Biomedical subjects

J F Fetsch

Publications and source records attributed to J F Fetsch.

27 records · Page 2Linked to original sources

PubMed1997

Sclerosing perineurioma: a clinicopathologic study of 19 cases of a distinctive soft tissue lesion with a predilection for the fingers and palms of young adults.

This report describes 19 cases of a distinctive sclerosing perineurial tumor of the hands. Fourteen patients were male and five were female (age range 9-55 years; median age 24.5 years). The process typically presented as a painless mass and was present from 6 months to 40 years before resection. Sites of involvement were the thumb (n = 6); index (n = 3), middle (n = 4), and ring (n = 4) fingers; and the palm (n = 2). The lesions were generally well marginated but nonencapsulated. They had a firm, fibrous consistency and ranged in size from 0.7 to 3.3 cm in maximum dimension. Microscopic examination showed abundant dense collagen and variable numbers of small, epithelioid, and spindled cells exhibiting corded, trabecular, and whorled (onion bulblike) growth patterns. Immunoreactivity was present for epithelial membrane antigen (15 of 15); a cytokeratin cocktail containing AE1, AE3, and CK1 (four of 14); CAM 5.2 (one of 12); vimentin (12 of 12); muscle-specific actin (nine of 14); alpha-smooth muscle actin (six of 14); collagen IV (six of six); laminin (five of six); and CD99 (three of five). Ultrastructural features consistent with perineurial cells were noted. All of the lesions were locally excised. Follow-up was obtained for seven patients, with mean and median follow-up intervals of 12 years 7 months and 10 years 6 months, respectively. None of the lesions have recurred. This study advances the morphologic spectrum of perineurioma, a rare tumor of nerve sheath derivation. Familiarity with this distinctive subtype should help to avoid confusion with other processes, including a fibroma of tendon sheath, the sclerotic fibroma associated with Cowden's disease, an epithelioid neurofibroma, a late stage of tenosynovial giant cell tumor, and sclerosing adnexal tumors.

Adolescent↗

PubMed1997

Superficial angiomyxoma (cutaneous myxoma): a clinicopathologic study of 17 cases arising in the genital region.

Seventeen cases of superficial angiomyxoma (cutaneous myxoma) of the genital region are reported. Thirteen patients were female (age range: 15-33 years; mean: 21 years) and four were male (age range: 18-55 years; mean: 39 years). The sites of involvement in females were the labium majus or labium, not otherwise specified (n = 6), vulva (n = 4), groin (n = 2), and mons pubis (n = 1). All lesions in male patients involved the scrotum. The tumors were present from 2 months to 4 years before resection and ranged from 0.9 to 6 centimeters in maximal dimension; 10 tumors were 3 centimeters or less in size. The predominant reason for seeking medical attention was a slow growing painless mass. All lesions were locally excised. Follow-up was obtained for 9 patients with a mean and median follow-up interval of 135 and 95 months, respectively. A recurrence developed in three patients at 8 months, 7 years 11 months, and 20 years. No patient has been shown to have Carney's complex. The tumors were immunoreactive for vimentin (11/11), CD34 (11/11), muscle-specific actin (8/12), smooth muscle actin (9/11), S100 protein (5/13), and Factor XIIIa (5/9). No immunoreactivity was present for desmin (DE-R- 11), glial fibrillary acidic protein, estrogen receptor or progesterone receptor. Superficial angiomyxomas are probably derived from fibroblast-like cells capable of antigen modulation.

Adolescent↗

PubMed1996

Aggressive angiomyxoma: a clinicopathologic study of 29 female patients.

