and source records attributed to A Beham.

At least 37 records · Page 2Linked to original sources
PubMed1997

Epithelioid hemangioendothelioma of skin and soft tissues: clinicopathologic and immunohistochemical study of 30 cases.

Epithelioid hemangioendothelioma of soft tissues (EHE) represents a distinct entity with an unpredictable clinical course. We analyzed the clinicopathologic and immunohistochemical features in a series of 30 patients. Patient age range was 16-74 years (median 50); 18 of 30 patients were female. Eight tumors arose in the lower and two in the upper extremities, seven on the trunk, five each in the head/ neck and anogenital regions, two in the mediastinum, and one in the abdomen. Seventeen neoplasms were located in deep soft tissues, nine were subcutaneous or perifascial, and four were dermal; size ranged from 0.4 to 10 cm; in 11 cases the tumor was > 5 cm. Tumors with an infiltrative growth pattern were more common than entirely circumscribed lesions. The tumors were composed histologically of short strands, cords, or small clusters of epithelioid, round, to slightly spindled endothelial cells that formed at least focally, intracellular lumina and were set in a frequently myxohyaline stroma. Thirteen of 30 lesions showed angiocentric growth, which was occlusive in many cases. Immunohistochemically, all cases tested were positive for at least one endothelial marker (CD31, CD34, factor VIII, Ulex europaeus), six of 23 (26%) were positive for cytokeratin, and five of 11 (45%) were positive for alpha-smooth muscle actin. Median follow-up of 36 months (range 2-96) in 24 cases showed local recurrence in three cases and systemic metastases in five cases (21%); four patients (17%) died of tumor. Although more aggressive histologic features (striking nuclear atypia in eight cases, numerous spindled cells in 10, more than two mitoses per 10 high-power fields in nine, and small, more solid angiosarcomalike foci in four cases) tended to be related to poor clinical outcome, there was no clear correlation. Two metastasizing cases showed no histologically atypical features whatever. We suggest that EHE of soft tissue is better regarded as a fully malignant, rather than borderline, vascular neoplasm, albeit the prognosis is better than in conventional angiosarcoma.

Adolescent↗
PubMed1997

Cutaneous leiomyosarcoma.

We report the clinical, histopathologic, immunohistologic, and prognostic findings in 19 patients with cutaneous leiomyosarcoma, eight males and 11 females (mean age, 66 years; age range, 41-93 years). The tumors presented mainly as solitary lesions and were located on the head and neck (eight lesions), trunk (four lesions), upper extremities (three lesions), and lower extremities (four lesions). Histopathologically, two predominant growth patterns were observed: nodular (12 cases) and diffuse (seven cases). Neoplasms with a nodular growth pattern were characterized by high cellularity and prominent nuclear atypia, and they showed conspicuous mitoses, several necrotic cells, and sometimes extensive necrotic areas. By contrast, most cutaneous leiomyosarcomas with a diffuse growth pattern revealed low cellularity, well-differentiated smooth muscle cells, inconspicuous mitotic figures, and few or no necrotic cells. Immunohistologic investigations revealed all cutaneous leiomyosarcomas to express vimentin and smooth muscle actin. Pan-muscle actin (HHF-35) was also expressed in most cases (15 lesions). However, only 12 lesions showed positive staining for desmin. Remarkable was the expression of cytokeratins in five lesions. Clinical follow-up revealed local recurrences in five patients (three cases with nodular pattern and two lesions with a diffuse pattern) after a period ranging from 8 months to 3 years after surgical excision. No distant metastases have been observed in our series. We conclude that cutaneous leiomyosarcoma with a diffuse growth pattern may constitute a pitfall in histopathologic diagnosis because of the presence of only subtle criteria for malignancy. Cutaneous leiomyosarcoma may show different immunophenotypes, thus emphasizing the importance of using a large panel of antibodies (smooth muscle actin, HHF-35, desmin, vimentin, cytokeratins, and S-100 protein) in immunohistologic diagnosis. Cutaneous leiomyosarcoma sometimes reveals local recurrences, but it has negligible potential for distant metastases.

Actins↗
PubMed1997

Value of exfoliative cytology in differential diagnosis of epithelial hyperplastic lesions of the larynx.

