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PubMed1990

Granular cell tumors of the vulva.

Granular cell tumor (GCT), although nearly ubiquitous, is seen infrequently in the vulva. A review of the surgical pathology files from Grady Memorial Hospital, Atlanta, Georgia, from 1983 through 1987 identified eight cases of vulvar GCT. Five of the eight patients had more than one skin and soft tissue lesion. Two of the five had biopsy-proven multicentric GCT with a unique clinical course. One of the patients was a 32-year-old woman with multiple vulvar, lingual, laryngeal, bronchial and pulmonary GCT, necessitating multiple excisions and ultimately pneumonectomy. The second patient had multiple GCTs in the vulva and inguinal area and finally in both lungs, resulting in her death at age 39. No dependable microscopic features could be identified to distinguish benign GCT from its more aggressive variant. However, Feulgen DNA histomorphometry demonstrated aneuploidy in the patient with apparent lung metastases, whereas the tumors from patients with a benign course as well as from the patient with multiorgan involvement were diploid. In three of five patients who could be interviewed there was a history of soft tissue tumors in members of the family. The multifocal nature and possible familial component of GCT need to be explored further.

Adult↗
PubMed1995

Benign and malignant muscle tumors in the hand and forearm.

Although soft tissue tumors are common in the hand and forearm, muscle tumors are relatively rare. Although presentation may be similar between benign and malignant muscle tumors, prognosis can differ tremendously. Accurate and early diagnosis is critical to minimize morbidity.

Diagnosis, Differential↗
PubMed2004

Myofibroblastic inflammatory tumors of the bladder.

Bladder myofibroblastic inflammatory tumors are rare benign proliferative lesions that can simulate both urothelial and connective tissue malign neoplasms, making a preoperative diagnosis nearly impossible. Conservative treatment appears to be curative, even though local recurrences have been reported, and so malignancy should be ruled out above all by means of an accurate follow-up. Two cases are described, 1 of which with the longest follow-up period reported so far (8 years).

Adult↗
PubMed2005

Post-menopausal vaginal angiomyofibroblastoma: a case report.

Angiomyofibroblastoma (AMF) is a recently described, rare, benign soft tissue vulvovaginal tumour that occurs mainly but not exclusively in the vulval region of pre-menopausal women (Fletcher et al. in Am J Surg Pathl 16:373; 1992). The first case was diagnosed in 1992. We report a case of a post-menopausal woman with a 2-month history of a rapidly growing painless vaginal tumour and thus drawing the attention of gynaecologist as well as general practitioners to the fact that this rare phenomenon can occur outside the vulva.

Female↗
PubMed2000

Is anti-h-caldesmon useful for distinguishing smooth muscle and myofibroblastic tumors? An immunohistochemical study.

Misinterpretation of positive staining of antibodies to desmin, smooth muscle actin, and muscle actin as representing smooth muscle differentiation in the context of a spindle cell tumor is not uncommon. Anti-h-caldesmon is a promising novel immunohistochemical reagent for more specific smooth muscle differentiation. We studied 72 tumors (11 leiomyosarcomas, 26 malignant fibrous histiocytomas [MFHs], 11 fibromatoses, 11 cellular cutaneous fibrous histiocytomas [CCFHs], 5 malignant peripheral nerve sheath tumors, 4 synovial sarcomas, and 4 cases of nodular fasciitis), the reactive myofibroblastic response in 5 cases of acute cholecystitis, and the desmoplastic response surrounding 5 invasive breast carcinomas. Tissues were examined for expression of h-caldesmon, desmin, smooth muscle actin, and muscle actin. Diffuse staining for h-caldesmon was present only within the leiomyosarcomas. Focal staining for h-caldesmon involving less than 1% of lesional cells was present in 3 of 26 MFHs and 1 of 11 CCFHs. There was overlap in staining for the other "myoid" markers in all of the lesions that contained myofibroblasts. Anti-h-caldesmon seems to be a reliable marker of smooth muscle differentiation, and its inclusion in a panel of myoid immunohistochemical reagents should allow distinction of smooth muscle and myofibroblastic tumors.

