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PubMed1991

Focal myositis, a benign inflammatory pseudotumour: CT appearances.

Focal myositis is a rare benign pseudotumour of skeletal muscle, of unknown cause. Clinically it presents as an enlarging mass within muscle, usually of an extremity, and is often mistaken for a soft tissue neoplasm. The diagnosis is made by biopsy which reveals characteristic histological changes of inflammation, focal degeneration and regeneration, and some evidence of denervation. The CT findings include irregularity and enlargement of the muscles involved, with diffuse, poorly defined fatty infiltration of the muscle planes, but no evidence of an associated mass. We present two cases of focal myositis of the calf, both of which mimicked a soft tissue neoplasm, and in which CT was helpful in determining the nature and extent of the abnormality, for needle biopsy, and follow-up.

Adult↗
PubMedDate not supplied

Alveolar soft-part sarcoma of the orbit.

Alveolar soft-part sarcoma is a malignant soft tissue neoplasm that involves mainly the deep soft tissues of the extremities, particularly the thighs and the buttock. Involvement of the orbit is uncommon. We describe a young child with such a tumor and illustrate the characteristic features.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1998

Controversial issues in diagnosis of liposarcomas of the thigh. A report of 18 cases.

Liposarcoma is a common soft tissue neoplasm mainly found in the thigh. Early diagnosis and appropriate treatment are essential for survival, but clinical diagnosis may not be easy, and the importance of imaging is controversial as it often fails to indicate the presence of liposarcoma so that treatment is delayed. We report our experience of 18 cases of liposarcoma (16 of which in the thigh). This experience has led us to revise our opinions about imaging, clinical, and histopathological diagnosis, and the surgical and medical treatment. We now suggest that any hard, subfascial mass in an adult should be considered as a soft tissue neoplasm. Of these neoplasms, liposarcoma is the most common type, after malignant fibrous histiocytoma. Diagnosis by imaging techniques is rarely possible, so that integration with clinical data is essential. Among the various imaging techniques, computed tomography and ultrasound seem to be most useful.

Adult↗
PubMed2006

Diffuse-type tenosynovial giant cell tumor in association with neurofibromatosis type 1-Noonan syndrome: possibly more than a chance relationship.

A case of diffuse-type tenosynovial giant cell tumor arising in the left upper extremity is reported in a 23-year-old man with neurofibromatosis type 1 (NF1)-Noonan syndrome. The predominately mononuclear cellular proliferation with psammomatous calcifications had the immunohistochemical and ultrastructural features of a fibrohistiocytic neoplasm. This uncommon type of soft tissue neoplasm occurring in this unique clinical setting served to open an inquiry into the subject of non-neurogenic tumors in association with NF1 and Noonan syndrome, both manifested in our patient. Nonossifying fibroma of bone as a presumptive fibrohistiocytic tumor is an uncommon but well-documented manifestation in NF1, whereas in Noonan-like syndrome, both giant cell granuloma and pigmented villonodular synovitis are recognized as associated lesions with histologic and immunophenotypic similarities with the diffuse-type tenosynovial giant cell tumor.

Adult↗
PubMed1992

Vascular soft tissue tumors: medical imaging.

Histologically, tumors of the vascular system are divided into three categories. Benign types can be localized (hemangioma) or involve large segments of the body (angiomatosis). Vascular tumors of intermediate malignancy are known as hemangioendotheliomas. Angiosarcoma is one of the rarest malignant soft tissue neoplasms. The authors present the morphological and SI features of 17 histologically proven vascular tumors (16 benign, 1 malignant) examined by MRI. The results are compared with the findings on other imaging techniques. On T1-WI, the tumors are slightly hyperintense to muscle with interspersed areas of low SI corresponding to fibrous septa, calcifications, or fastly flowing blood, and areas of SI equal to fat. On T2-WI, the lesions are more homogeneous and markedly hyperintense to fat. MRI is superior to all other imaging techniques for evaluation of extent and characterization of vascular soft tissue tumors. Angiography remains helpful in demonstrating feeding or draining vessels.

Adolescent↗
PubMed1993

Radiosensitivity in vitro of human soft tissue sarcoma cell lines and skin fibroblasts derived from the same patients.

