Biomedical subjects

S W Weiss

Publications and source records attributed to S W Weiss.

At least 73 records · Page 4Linked to original sources

PubMed1993

Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis.

We report the clinical and pathological features of nine distinctive, but relatively unknown, vascular tumors of infancy and childhood presenting as soft tissue masses often associated with locally aggressive disease, lymphangiomatosis and Kasabach-Merritt syndrome. The patients, four males and five females, were all in their first decade of life except for two (median, 2 years; range, 5 months to 19 years). These tumors involved deep soft tissues of the upper extremity (four cases), retroperitoneum (two cases), chest wall, scalp, and neck (one case each). Four patients also had Kasabach-Merritt syndrome, and three patients had lymphangiomatosis. Lymphangiomatosis consisted of diffusely infiltrating lymphangioma of soft tissue (three cases) and in two by the additional presence of bone lesions. In one of these three cases, lymphangiomatosis antedated the diagnosis of the vascular tumor, and in the remainder they were concurrently diagnosed. Tumors were characterized by infiltrating, interconnecting sheets or irregular nodules of slender endothelial cells lining crescentic or slit-like vessels and, less commonly, rounded capillary-type vessels. Within some tumors, nests of epithelioid endothelial cells with prominent eosinophilic cytoplasm containing finely granular hemosiderin, hyaline droplets, and cytoplasmic vacuoles were identified. Smaller amounts of hemosiderin were observed within the spindled endothelial cells and microthrombi could be seen occasionally within the tiny lumina. Nuclear atypia was minimal within these tumors and mitotic figures were infrequent, averaging 2 to 3/10 high-power fields (HPF) (range 0-7/10 HPF). Larger, well-formed feeding vessels were present at the periphery of the tumor. The endothelium of these vessels expressed factor VIII-AG, CD34, and bound Ulex europaeus, and contained an occasional perithelial cell expressing muscle-specific actin. In contrast, the spindled tumor cells expressed only CD34. Human papilloma virus (HPV)-16-like DNA transcripts, which have been identified in cases of Kaposi's sarcoma, were not detected by polymerase chain reaction in two cases. Follow-up information revealed that four patients were alive without disease after wide excision, multiple excision(s), or amputation (one case); three were alive with disease; and two died, one from lymphangiomatosis with respiratory compromise and the other from hemorrhage complicating Kasabach-Merritt syndrome. It appears that treatment should consist of wide local excision and supportive therapy for associated symptoms.

Adolescent↗

PubMed1993

CD-34 is expressed by a distinctive cell population in peripheral nerve, nerve sheath tumors, and related lesions.

The pattern of CD-34 antigen (human progenitor cell antigen) immunoreactivity was studied within normal nerve, and a variety of nerve sheath and neuroectodermal tumors. Besides normal nerves, 111 soft tissue tumors were studied, including 17 neurofibromas, 10 neurilemomas, 12 malignant peripheral nerve sheath tumors, 1 melanocytic schwannoma, 21 fibroblastic lesions, 31 fibrohistiocytic lesions, seven neuroectodermal lesions, and 10 miscellaneous tumors. CD-34-positive dendritic cells were consistently identified within the endoneurium of normal nerve, all neurofibromas, dermatofibrosarcomas, and Antoni B (but not Antoni A) areas of neurilemomas. CD-34 was not expressed in the majority (eight of 10 cases) of malignant peripheral nerve sheath tumors. CD-34 was also lacking in all fibroblastic lesions (nodular fasciitis, fibromatosis, keloid, fibrosarcoma) and in neuroectodermal tumors that are not generally considered to show true nerve sheath differentiation (neurotropic melanoma, clear cell sarcoma, neuroepithelioma). We conclude that CD-34 (or a closely related epitope) defines a normally occurring nerve sheath cell that appears to be cytologically and immunophenotypically distinct from a fibroblast and conventional Schwann cell. The antigen can also be localized to benign nerve sheath tumors, but tends to be lost in malignant ones. The consistent presence of CD-34 within all 13 cases of dermatofibrosarcoma protuberans can be used as evidence in support of the view that these lesions are variants of nerve sheath tumors, and distinct from benign fibrous histiocytomas which consistently lack the antigen. Finally, expression of CD-34 by one of three giant cell fibroblastomas reinforces the close relationship between this tumor and dermatofibrosarcoma protuberans.