In a comparative random study we examined material from suspicious laryngeal lesions from 85 patients. Material was obtained simultaneously by exfoliative cytology and biopsy. The diagnoses included keratosis, papilloma/papillomatosis, dysplasia/carcinoma in situ and invasive carcinoma. In each case we were able to demonstrate the cytological diagnostic criteria in the respective histological specimens. We illustrate this in the paper with some examples. Due to the excellent correlation between cytology and histology we are convinced that exfoliative laryngeal cytology is very significant in differential diagnosis of epithelial hyperplastic lesions.

Biopsy↗
PubMed1997

Köhlmeier-Degos's disease with primary intestinal manifestation.

Disseminated intestinal and cutaneous thromboangiitis (synonyms: Köhlmeier-Degos's syndrome, malignant atrophic papulosis, progressive arterial mesenterial vascular occlusive disease) is a rare, systemic vascular disease that is mainly manifested in the skin, gastrointestinal tract, and nervous system. The disease first appears as a necrotizing papulous dermatosis; as it generalizes, infarcted necroses develop in internal organs. These ischemic complications are the reason for the usually fatal outcome of the disease. A case report of a primary intestinal manifestation of this disease illustrates the clinical course, diagnosis, histopathologic findings, and differential diagnosis, with consideration of the current literature. Deposits of acid mucopolysaccharides and humoral immune mechanisms appear to play a role in the etiology and pathogenesis of this usually fatal vascular disease.

Humans↗
PubMed1997

Lateral neck cysts--the branchial theory revisited. A critical review and clinicopathological study of 97 cases with special emphasis on cytokeratin expression.

Theories regarding the origin of lateral neck cysts (LNC) range from congenital branchial pouch abnormalities to acquired salivary gland inclusions within lymph nodes. We analyzed 97 LNC histologically and evaluated their cytokeratin (CK) profile in a search for their derivation. 77/97 LNC were located in soft tissues, 20/97 within lymph nodes. LNC of young patients and of recent symptomatic presentation in older patients were lined by respiratory epithelium with scant lymphoid tissue, with expression of "simple epithelial" CK in ciliated cells and bimodal expression of "simple" and "stratified-epithelial-type" CK in basal cells. In longer standing symptomatic LNC, respiratory epithelium alternated with transitional-type pseudostratified epithelium with intraepithelial Langerhans cells and lymphoid hyperplasia, or consisted exclusively of squamous epithelium. We propose that respiratory epithelium is the "native" epithelium of LNC and squamous metaplasia results from inflammation induced stem cell hyperplasia in respiratory epithelium, evidenced by co-expression of "simple" and "stratified-epithelial-type" CK in all cells of transitional-type pseudostratified epithelium, the early stage in metaplastic transformation. Respiratory epithelium predominates in early LNC, lines pharyngeal tonsils and the recessus tonsillo-tubalis, and is a minor constituent of palatine tonsil but is not present in salivary glands. None of the LNC contained dysplasia, atypia, or carcinoma, or were associated with a primary carcinoma of tonsils or head and neck. We demonstrate that LNC arise from developmental remnants (congenital) of the 2nd branchial pouch, which may lie dormant for many years. Symptomatic enlargement, squamous metaplasia and lymphoid hyperplasia ensue as a consequence of immunologic stimulation a development reflected in hyperplastic palatine tonsils.

Adolescent↗
PubMed1996

Importance of the Bcl-2 family in cell death regulation.

Bcl-2 was first identified as a novel transcript associated with the t(14;18) chromosomal breakpoint which occurs in most follicular lymphomas. The deregulated expression of bcl-2 was found to contribute to multistep neoplasia through the suppression of cell death, or apoptosis, in transgenic mouse models. Bcl-2 was subsequently shown to be normally expressed in a variety of tissues and to significantly inhibit the induction of apoptosis in many experimental systems. Bcl-2 is now known to be structurally similar to other proteins, in particular within the domains referred to as BH1 and BH2. This multigene family of cell death regulators includes members which enhance rates of apoptosis, including bcl-xs and bax, and those which inhibit apoptosis, including MCL-1 and bcl-xL. Members of the bcl-2 family physically interact with other proteins, including other family members and these interactions appear to modulate their function. The mechanism(s) by which bcl-2 family members regulate cell death remain in large part unknown, although recent evidence suggests that bcl-2 may interfere with cellular signalling events involved in apoptosis induction.

Amino Acid Sequence↗
PubMed1996

Choristoma of the middle ear.