Skin fibroblast cell strains and tumour cell lines were established from 12 patients with various types of soft tissue neoplasms, and radiation survival curve parameters were measured in vitro. Soft tissue sarcoma cells were consistently more sensitive to X-irradiation than fibroblasts isolated from the same patient, and were also more sensitive as a group than cell lines derived from 34 other human tumours. There was a general correlation in radiosensitivity between fibroblasts and tumour cells derived from the same patient, indicating that some component of tumour cell sensitivity may relate to genetic factors in the host. Such genetic factors, however, do not explain all of the heterogeneity in tumour cell response. The response of soft tissue sarcoma in vivo may be dependent on complex radiomodifying factors other than inherent radiation sensitivity, thus making it difficult to predict clinical outcome by use of assays which use survival of irradiated tumour cell lines in vitro as an endpoint.

Adult↗
PubMed1999

Fluorodeoxyglucose positron emission tomography of soft tissue tumours: is a non-invasive determination of biological activity possible?

Since musculoskeletal tumours comprise a large heterogeneous group of entities with different biological behaviour, clinical diagnosis of such lesions can be very difficult. The aim of this prospective study was to assess the usefulness of 2-[F-18]-fluoro-2-deoxy-d-glucose (FDG) positron emission tomography (PET) in the non-invasive evaluation of soft tissue tumours. One hundred and two patients with suspected soft tissue neoplasms were investigated by FDG-PET. The uptake of FDG was evaluated semiquantitatively by determining the tumour to background ratio (TBR). All patients underwent biopsy, resulting in the histological detection of 39 high-grade sarcomas, 16 intermediate-grade sarcomas, 11 low-grade sarcomas, 25 benign tumours, 10 tumour-like lesions such as spontaneous myositis ossificans (n = 6) and one non-Hodgkin lymphoma. All lesions except for two lipomas disclosed an increased FDG uptake. Sarcomas showed significantly higher TBR values than latent or active benign lesions (P<0.001) and aggressive benign lesions (P<0.05). Using a TBR cut-off level of 3.0 for malignancy, sensitivity of FDG-PET was 97.0%, specificity 65.7% and accuracy 86. 3%. From our data there are three main conclusions: (1) Except for patients with pseudotumoral myositis ossificans, lesions with a TBR >3 were sarcomas (91.7%) or aggressive benign tumours (8.3%). (2) Tumours with a TBR <1.5 were latent or active benign lesions, exclusively. (3) The group with intermediate TBR values (<3 and >1. 5) comprised primarily latent or active benign lesions, but also four aggressive benign tumours and two low-grade sarcomas. Our data suggest that FDG-PET represents a useful tool for the evaluation of the biological activity of soft tissue neoplasms.

Female↗
PubMedDate not supplied

[Dixon's technic for fat suppression. Its potential applications in combination with gadolinium-DTPA].

Modification of the Dixon technique enables the reconstruction of water and fat images operating at midfield strength. The technique was combined with intravenous administration of gadolinium-DTPA in five patients with lumbosacral spine conditions, four patients with soft tissue neoplasms and three with skeletal neoplasms; all patients were examined at 0.5 T. Mean attenuation of fat signal in T1-weighted images was 85% and implied a redistribution of the gray-scale dynamic range. This allowed easier appreciation of normal and abnormal enhancement in both areas containing fat tissue and areas without it. This was useful in the evaluation of fresh scars in two patients recently submitted to lamino-discectomy and of the prevertebral extension of the inflammatory process in one patient with spondylodiscitis. In the patients with soft tissue neoplasms the combination of fat suppression and Gadolinium was helpful to assess lesion size. Finally, in the patients with skeletal neoplasms fat-suppressed images obviated for the lesion disappearance observed on conventional T1-weighted SE images after contrast administration. The combination of intravenous administration of gadolinium-DPTA with Dixon's fat-suppression technique is a new promising capability of midfield MRI.

Adipose Tissue↗
PubMed2005

Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature.