Actins↗
PubMed2001

Myofibrosarcoma: a clinicopathologic study.

The concept of soft tissue sarcomas composed predominantly of myofibroblasts has been controversial. We examined a series of such lesions of low- and intermediate-grade malignancy to further define their clinical and pathologic features. Histologic appearances of four cases diagnosed as myofibrosarcoma by electron microscopy were reviewed. Eleven additional cases with similar morphology were then identified from 249 tumors originally indexed as fibrosarcoma. Electron microscopy was performed on five of these, and immunohistochemistry was carried out on all cases for which material was available. There were 11 men and 4 women aged 33 to 73 years (median, 54 yrs; mean, 53 yrs). Lesions mainly involved the head and neck, extremities, and trunk and ranged in size from 1.5 to 12 cm. The tumors were composed of bland or pleomorphic stellate to spindled cells with eosinophilic cytoplasm and tapered nuclei in a collagenous stroma; 10 were grade 1 and five were grade 2. All sarcomas displayed fascicular or storiform patterns, and some of the grade 1 lesions superficially mimicked nodular fasciitis. Electron microscopy of nine cases showed myofibroblastic differentiation, and immunohistochemistry showed smooth muscle actin in 13 of 15 cases, muscle-specific actin in 7 of 9, desmin in 6 of 14, and cytokeratin in 0 of 11. Four of nine grade 1 and three of four grade 2 tumors recurred (one twice), and one grade 2 tumor metastasized to the lungs. Myofibrosarcomas are indolent low-grade or occasionally aggressive intermediate-grade sarcomas which can be recognized by light microscopy. Their clinical importance lies in the resemblance, particularly of low-grade examples, to reactive or pseudosarcomatous conditions.

Adult↗
PubMed2002

Expression of ALK1 and p80 in inflammatory myofibroblastic tumor and its mesenchymal mimics: a study of 135 cases.

Abnormalities of chromosome 2p23 with expression of ALK1 and p80 occur in both inflammatory myofibroblastic tumor (IMT) and anaplastic large cell lymphoma. This immunohistochemical study investigates whether the ALK family of neoplasms includes fibroblastic-myofibroblastic, myogenic, and spindle cell tumors. Formalin-fixed paraffin-embedded archival tissues from 10 IMTs and 125 other soft tissue tumors were stained for ALK1 and p80 with standard immunohistochemistry. ALK1 and/or p80 reactivity was observed in a cytoplasmic pattern in IMT (4/10; 40%), malignant peripheral nerve sheath tumor (4/10; 40%), rhabdomyosarcoma (6/31; 19%), leiomyosarcoma (1/10; 10%), and malignant fibrous histiocytoma (1/11; 9%). No staining was observed in nodular fasciitis, desmoid, infantile myofibromatosis, infantile fibrosarcoma, synovial sarcoma, leiomyoma, or myofibrosarcoma. Alveolar rhabdomyosarcomas (4/16; 25%) displayed a distinctive dot-like cytoplasmic positivity. No cases displayed nuclear reactivity. Fluorescent in situ hybridization on 12 of the positive cases revealed a combination of abnormalities including ALK break-apart signals, nucleophosmin (NPM)/ALK fusions, or extra copies of 2p23. This study demonstrates that in addition to IMT, abnormalities of ALK1 and p80 expression with a variety of structural chromosomal changes are found in several sarcomas, especially rhabdomyosarcoma and malignant peripheral nerve sheath tumor. Although immunoreactivity in non-IMTs cannot distinguish between structural abnormalities involving 2p23 or additional copies of 2p23, it supports the concept of ALK involvement in a larger group of neoplasms, some of which have other documented clonal abnormalities. In IMT, immunohistochemistry for ALK1 and p80 is useful as an indicator of a 2p23 abnormality, but it must be interpreted in the context of histologic and other clinicopathologic data if used as an adjunct to differential diagnosis.

Activin Receptors, Type I↗