Choristoma is a mass of tissue histologically normal for an organ or part of the body other than the site at which it is located. A rare case of ectopic salivary gland choristoma in the middle ear is described in a 4-year-old boy whose only symptom was a 50 dB conductive hearing loss in the presence of a normal tympanic membrane.

Audiometry↗
PubMed1996

Paragangliomas of the carotid bifurcation: oncological aspects of vascular surgery.

The present study reviews in concise form the past 12 years of our clinical experience with paragangliomas of the carotid body. Every aspect of the anatomical, histological and biological and biological behaviour of paragangliomas of the carotid body has been recorded in order to be able to define better surgical management and the clinical prognosis. In addition to the conventional histological methods of investigation we also applied immunohistochemistry and made use of electron microscopy. Eighteen tumours of the carotid body in 12 female and four male patients with a mean age of 54 years (34 to 70 years) were treated surgically. Angiography of the aortic arch and supra-aortic branches was undertaken diagnostically in every case. In three patients computer tomography and magnetic resonance imaging demonstrated spreading of the tumour up to the base of the skull and pre-operative embolization of the arteries supplying the tumour was performed to decrease tumour vascularity. In four cases (22%) resection of the internal carotid artery and its reconstruction by a saphenous vein graft was required in order to achieve the therapeutic aim of a complete extirpation of tumourous tissue and preserving the vascular system of the internal carotid artery. In three cases (16%) there was histological evidence of a metastasizing process to the local lymph nodes of the neck and, due to this criterion of malignancy, 50-60 Gy of radiation was applied to the site of the tumour after surgery. Four patients experienced a transient neurological deficit in the supply area of the hypoglossal and the recurrent nerves. In one case, the vagus nerve could not be retained. In an average follow-up of 102 months we recorded one case of local recurrence 13 months after the initial resection and radiation treatment applied to a malignant paraganglioma. All the other patients were doing well without evidence of recurrent disease.

Carotid Body Tumor↗
PubMed1996

Myxofibrosarcoma. Clinicopathologic analysis of 75 cases with emphasis on the low-grade variant.

Myxofibrosarcoma is one of the most common sarcomas in the extremities of elderly patients. We analysed the clinicopathologic features in a series of 75 patients. All patients were adults (range, 22-91 years; median, 66 years) with an approximately equal incidence in men and women. Thirty-five tumors arose in the lower and 25 in the upper extremities, nine on the trunk, two each in the retroperitoneum and the head and neck region, and one each in the pelvis and penis. Forty-eight cases (69.5%) were located in dermal or subcutaneous tissues. Distinctive histologic features included the following: a commonly nodular growth pattern; a myxoid matrix containing elongated, curvilinear capillaries; and fusiform, round or stellate tumor cells with indistinct cell margins, slightly eosinophilic cytoplasm, and hyperchromatic atypical nuclei. These lesions varied from a hypocellular, mainly myxoid, and purely spindle-cell appearance (low-grade neoplasms) to high-grade, pleomorphic (malignant fibrous histiocytoma-like) lesions with multinucleated giant cells, high mitotic activity, and areas of necrosis. Immunohistochemistry in 44 cases revealed only vimentin and occasional actin positivity. Ultrastructurally, tumor cells had a fibroblastic phenotype. DNA flow cytometry and proliferation analysis showed an association between aneuploidy and histologic grade. An average follow-up of 45 months (range, 5-300 months) in 60 cases has revealed local recurrence in 33 cases (54%). Thirteen patients developed metastases, and 13 tumor-related deaths occurred. A short interval to first local recurrence was associated with poor clinical outcome. The rate of local recurrence was independent of histologic grade, but only intermediate and high-grade neoplasms metastasized. The depth of the primary lesion did not influence the incidence of local recurrence. However, in deep-seated neoplasms, the incidence of metastases was higher and the percentage of tumor-related deaths was twice as high as in superficially located lesions, reflecting the fact that deep-seated lesions tended to be higher-grade, larger tumors. Myxofibrosarcoma tends to become progressively higher grade in recurrences, as demonstrated in five cases in our series. The poorly recognized low-grade myxofibrosarcoma is emphasized, as proper diagnosis and treatment and scrupulous follow-up are mandatory to avoid local recurrence and gradual tumor progression to a higher-grade neoplasm that may then metastasize.

Adult↗
PubMed1995

Malignant schwannoma of the breast.

