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Biomedical subjects

S W Weiss

Publications and source records attributed to S W Weiss.

At least 91 records · Page 5Linked to original sources

Ossifying fibromyxoid tumor of soft parts. A clinicopathological analysis of 59 cases.

We describe 59 cases of a microscopically unique neoplasm that has not been previously reported. The tumor almost exclusively affected adults (range 14-79 years) and had a male predominance (38 men and 21 women). It presented in most cases as a small, painless, well-circumscribed mass (median, 4 cm) in subcutis or muscle. It occurred chiefly in the upper and lower extremities (40 cases) and less frequently in the trunk (11 cases) and the head and neck region (eight cases). Microscopically, the tumor was partly lobulated and composed of small, round cells that had vesicular nuclei and indistinct cytoplasm. Typically, the cells were arranged in a cord- or nestlike pattern within a myxoid matrix that frequently showed transitions toward hyaline fibrosis and focal osteoid formation. In about two-thirds of the cases, the cells contained immunoreactive S-100 protein. An additional typical feature, seen in 48 (81%) of the 59 cases, was the presence of an incomplete shell of mature bone in the capsular region of the tumor. Follow-up information, available in 41 cases, revealed that 11 patients (27%) experienced one or more recurrences. One patient with three recurrences developed a second tumor in the opposite thigh, presumably a metastasis. None of the patients died of the tumor, but three died of causes unrelated to the disease. Although the histogenesis is uncertain, cartilaginous or neural origin seem to be most likely. Until this issue is resolved, we prefer the descriptive and less committal designation of "ossifying fibromyxoid tumor of soft parts."

Adolescent↗

Palisaded myofibroblastoma. A benign mesenchymal tumor of lymph node.

We report 22 examples of an unusual and distinctive benign mesenchymal tumor arising exclusively from lymph nodes of the groin. The tumor, which presents clinically as a swelling, is composed of spindled cells arranged in solid sheets or short, vaguely palisaded fascicles similar to a neurilemoma. The spindled cells blend gradually with large mats of eosinophilic material that appear as thick bands, ellipses, or circular profiles, depending on the plane of section. These eosinophilic structures, which represent a highly characteristic feature of the tumor, contain deeply eosinophilic, collagen-rich cores surrounded by a weakly eosinophilic, actin-rich cuff. The actin within these eosinophilic structures is derived by coalescence of intracellular actin globules extruded from neighboring cells. In all cases, a thin, compressed rim of normal lymph node was identified. Immunohistochemical analysis indicates that the cells express actin but lack S-100 protein, synaptophysin, desmin, keratin, and epithelial membrane antigen. Delicate, linear striations were identified in only two cases by conventional histochemical techniques. The foregoing features suggest that the tumor is related to a myofibroblast or a specialized smooth-muscle cell. These tumors, therefore, probably arise from smooth-muscle-like cells, which are normally present in some lymph node capsules or stroma. Follow-up information on 17 patients indicated that all are alive and well without any evidence of recurrence or metastasis.

Adult↗

Malignant lymphoma. A study of 75 cases presenting in soft tissue.

We report 75 cases of malignant lymphoma presenting in soft tissue taken from the files of the Armed Forces Institute of Pathology. All histologic subtypes with the exception of lymphoblastic lymphoma were represented. Our findings indicate that virtually any soft tissue site may be involved; there is no sex predilection; and size is not helpful in predicting survival. Among the 55 patients for which race was known, there were no black patients. Thirty-three patients with extensive evaluations at the time of diagnosis had no evidence of disseminated disease, but eight of these exhibited widespread disease within 3 months of diagnosis, and seven of the eight died of disease (median survival, 4 months). The remaining 25 patients had much better outcomes; 18 of 19 with intermediate and high-grade lymphomas were alive and well at a median of 74 months after diagnosis. Some tumors exhibited a propensity for involvement of remote soft tissue sites.

Humans↗

Pedal hemangioma (venous malformation) occurring in Turner's syndrome: an additional manifestation of the syndrome.

Two infants with Turner's syndrome and an unusual vascular tumor or malformation of the feet are described. In one child, the lesion developed in the dorsal metacarpal area, whereas in the other, the lesion was located in a bilaterally symmetrical distribution over the dorsum of the feet. Both lesions were characterized by a proliferation of tortuous, thick-walled veins with imperfectly formed muscular walls. Redundancy of the endothelial lining leading to intimal "webs" and intraluminal vascular channels was prominent. The similarity of these two lesions clinically and histologically suggests that they do not represent a fortuitous occurrence, but an additional manifestation of Turner's syndrome. Furthermore, their existence indicates that the vascular abnormality in Turner's syndrome may be more generalized than previously recognized and may include abnormalities of peripheral blood vessels in addition to those of the heart and aorta.