PEComas, occasionally associated with the tuberous sclerosis complex, are defined by the presence of perivascular epithelioid cells that coexpress muscle and melanocytic markers. This family of tumors includes angiomyolipoma (AML), clear cell sugar tumor of the lung (CCST), lymphangioleiomyomatosis (LAM), and very rare tumors in other locations. Because non-AML/non-LAM PEComas are extremely rare and their natural history and prognostic features undefined, we present our experience with 26 PEComas of soft tissue and the gynecologic tract, the largest series to date. We also performed a detailed review of the literature, with special attention to features predictive of clinical behavior. All PEComas exclusive of AML and LAM were retrieved from our consultation files. Immunohistochemistry for pan-cytokeratin (CK), S-100 protein, smooth muscle actins (SMA), desmin, vimentin, HMB45, Melan-A, microphthalmia transcription factor (MiTF), TFE3, CD117, and CD34 was performed. Clinical follow-up information was obtained. Fisher's exact test was performed. The median patient age was 46 years (range, 15-97 years); there was a marked female predominance (22 females, 4 males). Sites of involvement included the omentum or mesentery (6 cases), uterus (4 cases), pelvic soft tissues (3 cases), abdominal wall (2 cases), uterine cervix (2 cases), and vagina, retroperitoneum, thigh, falciform ligament, scalp, broad ligament, forearm, shoulder, and neck (1 case each). The tumors ranged from 1.6 to 29 cm in size (median, 7.8 cm). Tumors were epithelioid (N = 9), spindled (N = 7), or mixed (N = 10). Multinucleated giant cells were present in 18 cases. High nuclear grade was noted in 10 cases, high cellularity in 7 cases, necrosis in 8 cases, and vascular invasion in 3 cases. Mitotic activity was 0 to 50 mitotic figures (MF)/50 high power fields (HPF) (median, 0 MF/50 HPF) with atypical MF in 6 cases. IHC results were: SMA (20/25), desmin (8/22), HMB45 (22/24), Melan-A (13/18), MITF (9/18), S-100 protein (8/24), CK (3/23), vimentin (12/14), TFE3 (5/17), c-kit (1/20), and CD34 (0/7). Clinical follow-up (24 of 26 patients, 92%; median, 30 months; range, 10-84 months) showed 3 local recurrences and 5 distant metastases. At last available clinical follow-up, 2 patients (8%) were dead of disease, 4 patients (17%) were alive with metastatic or unresectable local disease, and 18 patients (75%) were alive with no evidence of disease. No patient in our series had a history of tuberous sclerosis complex. Recurrence and/or metastasis was strongly associated tumor size > median size (8 cm), mitotic activity greater than 1/50 HPF, and necrosis. We conclude that PEComas of soft tissue and gynecologic origin may be classified as "benign," "of uncertain malignant potential," or "malignant." Small PEComas without any worrisome histologic features are most likely benign. PEComas with nuclear pleomorphism alone ("symplastic") and large PEComas without other worrisome features have uncertain malignant potential. PEComas with two or more worrisome histologic features should be considered malignant. Occasional PEComas express unusual markers, such as S-100 protein, desmin, and rarely CK. The role of TFE3 in PEComas should be further studied.

Adolescent↗
PubMed1993

Bednar tumor pattern in recurring giant cell fibroblastoma.

Giant cell fibroblastoma (GCF) and Bednar tumor (BT) have been perceived as two rare, not directly related, soft tissue neoplasms. Recent studies, separately conducted on each process, have presented evidence indicating that, in each instance, the tumor seemed to be related to the dermatofibrosarcoma protuberans. The case being reported provides additional evidence supporting those previous results and, for the first time, documents a direct link between GCF and BT, as seen in this girl who had a vulvar GCF resected at 1-year of age, with recurrence, 1.5 years later, as a mixed BT-GCF.

Female↗
PubMed2000

Primary giant cell tumor of soft tissues: a study of 22 cases.