Antigens, CD↗

PubMed1993

Ischemic fasciitis: a juxta-skeletal fibroblastic proliferation with a predilection for elderly patients.

We are reporting six examples of a distinctive reactive fibroblastic proliferation occurring predominantly in elderly bed-ridden or convalescent patients that presents as an ill-defined mass often overlying a bony prominence or in close apposition to the periosteum of bone. Unlike conventional fasciitis, the lesion achieves a relatively larger size and is characterized by a biphasic appearance with an outer fringe of mitotically active fibroblasts and capillaries circumscribing a central area of liquefactive and focally coagulative necrosis. Follow-up information in all patients indicated no evidence of aggressive behavior. We believe these lesions arise on an ischemic basis, similar to a decubitus ulcer, from long-standing or intermittent pressure on the soft tissue structures. Because of their large size and close proximity to bone, they are commonly mistaken for a sarcoma.

Adult↗

PubMed1992

SAS amplification in soft tissue sarcomas.

Gene amplification is an important mechanism of increased gene expression in a number of human solid tumors. We have recently identified and cloned sequences from a novel DNA amplification unit in malignant fibrous histiocytoma. The amplified sequences are derived from chromosome 12q13-14 and encode a gene designated SAS (sarcoma amplified sequence). In the present study, a series of soft tissue sarcomas was studied to characterize further the phenomenon of SAS amplification. Seven of 22 (32%) malignant fibrous histiocytomas and three liposarcomas contained SAS amplification. Strikingly, all of the tumors with SAS amplification occurred in central sites (i.e., in the abdominal or inguinal regions) rather than in the extremities (i.e., in the arms of legs). These observations demonstrate that SAS amplification occurs with a significant frequency in mesenchymal tumors and is particularly associated with abdominal disease.

Adult↗

PubMed1992

Fibrosarcoma-malignant fibrous histiocytoma of the breast. A clinicopathological study of 32 cases.

We report the clinical and pathologic features of 32 sarcomas of the breast with features spanning the spectrum of fibrosarcomas-malignant fibrous histiocytomas. Neoplasms were categorized as high- or low-grade lesions depending on a combination of the degrees of atypia and mitotic activity. The majority of high-grade lesions had marked (3+) nuclear atypia and at least five mitotic figures per 10 hpf. High-grade lesions with moderate (2+) nuclear atypia had a mitotic activity of six or more mitotic figures per 10 hpf. All low-grade lesions had five or fewer mitotic figures per 10 hpf, and none had a score of the nuclear grade times mitotic figures of more than 10. The average mitotic activity in low-grade lesions was two mitotic figures per 10 hpf; the high-grade lesions had 12 mitotic figures per 10 hpf. Sixty-nine percent of the low-grade fibrosarcomas-malignant fibrous histiocytomas showed mild (1+) cytologic atypia, and 69% of the high-grade lesions showed severe (3+) cytologic atypia. The herringbone pattern was associated with a more favorable prognosis than the malignant fibrous histiocytoma pattern. Compared to the high-grade lesions, low-grade fibrosarcomas-malignant fibrous histiocytomas were slow-growing, produced fewer recurrences, and did not metastasize. Of the 16 women with low-grade lesions, all were free of tumor at last contact, despite recurrence in more than half of the patients. In contrast, 31% of the patients with high-grade lesions died of tumor, and 13% were alive with disease. Twenty-five percent of women with high-grade lesions developed distant metastases.

Adult↗

PubMed1992

Angiomatosis of soft tissue. An analysis of the histologic features and clinical outcome in 51 cases.