Foot Diseases↗

Postirradiation malignant fibrous histiocytoma expressing cytokeratin. Implications for the immunodiagnosis of sarcomas.

A malignant fibrous histiocytoma of the sacrum complicating the course of radiation therapy for endometrial carcinoma is presented. Although the tumor fulfilled the clinical, radiologic, and histologic criteria for a postirradiation malignant fibrous histiocytoma of bone, it also expressed cytokeratin. That this immunoreactivity reflected keratin synthesis by the tumor and not an unusual pattern of cross-reactivity with another intermediate filament such as vimentin is strongly suggested by the reproducibility of the immunoreactivity utilizing both polyclonal and monoclonal antibodies and extinction of the immunoreactivity following absorption of the primary antiserum with keratin proteins. This is the first reported instance of keratin expression by a malignant fibrous histiocytoma; it indicates that sarcomas apart from synovial sarcoma and epithelioid sarcoma may sometimes express this protein.

Aged↗

Multicystic mesothelioma. An analysis of pathologic findings and biologic behavior in 37 cases.

We report the clinicopathologic findings of 37 cases of multicystic mesothelioma. The tumor, which occurs most frequently in young to middle-aged women, affects chiefly the pelvic peritoneum--particularly the cul de sac, uterus, and rectum. It grows along the serosa as multiple, translucent, fluid-filled cysts. Occasionally, it manifests as a solitary or free-floating mass. The tumor is made up of mesothelial-lined cysts embedded in a delicate fibrovascular stroma. The mesothelial cells may be flattened or cuboidal. Adenomatoid change or squamous metaplasia of the mesothelium occurs in one-third of cases. In a significant percentage of cases, the stroma shows marked inflammatory changes that make it difficult to recognize the underlying neoplastic nature. Follow-up information in 25 patients showed that 21 patients were alive, two had died of tumor, and two died of other causes. One of the two patients who died of their tumors was an infant whose tumor showed transition to conventional mesothelioma; the other was a man who had refused therapy. The extent of tumor at the time of diagnosis did not predict survival. The low incidence of previous surgery, the lack of prior abdominal infections, and the documentation of disease-related mortality all support a neoplastic, rather than a reactive, basis for this lesion.

Abdominal Neoplasms↗

Myofibroblastoma of the breast. Sixteen cases of a distinctive benign mesenchymal tumor.

The clinical and pathologic findings of 16 examples of a distinctive stromal tumor of the breast designated as "myofibroblastoma" are reported. Eleven of the 16 patients were men, and the average age at presentation was 63 years. Fourteen were treated by local excision and two by simple mastectomy. None of the lesions recurred or metastasized. The tumors were grossly nodular and well-demarcated from the surrounding mammary tissue. Ducts and lobules were not engulfed by the neoplasm. Microscopically, the lesions were formed by uniform, slender, bipolar spindle cells haphazardly arranged in fascicular clusters separated by broad bands of hyalinized collagen. Ultrastructural examination of four lesions identified a predominance of myofibroblasts. Immunoreactivity for S-100 protein and cytokeratin was absent in the 10 tumors examined, but desmin immunoreactivity was focally present in three lesions. The differential diagnosis of myofibroblastoma includes reactive processes and benign neoplasms such as nodular and proliferative fascititis, fibromatosis, spindle-cell lipoma, neurofibroma, neurilemmoma, and leiomyoma. Malignant neoplasms such as stromal sarcoma, malignant fibrous histiocytoma, and spindle-cell or metaplastic carcinoma should not be confused with a myofibroblastoma. The clinical significance of this entity lies primarily in its recognition as a distinctive benign neoplasm.

Adult↗

Liposarcoma of the soft tissues.

A case report presents a liposarcoma of the extremities and an overall review of this disease entity. Past and present histologic classifications of liposarcoma are discussed, as are the clinical behavior and treatment options in relation to the surgical stage of the tumor. The importance of a well-planned biopsy site is stressed.

Adult↗

Sarcomas and sarcomatoid neoplasms of the major salivary gland regions. A clinicopathologic and immunohistochemical study of 67 cases and review of the literature.