Twenty-two cases of giant cell tumor of soft tissues (GCT-ST) identified in the Mayo Clinic files and the consultation files of two of the authors (A.G.N., C.D.M.F.) were analyzed clinicopathologically. Age at presentation ranged from 5 to 80 years (median, 43 years), and there was no sex predilection (12 male, 10 female). Duration of symptoms ranged from 2 to 12 months (median, 4.5 months), and a painless growing mass was the most common complaint. The lower limbs were the most frequent location (50%), followed by the trunk (31.8%) and the upper limbs (13.6%). The size of the tumors ranged from 1 to 10 cm, and they tended to be superficial (86.4%), forming well-circumscribed (72.7%), multinodular (86.4%) masses. Histologically, all tumors consisted of a mixture of mononuclear cells showing vesicular, round to oval nuclei and osteoclastlike, multinucleated giant cells distributed uniformly throughout the tumors. Foci of stromal hemorrhage were observed in 11 tumors (50%); nine tumors (40.1%) showed metaplastic bone formation and six (27.2%) showed aneurysmal bone cystlike areas. Necrosis was absent in all but one tumor. Mitotic figures were present in all but one tumor, ranging from two to more than 30 mitoses per 10 high-power fields (HPFs; median, 9.5 mitoses per 10 HPFs) and were typical in aspect. Vascular invasion was identified in seven tumors (31.8%), and none of the tumors showed marked cellular atypia or pleomorphism. The tumors were treated surgically, and follow-up information was available for 16 patients (duration of follow-up, 2 to 130 months; median, 51 months). Only one of the 16 patients (6.2%) had local recurrence and lung metastases; this patient died of the tumor. In conclusion, GCT-ST occurs as a primary soft-tissue neoplasm and is identical clinically and morphologically to giant cell tumor of bone. Provided that GCT-ST is treated adequately by complete excision, a benign clinical course is expected because episodes of distant metastasis and tumor-associated death seem to be exceedingly rare.

Adolescent↗
PubMed2002

Plexiform fibrohistiocytic tumor: a rare low-grade malignancy of children and young adults.

Plexiform fibrohistiocytic tumor (PFT) is a low-grade, superficial, soft tissue neoplasm with a limited but significant ability to metastasize. This type of tumor only rarely presents in the skin of the head and neck. Clinicians first encountering young patients with facial neoplasia, such as a PFT, might be unaware of its exact oncologic potential and instead be primarily concerned with the cosmetic outcome. We treated a 17-year-old boy with a PFT on his cheek who was initially treated only by shave biopsy. The tumor subsequently recurred and metastasized to the cervical lymph nodes 3 years after the initial biopsy. Therefore, appropriate initial therapy for PFT requires complete excision with negative resection margins.

Adolescent↗
PubMed1998

Congenital fibrosarcoma of the upper extremity.

Congenital or infantile fibrosarcoma is a rare soft-tissue neoplasm that should be considered in the differential diagnosis of a large extremity mass presenting at birth. These tumors are notoriously misdiagnosed at birth as either hemangiomas or lymphatic malformations. Definitive diagnosis is made by physical examination, special radiologic studies, and biopsy. Although histologically similar to fibrosarcomas occurring in adults, the congenital lesions differ in their clinical behavior; metastases are rare, local recurrence is common, and the prognosis is good with wide local excision combined with chemotherapy. Amputation should be reserved for chemoresistant patients in whom the involvement of neurovascular structures by the tumor make a limb-sparing aggressive excision impossible.

Adult↗
PubMed2005

A review and update of morphologically bland vulvovaginal mesenchymal lesions.

Vulvovaginal mesenchymal lesions composed of morphologically bland spindle-shaped cells often pose a particular diagnostic problem for the surgical pathologist not only because of the rarity of these lesions but also because of the wide array of entities with overlapping morphologic features. Included in this group of lesions are soft tissue neoplasms that may arise at any site and those that are characteristic of, or relatively specific to, the vulvovaginal region. Lesions that are relatively specific to the vulvovaginal region include well-known neoplasms such as aggressive angiomyxoma and angiomyofibroblastoma as well as more recently described lesions such as cellular angiofibroma and superficial cervicovaginal myofibroblastoma. Fibroepithelial stromal polyp, superficial angiomyxoma, and smooth muscle neoplasms also can occur in, but are not specific to, this site. In this review, the clinicopathologic features of these lesions are described with an emphasis on recent developments. The value of ancillary studies, especially immunohistochemistry, is discussed, although it is stressed that in general these are of limited value and routine morphology remains the mainstay in diagnosis. Morphologically bland spindle cell lesions that are not characteristic of the vulvovaginal region, but which also may occur here, are briefly discussed as are a variety of extremely rare mesenchymal lesions that have recently been described at this site.

Angiofibroma↗