The clinicopathologic features and clinical behavior of 51 cases of angiomatosis of soft tissue are analyzed. We have defined this lesion as a histologically benign vascular lesion that affects a large segment of the body in a contiguous fashion either by vertically involving multiple tissue types (e.g., subcutis, muscle, bone) or by involving similar tissue types (e.g., multiple muscles). Such lesions usually present in the first two decades of life and have a highly characteristic but not totally specific histologic pattern. The common pattern consists of a haphazard proliferation of vessels of varying sizes, particularly large veins. The latter have irregularly attenuated walls and intimal redundancies. However, the most distinctive feature is the presence of clusters of capillary vessels residing within or just adjacent to the vein walls. A second but uncommon pattern is that of clusters of capillary-sized vessels infiltrating the soft tissues. Both types are typically associated with large amounts of fat, suggesting that these lesions are more generalized mesenchymal proliferations rather than exclusive vascular lesions. This idea is supported by one unique case that included as part of the lesion a diffuse proliferation of glomus cells. Follow-up information in 25 cases (median 5 years; range 1-24) indicated that 22 patients experienced local recurrences. Nine patients developed more than one recurrence. There was no correlation between the age of onset of the lesion and the number of recurrences.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1992

Elastofibromatous change of the rectum. A lesion mimicking amyloidosis.

We report the case of a 58-year-old woman who had a 7-year history of multiple myeloma and multiple rib fractures and who presented with dysphagia. She underwent thorough gastrointestinal evaluation to rule out the possibility of amyloidosis. Although upper gastrointestinal biopsies were negative, the rectal biopsy was characterized by extensive smudgy eosinophilic deposits in the submucosa that closely resembled amyloid, except that they were not congophilic. Fibers with serrated borders characteristic of those in elastofibroma were identified and confirmed by means of elastic stain and electron microscopy. Elastofibromatous change of the gastrointestinal tract is a rare lesion that has been reported once previously in association with gastric ulcer. This case illustrates that it may occur as a spontaneous or subclinical finding in the absence of other pathologic lesions. The close resemblance between elastofibromatous change and amyloid deposits necessitates the appropriate histochemical and ultrastructural studies.

Amyloidosis↗

PubMed1992

Well-differentiated liposarcoma (atypical lipoma) of deep soft tissue of the extremities, retroperitoneum, and miscellaneous sites. A follow-up study of 92 cases with analysis of the incidence of "dedifferentiation".

Ninety-two cases of well-differentiated liposarcoma of deep soft tissue of the extremity, retroperitoneum, and groin with follow-up information of at least 2 years and no evidence of dedifferentiation at the time of diagnosis were studied to determine their long-term behavior. The tumors occurred most commonly in the muscles of the extremity (46 cases), followed by the retroperitoneum (23 cases), groin (14 cases), and miscellaneous sites (nine cases). Tumors in the retroperitoneum recurred in nearly all cases (21 of 23 cases), occasionally caused patient death, and dedifferentiated in four cases (median time to dedifferentiation, 8 years). Tumors in the groin had a similar high recurrence rate (11 of 14 cases), caused death of patients (two of 14 cases), and dedifferentiated (four of 14 cases). In contrast, those in the extremity recurred less frequently (20 of 46 cases) and had no disease-related mortality. Three of 46, however, underwent dedifferentiation (median time to dedifferentiation, 7 years). Of the 11 cases that underwent dedifferentiation, the interval between diagnosis and dedifferentiation ranged from 2 to 18 years (median time, 9 years; average time, 11 years). Six of the 11 patients showed dedifferentiated foci in the first recurrence, and three died of metastatic disease. Our study indicates that the behavior of well-differentiated liposarcomas is strongly influenced by location. Although the prevailing view is that dedifferentiation is restricted to tumors of the retroperitoneum, our study indicates that it is not a site-specific phenomenon, but is more likely a time-dependent phenomenon seen in situations with a high likelihood for clinical persistence of disease for a long period. Dedifferentiation identifies a tumor that is potentially more aggressive; yet the progression of the disease following dedifferentiation may be highly variable and probably depends on a number of factors, including the amount of dedifferentiation and type of therapy. Although atypical lipoma is an acceptable term for well-differentiated liposarcomas of the subcutis, it fails to convey the potentially life-threatening properties of retroperitoneal tumors. For these lesions as well as those in the deep soft tissues of the extremity, retention of the term well-differentiated liposarcoma is advocated.

Adult↗

PubMed1991

Evaluation of CD68 and other histiocytic antigens in angiomatoid malignant fibrous histiocytoma.