Sixty-seven cases of sarcomas and sarcomatoid neoplasms of the major salivary gland regions were studied in order to determine the clinical and histomorphologic features and biologic behavior. Fifty-seven of these proved to be sarcomas and the two most common types were malignant schwannoma (11) and fibrosarcoma (9). Nine sarcomas could not be subclassified morphologically. Ten cases, originally believed to be sarcomas, proved by means of immunohistochemistry to be either carcinomas (five cases) or melanomas (five cases). Fifty-nine of the 67 cases occurred in the parotid gland regions, and the remaining eight occurred in the submandibular regions. Twenty of the 67 cases were thought to arise from within the gland, nine from paraglandular tissues, and insufficient data was present to anatomically categorize the other 38 cases. The mean age of occurrence was 42 years for men and 38 years for women. A swelling was the presenting symptom in 64 cases, with a mean duration of 4.3 months. Pain, tenderness, or paralysis were noted in 17 cases, but the swelling was painless in seven cases. Follow-up data of 42 sarcoma patients revealed that 17 experienced recurrences, 16 developed metastases, and 15 died of disease. These rates were lowest among patients with tumors arising from within the gland (Group I) and highest among those patients with tumors of paraglandular origin (Group III). Mean survival time for those dying of disease was 2.4 years, and a 5-year survival time appeared to be a significant indicator of cure. The most successful therapy was either parotidectomy (superficial or total) or a combination of surgery and radiation. The morphologic and the immunohistochemical evidence suggest that the majority of the tumors represent true sarcomas that may arise from undifferentiated pluripotential cells, but that the remainder (15%) represent epithelial malignancies.

Adolescent↗

The varied radiographic manifestations of retroperitoneal malignant fibrous histiocytoma revealed through 27 cases.

Malignant fibrous histiocytoma, a pleomorphic sarcoma, represents the most common soft tissue sarcoma of late adult life. Approximately 15 per cent of the cases of malignant fibrous histiocytoma arise within the abdominal cavity or retroperitoneum. Although tumors in this series arose in many different sites, their proximity to the kidney necessitated a nephrectomy in 44 per cent of the cases (12 of 27). In 3 cases calcification was noted, which varied from a few small speckled areas to extensive large, coarse densities. The masses tended to compress or displace adjacent structures and vessels radiographically. Renal, duodenal and cecal invasion occasionally occurred. Ultrasonically, most tumors were hypoechoic (9 cases), with a few having a mixed pattern (2). On computerized tomography the masses were well circumscribed, with computerized tomography numbers in the range of solid tissue except for a few small areas of decreased density. The vascularity was variable, with 8 tumors being hypovascular and 8 showing a moderate to hypervascular pattern. Interestingly, the tumor derived a part of its blood supply from the renal vasculature in 12 cases (44 per cent). Even though malignant fibrous histiocytoma often was diagnosed incorrectly as an intrarenal or adrenal neoplasm, a cleavage plane usually could be identified between the tumor and the renal parenchyma in 1 of the studies, especially on the angiogram. This finding suggested its extrarenal origin. Knowing the tendency of malignant fibrous histiocytoma to arise from or be physically near the kidney and/or adrenal gland in elderly men may be of some diagnostic value.

Adult↗

Estrogen receptor protein in bone and soft tissue tumors.

Thirty-three histologically diverse bone and soft tissue tumors were analyzed biochemically for the presence of estrogen receptor protein (ERP) and progesterone receptor by means of a conventional, commercially available, steroid-binding assay (dextran-coated charcoal method) on fresh frozen tissue. These results were compared with analysis of ERP by using a specific monoclonal antibody both in an enzyme immunoassay and on frozen tissue sections by using immunohistochemical procedures. Frozen tissue sections were also examined for the presence of estrogen and progesterone receptors using fluorescein-labeled steroids. Six of the 33 tumors (18%) contained low levels of ERP ranging from 19 to 73 fmol/mg as determined by the dextran-coated charcoal method. The remaining 27 cases contained no (less than 10 fmol/mg) ERP. The ERP-positive group included a fibromatosis, leiomyosarcoma, liposarcoma (2 cases), neural sarcoma, and a synovial sarcoma. Four were high grades sarcomas, and two were low grade sarcomas. There was excellent agreement between the ERP levels determined by the dextran coated charcoal method and those determined by enzyme immunoassay. ERP could not be demonstrated immunohistochemically on frozen tissue sections of the tumors even though it could be demonstrated in breast carcinomas serving as positive controls. The failure of the immunohistochemical technique may be related to the low levels of ERP in these tumors and the difficulty of detecting antigen at threshold levels. Cytochemical localization of receptor protein employing fluoresceinated steroids did not correlate with cytosolic ERP as determined by enzyme immunoassay or the dextran coated charcoal method. Moreover, the high level of background fluorescence gave rise to a significant amount of intraobserver and interobserver variation. Although the clinical significance of ERP protein in mesenchymal tumors is still uncertain, the present findings, coupled with various clinical observations suggesting hormonal dependency of some mesenchymal tumors, indicate that investigation of a larger group of patients amenable to statistical analysis is warranted.

Adolescent↗

Proliferative fibroblastic lesions. From hyperplasia to neoplasia.