BACKGROUND: Aggressive angiomyxoma is an uncommon mesenchymal tumor that preferentially involves the pelvic and perineal regions of females. Since its initial description in 1983, approximately 65 cases have been reported in the English literature. METHODS: The clinical and pathologic features of 29 cases of aggressive angiomyxoma were evaluated in a review of archival material from the Armed Forces Institute of Pathology (1960-1992). Histochemical stains for mucosubstances and immunohistochemistry (avidin-biotin complex method) were utilized to characterize the neoplasms further. RESULTS: All patients were females, between 16 and 70 years (median; 34 years). The soft tissues of the pelvis, perineum, vulva, buttock, retroperitoneum, and inguinal regions were involved. The majority of the tumors were > or = 10 centimeters in greatest dimension. Follow-up ranging from 8 to 198 months (mean, 93 months; median, 95 months) was available for 22 patients. Eight patients developed recurrent tumor, from 10 months to 7 years after the initial resection. No patient developed metastases and there were no tumor related deaths. Histologically, the neoplasms were sparsely to moderately cellular and predominantly composed of bland, relatively nondescript. stellate and spindled cells embedded in a loosely collagenized matrix with scattered vessels of varied caliber. A few cases contained some tumor cells with more abundant eosinophilic cytoplasm that raised the possibility of focal smooth muscle differentiation. The tumor matrix was no more than weakly reactive for mucosubstances; thus, while glycosaminoglycans are present to a limited extent, edema fluid appears to be a major component of the noncollagenous stroma. The neoplastic cells were at least focally immunoreactive for desmin (22/22), smooth muscle actin (19/20), muscle specific actin (16/19), vimentin (17/17), CD34/QBEND-10 (8/16), and estrogen (13/14) and progesterone (9/10) receptor. All of the examined tumors were negative for S100 protein (20/20). Ki67 (MIB1) immunoreactivity was present in <1% of the tumor nuclei in all 16 cases tested. CONCLUSIONS: Aggressive angiomyxoma is a distinctive, locally aggressive, mesenchymal tumor that appears to be relatively site specific and has a peak incidence in females in the fourth decade of life. There is a strong propensity for local recurrence but metastatic disease has not been reported. Since the first evidence of recurrence may be many years after the initial resection, long term follow-up is required. The neoplastic cells of aggressive angiomyxoma exhibit fibroblastic and myofibroblastic features and appear to be hormonally influenced. The possibility that the progenitor cell has a capacity for smooth muscle differentiation is raised.

Adolescent↗

PubMed1995

A review of 40 patients with extraskeletal osteosarcoma.

BACKGROUND: Extraskeletal osteosarcomas are rare malignancies that account for about 1% of all soft tissue sarcomas. Few large series have been reported. METHODS: Clinical records and histologic slides of all patients with extraskeletal osteosarcomas treated at the Mayo Clinic between 1915 and 1988 were reviewed. RESULTS: The study group consisted of 40 patients, most of whom presented in the sixth and seventh decades of life (mean age, 50.7 years). There was a male predominance (male-to-female ratio, 1.9:1). The lower limbs most commonly were involved (68%), usually the thigh and buttock regions. Ninety-three percent of tumors presented as an enlarging soft tissue mass, with a history of trauma in nine patients. In nine patients, the lesions were initially interpreted histologically as benign, most commonly as myositis ossificans. Histologically, all were high grade osteosarcomas. Multiple local recurrences (45%) are a feature of this tumor. All recurrences occurred within 3 years. Distant metastasis (65%) is also common and is usually to the lungs (81%). Radical resections appear to be the best option for local control, with resection of the pulmonary metastasis occasionally producing a cure. By univariate analysis of Kaplan-Meier survival curves, the patients with predominantly chondroblastic tumors fared better than those with predominantly osteoblastic tumors (P = 0.03). Analysis of survival differences of the three main subtypes together (osteoblastic, chondroblastic, and fibroblastic) was not significant. A small-sized initial lesion did not equate with better survival. Seventy-three percent died of the disease, with a mean follow-up of 5.9 years. CONCLUSION: Extraskeletal osteosarcoma is a high grade malignant tumor associated with a 5-year survival rate of 37% (95% confidence interval, 28%-59%). Local recurrences and distant metastasis are common and usually occur by 3 years after excision. Patients with the chondroblastic subtype survive longer than those with the osteoblastic subtype.

Adult↗

PubMed1993

Synovial sarcoma of the abdominal wall.