Immunohistochemistry for the histiocyte antigens CD68, L1, factor XIIIa, and S100 protein was performed on 19 cases of angiomatoid malignant fibrous histiocytoma (MFH) and for keratin, leukocyte common antigen, factor VIII-related antigen, muramidase, and desmin on six of these cases. Nine of 19 cases expressed the histiocyte differentiation antigen CD68, and in three cases expression was seen in greater than 90% of cells. Of the 19 cases studied, three contained rare S100-positive tumor cells, and two expressed factor XIIIa within tumor cells. In three of the six cases, rare desmin-positive tumor cells were identified. The neoplastic cells did not express the other antigens studied. CD68 was not detected in any of 12 cases of storiform-pleomorphic and myxoid MFH or in any of 24 cases of normal tissues or nonfibrohistiocytic mesenchymal tumors except for granular cell tumors. These results indicate that either the cells of angiomatoid MFH differentiate along lines of a tissue macrophage or alternatively, and perhaps more likely, are mesenchymal tumors with a phenotypic alteration, paralleling an enhanced capacity for phagocytosis and acquisition of lysosomes. In either event, the immunophenotype contrasts with conventional MFH and provides another independent set of observations in support of a separation of angiomatoid from conventional MFH.

Antibodies, Monoclonal↗

PubMed1991

Cytometry and morphometry of malignant fibrous histiocytoma of the extremities. Prediction of metastasis and mortality.

Flow cytometry and nuclear morphometry were compared with traditional pathologic grading techniques for predicting the course of malignant fibrous histiocytoma of the extremities. Clinical, pathologic, and flow/morphometric variables from 53 cases were tested by Cox regression for prediction of distant recurrence and mortality. Tumor grading based on extent of tumor necrosis was a significant predictor for both disease-free survival (p = .014) and overall survival (p = .003). The fraction of nuclei in the S + G2M segment of DNA histograms was significant for disease-free survival (p = .007), and remained significant (p = .033) in a joint Cox model with necrosis-based grade (p = .004 for the bivariate model). Relative risk for recurrence varied nearly 10x between the 10th and 90th percentiles of grade and (S + G2M)1/2. Overall survival was predicted by a nuclear shape feature termed "R" (p = .000008), the casewise difference (residual) between expected and observed nuclear perimeter as a function of average Feret diameter. In a bivariate Cox model, relative risk of mortality varied 35x between the 10th and 90th percentiles of grade and R. Cytometric and morphometric data contain information about recurrence-free and overall survival beyond that available from more usual clinical and pathologic features. It seems likely that nuclear morphometry, in particular, will prove to be a useful aid for estimating the prognosis of patients with malignant fibrous histiocytoma of the extremities.

DNA, Neoplasm↗

PubMed1991

Epithelioid variant of malignant peripheral nerve sheath tumor (malignant epithelioid schwannoma).

Twenty-six cases of malignant peripheral nerve sheath tumor with a predominant epithelioid pattern were studied to determine the range of its histologic patterns, immunophenotype, and biologic behavior. The tumor presented as an asymptomatic mass either in superficial (16 cases) or in deep soft tissue (10 cases) of the extremity. Characteristically, those in deep soft tissue were composed of vague nodules of varying cellularity made up of cords or strands of rounded epithelioid cells with prominent nucleoli. Those in superficial soft tissue were uninodular masses composed of tight clusters of cells showing cell-to-cell molding but possessing the same prominence of nuclei and mitotic activity as those in deep soft tissue. Several were associated with a preexisting benign nerve sheath tumor. A number of cases deviated from the above description, including cases that resembled a clear cell carcinoma, a malignant rhabdoid tumor, and a pleomorphic sarcoma. The majority of cases (80%) strongly expressed S-100 protein and neuron-specific enolase, but all lacked a melanoma-associated antigen (as defined by HMB-45) and cytokeratin. Stains for type IV collagen defined linear immunoreactivity around single cells and groups of cells. This pattern did not differ substantially from that of melanomas and therefore did not serve as a reliable discriminant. Follow-up information indicated a more favorable course for those in superficial soft tissue compared with those in deep sites. Two of 16 patients in the former group developed metastatic disease compared with three of 10 in the latter group. Tumors in superficial soft tissue may be eminently treatable and curable, depending on size.