Malignant schwannoma (MS) is a tumor of the Schwann or nerve sheath cells, most frequently occurring in the lower and upper extremities, trunk and head region. We report the third known case of MS of the breast, which occurred in a 27-year-old woman. The palpable tumor, about 1.2 cm in diameter, was localized in the upper inner quadrant of the right breast. After ultrasonography, mammography and fine needle aspiration cytology, the tumor was removed surgically.

Adult↗
PubMed1995

Ossifying fibromyxoid tumour of the neck clinically mimicking a primary neoplasm of the thyroid gland.

We present a 50-year-old male patient with an ossifying fibromyxoid tumour (OFMT) of the prethyroidal soft parts infiltrating the thyroid gland in coincidence with recurrent goitre, 20 years after strumectomy. The tumour, initially misdiagnosed as thyroid carcinoma and malignant schwannoma respectively, was histologically composed of typical solid cell-formations and shelly-like mature bone at the periphery. In addition, in the tissue adjacent to the tumour some foreign body granulomas with suture material and small proliferating nerves were present as a residual of the preceding strumectomy. We regard this finding as an indication of nerve sheat origin of OFMT. Follow up of 3 years after total thyroidectomy and local irradiation shows the patient free of recurrent and metastatic disease.

Diagnosis, Differential↗
PubMed1995

Extramedullary hematopoiesis in pilomatricomas.

We report on seven adult patients with pilomatricomas showing histopathologic findings of extramedullary hematopoiesis. There was no Ovidence of hematological disorders or systemic diseases. Clinically, the lesions appeared to be firm, painless nodules situated on the upper extremities, head, and back. Histopathologically, they represented stereotypical examples of regressive pilomatricomas with relatively small basaloid areas and large masses of cornified eosinophilic material containing shadow cells with variable areas of calcification or ossification. Bone marrow cellular elements, including myeloid and erythroid precursors, and (in two cases) megakaryocytes were present. The finding of extramedullary hematopoiesis in seven (5.8%) of 120 cases of pilomatricomas may suggest that this phenomenon is not uncommon in these neoplasms. Interestingly, hematopoietic infiltrates were histopathologically detected to be contiguous with areas of osseous metaplasia in only two of the seven pilomatricomas in our study. It should further be stressed that extramedullary hematopoiesis in regressive lesions of pilomatricomas is a localized phenomenon and that these findings may not be linked with a systemic hematological disorder. The significance of extramedullary hematopoiesis in pilomatricomas, however, remains to be determined.

Adult↗
PubMed1994

Coexistence of papillary and medullary carcinoma of the thyroid gland-mixed or collision tumour? Clinicopathological analysis of three cases.

We present three thyroid carcinomas displaying medullary and papillary components. In two cases the papillary component was characterized by typical papillae with a fibrovascular core; in one a follicular variant of papillary carcinoma was found. The papillary component was dominant in two and the medullary in one case. One tumour showed clear-cut borders between the two components, the others displayed an intermingled pattern. Both tumour components were seen in lymph node metastases with immunostaining with antibodies to calcitonin, chromogranin A, carcinoembryonic antigen, other neuroendocrine markers and thyroglobulin. At least two of our cases are true mixed carcinomas probably arising from a common stem cell.

Adult↗
PubMed1994

Dedifferentiated liposarcoma. Clinicopathologic analysis of 32 cases suggesting a better prognostic subgroup among pleomorphic sarcomas.

Dedifferentiated liposarcoma is uncommon, and only a small number of cases have been documented. We describe the clinicopathologic features in a series of 32 cases. All patients were adults (age range, 37-83 years; median, 67 years); 20 were men and 12 were women. Commonest site was the retroperitoneum (fifteen cases); six cases arose in the limbs, four in the paratesticular region, three in the peritoneal cavity, two on the trunk, and one each in the buttock and larynx. One primary tumor was subcutaneous. Thirty tumors arose de novo (i.e., combined with well-differentiated tumor in the primary lesion), while two developed in recurrences of a previously well-differentiated liposarcoma. The well-differentiated component was most often lipoma-like and typically there was a histologically abrupt transition to spindle celled nonlipogenic tumor. The dedifferentiated component most often resembled either storiform "malignant fibrous histiocytoma" ("MFH") with limited pleomorphism or myxofibrosarcoma (myxoid "MFH"); the latter pattern is rarely otherwise seen in the retroperitoneum. A small number of cases showed appearances reminiscent of myxoid embryonal rhabdomyosarcoma. An unusual feature in three cases was the occurrence of a discontinuous micronodular pattern of dedifferentiation. Average follow-up of 5.6 years (range, 3 months to 33 years) in 27 cases have revealed local recurrence in 14 patients and systemic metastases in only four patients. The primary sites of the metastasising cases were upper back, thigh, retroperitoneum, and paratesticular region. There have been only seven tumor-related deaths. Good prognosis in de novo dedifferentiated liposarcomas seems unrelated to the extent or morphologic pattern of dedifferentiation. However, high mitotic activity in the dedifferentiated component was associated with a more aggressive clinical course. Our study underlines that dedifferentiation in peripherally located or even subcutaneous liposarcomas does occur, albeit rarely, and that dedifferentiated liposarcomas of the limbs may metastasize. The results suggest that dedifferentiated liposarcomas, as a subgroup among the "MFH-like" sarcomas, have a better prognosis than pleomorphic sarcomas as a whole.