BACKGROUND: Synovial sarcoma usually arises in the extremities and in close proximity to large joints. Reported examples arising in the anterior abdominal wall are rare. Because most accounts from this location consist of case reports, neither clinical nor prognostic features have been well delineated. METHODS: Twenty-seven synovial sarcomas of the abdominal wall (SSAW), retrieved from the Soft Tissue Registry of the Armed Forces Institute of Pathology, were reviewed and analyzed retrospectively. Immunohistochemical stains were performed in 18 cases. RESULTS: There were specimens from 12 male and 15 female patients, ranging in age from 8 to 58 years (median, 23 years). The tumors were classified as biphasic (14), predominantly monophasic fibrous (8), and poorly differentiated (5) types. The neoplasms occasionally were cystic and typically reacted, at least focally, with antibodies directed against keratin and/or epithelial membrane antigen. Eight tumors were smaller than 5 cm.; 17 tumors were 5 cm or larger in size. Dimensions were not recorded in two cases. Follow-up, ranging from 1 to 264 months, was obtained in 18 cases. Nine individuals were alive and well and eight were dead of disease at median follow-up intervals of 98 and 26 months, respectively. One patient was dead with disease, possibly secondary to chemotherapy-related causes. All nine patients who have died had clinical evidence of metastatic disease. Patients who presented with tumors 5 cm or larger had a less favorable outcome than whose with tumors smaller than 5 cm. (58% versus 40% mortality). A positive correlation was noted between increased mitotic activity and the mortality rate. Although patients with biphasic and predominantly monophasic fibrous tumors had a similar mortality rate (40% for both groups), patients with poorly differentiated synovial sarcoma fared worse (100% mortality). In general, poorly differentiated synovial sarcomas had a higher mitotic rate than either biphasic or monophasic fibrous examples. No appreciable difference in survival was evident based on the age at presentation (< 15 versus > or = 15 years of age). CONCLUSIONS: The survival rate for patients with SSAW is similar to that reported for synovial sarcoma in general. A high mitotic rate and the poorly differentiated subtype of synovial sarcoma both were associated with a poor prognosis and, to some extent, appear interrelated. Less favorable behavior also was noted when the tumors were large (> or = 5 cm). Pluripotential or arthrogenous mesenchyme may be implicated in the pathogenesis of these tumors.

Abdominal Muscles↗

PubMed1993

Calcifying fibrous pseudotumor.

We report 10 cases of a distinctive benign fibrous lesion characterized by the presence of abundant hyalinized collagen with psammomatous or dystrophic calcifications and a lymphoplasmacytic infiltrate. The lesions were present from 2 months to 10 years before resection and ranged in size from 2.5 to 15 cm. They involved subcutaneous and deep soft tissues and, although relatively well-circumscribed, occasionally infiltrative borders or entrapped structures were seen on microscopic examination. The lesions were located in the extremities (three cases), trunk (two cases), scrotum (two cases), groin (one case), neck (one case), and axilla (one case). Both sexes were equally affected. The mean and median ages of the patients were 16.2 and 18.5 years, respectively (range, 1 to 33 years). All cases were initially managed by simple local excision. Follow-up ranging from 2 months to more than 10 years (median, 41.5 months) was available in six cases and revealed a local recurrence in one instance; this became clinically apparent about 7.5 years after the initial resection. Morphologic features and follow-up data suggest this may be a unique form of fibrous pseudotumor.

Adolescent↗

PubMed1992

MR imaging of extraabdominal desmoids: differentiation from other neoplasms.

A retrospective review of MR images of 36 patients with histologically proved extraabdominal desmoids was done to define the MR characteristics of these tumors and to determine if MR could be used to differentiate desmoids from other benign and malignant soft-tissue neoplasms. The desmoids evaluated included eight primary and 30 recurrent lesions. Our study was conducted in parallel with another study in which the MR appearance of 95 benign and malignant soft-tissue masses was evaluated, and the MR images of these masses were compared with our findings. The four desmoids from that study are included in our data. The signal intensity of the tumor on T1- and T2-weighted images was graded relative to the intensities of muscle and fat. Homogeneity, margin, neurovascular and bone involvement, and fibrosis (low-signal regions within the tumor on both T1- and T2-weighted images) were evaluated. On MR imaging, the desmoids showed inhomogeneous signal (97%), poor margination (89%), neurovascular involvement (58%), and bone involvement (37%). Fibrosis was present in 88% of primary desmoids and 90% of recurrent ones, and intermediate signal (greater than that of muscle and less than that of fat) was present in 75% and 50% of these, respectively. Our results show that the MR features of desmoids have characteristics that are commonly found in malignant tumors (inhomogeneous signal, poor margination, and neurovascular involvement). MR features of desmoids that distinguish them from malignant neoplasms are the presence of fibrosis and intermediate signal in the regions of the tumor.

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↗