Adolescent↗

PubMed1991

Observations concerning the pathogenesis of epithelioid hemangioma (angiolymphoid hyperplasia).

Epithelioid hemangioma (EH) is a benign vascular lesion that is characterized by well-formed, capillary-sized vessels lined by histiocytoid or epithelioid endothelial cells and often accompanied by a secondary inflammatory infiltrate. Whether or not these lesions are reactive or neoplastic has been debated and is reflected in the various designations, such as angiolymphoid hyperplasia with eosinophilia (ALHE), pseudopyogenic granuloma, and inflammatory angiomatous nodules. In order to study the pathogenesis of this lesion, 96 examples of EH of superficial and deep soft tissue origin were reviewed, noting the frequency with which these lesions arose adjacent to a damaged vessel and the incidence of antecedent trauma. In 60 cases (63%), an artery or vein was associated with the mass and, in the majority (52 cases), the vessel was damaged, as evidenced by the presence of fibrointimal proliferation, discontinuity of the internal elastic lamina, and/or mural disruption. No evidence of active arteritis was seen in any case. Antecedent trauma was documented in 12 cases and, in one of these, the subsequent formation of an arteriovenous malformation and development of the EH were noted. EH associated with damaged vessels or those in which a preceding history of trauma was available did not differ histologically from cases in which these variables were absent. Our findings suggest that a significant percentage of EH of soft tissue are not neoplastic, but arise on a reactive basis probably secondary to damage and repair of an artery or vein.

Adolescent↗

PubMed1990

Ectopic hamartomatous thymoma: clinicopathologic, immunohistochemical, and histogenetic considerations in four new cases.

Four new cases of ectopic hamartomatous thymoma are presented. The tumor occurred either superficially or deep in the area of the sternoclavicular joint and consisted of solid islands of squamous epithelium which blended with spindled cells. Cysts lined by squamous epithelium, small glands, and fat also occurred in variable amounts. Both the spindled and epithelial regions of the tumor expressed keratin and muscle actin, but neither desmin nor S100 protein. The tumor probably originates from thymic anlage associated with the third pharyngeal pouch (thymus III), although origin from other structures such as thymus IV and the cervical sinus of His are discussed. Our experience indicates that the large size and extreme cellularity of the spindled portion of some tumors may result in the mistaken diagnosis of sarcoma.

Actins↗

PubMed1990

Laryngeal and hypopharyngeal liposarcoma. A clinicopathologic study of 10 cases with a comparison to soft-tissue counterparts.

We report the clinicopathologic findings of 10 cases of primary laryngeal and hypopharyngeal liposarcoma. The tumors occurred in patients ranging in age from 37 to 77 years. They showed a marked male to female predominance of 9 to 1. The tumors, which occurred exclusively in the supraglottic larynx or hypopharynx (pyriform sinus), caused airway obstruction; this was the most common presenting symptom. All of the cases were well-differentiated liposarcomas (grade I). Atypical cells, scattered lipoblasts, and infiltration differentiated malignant laryngeal fatty tumors from benign ones. To date, none of these tumors has metastasized; however, multiple recurrences are common. Surgery is the treatment of choice; radiotherapy should be decided on an individual basis.

Adult↗

PubMed1990

A distinctive cardiovascular lesion resembling histiocytoid (epithelioid) hemangioma. Evidence suggesting mesothelial participation.

This paper presents 14 examples of a distinctive cardiovascular lesion. The patients' ages ranged from 5 to 76 years (mean, 51 years). There were seven male patients and seven female patients. All of the lesions were small and represented incidental surgical findings. Ten were attached to the endocardium, three were free-floating in the pericardial cavity, and one was inside a dissecting aneurysm of the ascending aorta. Microscopically, the lesions were enclosed in a fibrinous network and composed of a solid proliferation of round to polygonal cells with centrally located nuclei. Immunohistochemically, the cells were negative for FVIII-related antigen and lysozyme, but they stained positively for keratin, especially when clustered in small micropapillary or tubule-like formations. The nature and pathogenesis of these lesions are uncertain. Their location and some of their microscopic features originally suggested a relationship with the entity described as histiocytoid (epithelioid) hemangioma. However, their intense immunoreactivity for keratin, occasional presentation in the pericardial sac, and marked morphologic similarities with nodular mesothelial hyperplasia as sometimes seen in hernia sacs point toward the alternative possibility of a reactive mesothelial nature. A possible pathogenetic mechanism for the endocardial cases is ingrowth of pericardial mesothelial cells along a perforation tract that may have developed at the time of a cardiac catheterization. There were no recurrences or metastases in any of the cases.