Adult↗
PubMed1993

Nasopharyngeal angiofibroma: an immunohistochemical study of 32 cases.

Thirty-two cases of nasopharyngeal angiofibroma, including 2 recurrences, all of which had been excised from males between 7 and 25 years, were subjected to systematic immunohistochemical study. Most of the tumour vessels, which lacked elastic laminae, were characterized by vascular walls of irregular thickness and variable muscle content. In places endothelial cells were only separated from the stroma by a single attenuated layer of contractile cells, whereas elsewhere the same vessel walls showed pad-like thickenings of their muscle coat. All cells of the vessel walls showed immunoreactivity for vimentin and smooth muscle actin, whereas desmin-positive cells were present only in small numbers in some vessels, generally those with thicker muscle coats. The stromal cells were decorated by vimentin antibodies only; however, in some more fibrotic hyaline areas the stromal cells displayed also reactivity for smooth muscle actin. In most cases S-100 protein-staining disclosed many nerves, and this accentuated their parital distortion by tumour tissue. Our findings provide an extended insight to the morphology of angiofibromas at this site, particularly highlighting the irregularity of their vascular walls, which, taken together with the lack of elastic laminae and elastic stromal fibres, can be held responsible for the typical pronounced tendency for haemorrhage in these lesions.

Adolescent↗
PubMed1993

Synovial metaplasia of the skin.

We present two female patients, aged 15 and 45 years, with synovial metaplasia of the skin. Both lesions, localized in the knee and hand respectively, arose after preceding local trauma. Case 1 had intradermal irregular cystic spaces in the adjacent myxoid stroma of which large polygonal eosinophilic cells were found. In contrast, case 2 was characterized by a longitudinal space within the lower dermis and subcutis which was lined by a membrane similar to hyperplastic synovium. The cells of the membrane showed an eosinophilic spindle shaped cytoplasm with processes towards the lumen. In both cases the eosinophilic cells, strongly suggestive of fibroblasts, showed staining for vimentin only, whereas no reactivity could be obtained with antibodies to actin, desmin, S-100 protein, Factor VIII related antigen, cytokeratin, epithelial membrane antigen, carcinoembryonic antigen and CD 68. The absence of CD 68 positivity differs from that seen in normal and hyperplastic synovium. Delayed wound healing around a nidus seems the most likely cause of the change.

Adolescent↗
PubMed1993

Involucrin expression in breast carcinomas: an immunohistochemical study.

The expression of involucrin, a structural component of the envelope of mature squamous epithelium, was studied in 166 paraffin-embedded breast carcinomas. In 41 cases (24.7%) involucrin-positive, light microscopically non squamous tumour cells were detected. The number of involucrin-positive tumour cells varied considerably from case to case. For further characterization, involucrin-positive cases were studied using monoclonal antibodies to various cytokeratins (PKK1, EAB 903, EAB 904) and, in selected cases, double immunostaining with antibodies to cytokeratins and involucrin were performed. Coexpression of involucrin and cytokeratins demonstrated by PKK1 was seen in all tumour cells, whereas coexpression of involucrin and cytokeratins detected by EAB 904 was only seen in single and scattered cells in a few cases. Cytokeratins detected by EAB 903 were not coexpressed with involucrin in our cases. Our results indicate heterogeneity of cytokeratins in breast carcinomas and suggest a dissociation in the regulation of involucrin and cytokeratin expression.

Breast Neoplasms↗