It is rare to find a pathologist whose ideas and beliefs are so sound and pervasive as to influence our thinking decades after his writing. Yet such was the case with Dr. Stout, to whom we owe our basic classification of fibroblastic tumors, our concept of fibromatosis, and our diagnostic approach to fibrosarcomas. We need to continue the progress Dr. Stout made in these areas and to search for biochemical and molecular differences in these tumors with the hope that this knowledge will lead to new avenues for therapy.

Cell Transformation, Neoplastic↗

Epithelioid hemangioendothelioma and related lesions.

Epithelioid hemangioendothelioma (EH) is the prototype of a group of vascular tumors characterized by an epithelioid or histiocytoid endothelial cell. This family also includes the epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia) and epithelioid forms of angiosarcoma. This review discusses the principal clinical, pathologic, and biologic differences among these three lesions. In particular the various manifestations of EH of soft tissue, bone, lung (previously called intravascular bronchioloalveolar tumor), and liver are discussed. Long-term follow-up data of EH of soft tissue and lung are provided.

Bone Neoplasms↗

Spindle cell hemangioendothelioma. A low-grade angiosarcoma resembling a cavernous hemangioma and Kaposi's sarcoma.

Twenty-six cases of a newly recognized form of vascular tumor are presented. The tumor may occur at any age, has a male predominance, and develops preferentially in the dermis and subcutaneous tissues of the distal extremities. Histologically it combines the features of both a cavernous hemangioma and Kaposi's sarcoma. It is composed of thin-walled cavernous vessels which may be dilated, partially collapsed, or filled with organizing thrombi and phleboliths. These areas are intimately associated with spindled areas reminiscent of Kaposi's sarcoma. The spindled areas differ from Kaposi's sarcoma by the presence of occasional epithelioid endothelial cells, which sometimes display vacuolization. Follow-up information in 14 cases indicates that nine patients experienced "recurrences." One patient, who also received radiotherapy, developed regional lymph node metastasis 40 years after diagnosis and following 19 recurrences. No patient, however, has died of his disease, despite relatively limited surgical excision. The term "spindle cell hemangioendothelioma" is suggested for this vascular tumor of low-grade malignancy.

Adolescent↗

Pigmented dermatofibrosarcoma protuberans (Bednar tumor). A pathologic, ultrastructural, and immunohistochemical study.

Described by Bednar as a "storiform neurofibroma," pigmented dermatofibrosarcoma protuberans is a rare neoplasm accounting for approximately 1-5% of all cases of dermatofibrosarcoma protuberans (DFSP). The lesion commonly presents as an exophytic, multinodular neoplasm of the dermis or subcutaneous tissue. It occurs predominantly in blacks. The majority are located on the trunk, and the remainder are more or less equally distributed in the upper and the lower extremities and the head and neck. Microscopically the lesion is characterized by spindled cells arranged in a tight storiform pattern and admixed with a small population of melanin-containing dendritic cells. The dendritic cells are the primary feature distinguishing this lesion from conventional DFSP. Three cell populations are identifiable by electron microscopy. The majority of cells resemble fibroblasts. A second population of cells exhibits long slender cell processes partially or completely invested by basal lamina. The third population of cells, also invested by basal lamina, contains both melanosomes and premelanosomes. The histogenesis of this neoplasm remains controversial. Although Bednar considered these lesions as variants of neurofibroma, S-100 protein could not be identified, and this finding contrasts significantly from the description of conventional neurofibroma, which almost always contains this antigen. Follow-up information available in nine cases indicates that this lesion may recur locally. Although distant metastases were not observed in our material, complete excision in conjunction with close follow-up care is indicated for this neoplasm of probable intermediate malignant potential.

Adolescent↗

Keratin in epithelioid sarcoma. An immunohistochemical study.

Intermediate-sized filaments have been noted in epithelioid sarcoma by previous investigators, two of whom have reported that the filaments represent vimentin. We utilized polyclonal antibodies directed against keratin and immunoperoxidase techniques (PAP) to stain 32 of the more than 300 cases accumulated at the AFIP . All of our material was formalin-fixed, paraffin-embedded. Seventy-five percent of our cases (24/32) showed positive immunoreactivity, a feature that may be of diagnostic help in distinguishing epithelioid sarcoma from modular fasciitis, benign and malignant fibrous histiocytoma, malignant melanoma, and necrotizing granuloma. In these cases, the reaction was enhanced using predigestion with trypsin. The immunoreactivity varied from tumor to tumor, perhaps due to formalin fixation. Since synovial sarcoma and mesothelioma may also be cytokeratin-positive, our findings indicate that keratin immunoreactivity is not confined to epithelial tumors and may also occur in neoplasms traditionally regarded as mesenchymal.

Adolescent↗