Adult↗

PubMed1990

Eccrine spiradenoma occurring in infancy mimicking mesenchymal tumor.

Eccrine spiradenoma (ES) rarely (less than 1%) occurs in infancy. These tumors differ from the conventional ES by the presence of superficial dermal nodules which display a less distinct two-cell pattern of immature adnexal epithelial cells and rarely ductule formation. These tumors may be mistaken for mesenchymal neoplasms involving the skin and subcutis of infants and young adults. Recognition of the histopathologic features and immunostains are required to make a definite diagnosis. We describe 2 cases of ES occurring in patients younger than one year. Detailed histopathologic and histochemical differential features of these tumors and mesenchymal neoplasms of the skin and subcutis commonly occurring in infants and young adults are discussed. The biologic behavior of infantile ES is benign, but complete excision is recommended to prevent recurrence. We speculate that these tumors may represent congenital hamartomatous growths.

Adenoma, Sweat Gland↗

PubMed1990

Angiomatoid malignant fibrous histiocytoma. A follow-up study of 108 cases with evaluation of possible histologic predictors of outcome.

Described by Enzinger in 1979, angiomatoid malignant fibrous histiocytoma is a distinctive tumor of adolescence and early adult life characterized by sheets of relatively bland rounded or spindled cells separated by areas of cystic hemorrhage and surrounded by a prominent inflammatory infiltrate and often a fibrous pseudocapsule. On the basis of the original 41 cases, the tumor has been considered a low-grade malignancy. We are reporting the clinicopathologic findings and follow-up information of 108 new cases of angiomatoid malignant fibrous histiocytoma to determine the long-term behavior of this tumor and whether various histologic features (atypia, mitoses, infiltrative borders, and inflammatory infiltrate) are useful in predicting outcome. Follow-up information was obtained in 94 (87%) cases. Local recurrences developed in 11 patients (12%), all of whom were cured by re-excision. The initial excision in all patients developing local recurrence appeared to be incomplete. Local recurrence was statistically associated with irregular tumor border and head and neck location. Five patients developed metastases. Four had only local metastases, which responded to surgery, whereas the fifth patient developed presumed pulmonary and cerebral metastases and died. The development of both local and distant metastases was correlated with invasion into the deep fascia or muscle but not to various histologic parameters such as mitotic rate and pleomorphism. We conclude that angiomatoid malignant fibrous histiocytoma is intrinsically a low-grade tumor, and assessment of various histologic parameters or grading provides little or no additional prognostic information. Because death from disease occurred in only one patient (1%) in our series, it seems reasonable to reclassify angiomatoid malignant fibrous histiocytoma with fibrohistiocytic tumors of intermediate malignancy rather than with the conventional malignant fibrous histocytoma, the majority of which are high-grade sarcomas.

Adolescent↗

PubMed1989

Giant cell fibroblastoma. A juvenile form of dermatofibrosarcoma protuberans.

A clinicopathologic analysis of 28 cases of giant cell fibroblastoma (GCF), a rare mesenchymal tumor occurring predominantly in the first decade of life, is presented. This disease presented as a painless, slowly enlarging, subcutaneous mass. The tumor recurred locally in 47% of the patients; however, metastasis was not detected. On microscopic examination, GCF showed an unique combination of spindle cell patterns, pleomorphic and multinucleated giant cells, myxoid areas, and distinctive sinusoid-like spaces. This unrecognized histomorphologic picture led to a misdiagnosis of sarcoma in 40% of the cases. The histogenesis of this lesion remains uncertain; however, based on both clinical and morphologic similarities, it is proposed that GCF is a juvenile form of dermatofibrosarcoma protuberans (DFSP).

Adolescent↗