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PubMed2000

Epithelioid/granular soft tissue lesions: correlation of cytopathology and histopathology.

Polygonal shaped epithelioid cells are not an infrequent component of soft tissue mass lesions. Such cells exist in association with other cellular shapes (eg, spindle, pleomorphic, stellate, etc) in both tumors and tumor-like conditions. Conversely, only a handful of soft tissue lesions contain either a pure or predominant epithelioid cell population. The cytoplasmic texture of such cells may be finely or coarsely granular, striated, vacuolated, or have combinations of these features. Since a relatively small body of literature on fine needle aspiration cytopathology of soft tissue exists (as compared to other body sites), and there is a lack of using the fine needle aspiration technique for soft tissue tumors in most medical centers, most pathologists are precluded from having the opportunity to correlate the cytopathology and histopathology of epithelioid soft tissue lesions. The aim of this review is to highlight the morphologic features (as seen in both tissue sections and aspiration smears) of this group of soft tissue lesions in order to assist the reader in their recognition, discuss the cytomorphologic and histomorphologic similarities and differences among these lesions, and delineate the limitations in their recognition particularly with the cytologic method.

Biopsy, Needle↗

PubMed2000

Nonrhabdomyosarcoma soft tissue sarcomas in children: is age at diagnosis an important variable?

PURPOSE: The associations between age at diagnosis, tumor characteristics, and outcome in children diagnosed with nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) were studied. METHODS: Retrospective review was conducted of 192 children from 1962 through 1996. Patients were divided into groups: birth to 1 year (n = 13), 1 to 5 years (n = 26), 5 to 10 years (n = 49), 10 to 15 years (n = 55), and older than 15 years (n = 49) of age at diagnosis. Characteristics including IRS group, histological grade and pattern, tumor size, and invasiveness were investigated. Survival rate was estimated by age group. The median follow-up was 8.8 years (range, 2 to 28 years). RESULTS: There were 81 group I patients, 40 group II, 41 group III, and 30 group IV. A significant difference of IRS groups among the age groups was seen (P = .034). There were no IRS group IV patients less than 1 year of age; 50% of IRS group IV patients were older than 15 years. A significant difference in the distribution of histological grade among the age groups (P = .032) was seen. Ten of 13 (77%) children less than 1 year of age had low-grade tumors, whereas 42%, 45%, 60%, and 37% of patients aged 1 to 5, 5 to 10, 10 to 15, and older than 15 years, respectively, had low-grade tumors. Patients older than 15 years had the highest incidence of invasive tumors (59%). Histological pattern also varied with age. The most prevalent histology in the less-than-1-year age group was infantile fibrosarcoma. No predominant histology was seen in the 1- to 5-year age group. Malignant fibrous histiocytoma was the most frequent histological subtype in children between 5 and 10 years of age. In the 10- to 15-year age group and children older than 15 years the malignant peripheral nerve sheath tumor and synovial sarcoma were the most prevalent subtypes. Without adjusting for any other factors, age group was prognostic of survival (P = .007). Patients less than 1 year at diagnosis had the best outcome, with a 5-year survival rate of 92% +/- 9%. Five-year estimates were lowest for patients older than 15 years (49% +/- 7%). CONCLUSIONS: Significant differences in IRS group, histological grade, and histological subtype were observed in different age groups. Infants with NRSTS were more likely to have low grade, less invasive, and lower stage tumors. These characteristics may account for their improved prognosis.

Adolescent↗

PubMed2003

Cloning of an Alpha-TFEB fusion in renal tumors harboring the t(6;11)(p21;q13) chromosome translocation.

MITF, TFE3, TFEB, and TFEC comprise a transcription factor family (MiT) that regulates key developmental pathways in several cell lineages. Like MYC, MiT members are basic helix-loop-helix-leucine zipper transcription factors. MiT members share virtually perfect homology in their DNA binding domains and bind a common DNA motif. Translocations of TFE3 occur in specific subsets of human renal cell carcinomas and in alveolar soft part sarcomas. Although multiple translocation partners are fused to TFE3, each translocation product retains TFE3's basic helix-loop-helix leucine zipper. We have identified the genes fused by the chromosomal translocation t(6;11)(p21.1;q13), characteristic of another subset of renal neoplasms. In two primary tumors we found that Alpha, an intronless gene, rearranges with the first intron of TFEB, just upstream of TFEB's initiation ATG, preserving the entire TFEB coding sequence. Fluorescence in situ hybridization confirmed the involvement of both TFEB and Alpha in this translocation. Although the Alpha promoter drives expression of this fusion gene, the Alpha gene does not contribute to the ORF. Whereas TFE3 is typically fused to partner proteins in subsets of renal tumors, we found that wild-type, unfused TFE3 stimulates clonogenic growth in a cell-based assay, suggesting that dysregulated expression, rather than altered function of TFEB or TFE3 fusions, may confer neoplastic properties, a mechanism reminiscent of MYC activation by promoter substitution in Burkitt's lymphoma. Alpha-TFEB is thus identified as a fusion gene in a subset of pediatric renal neoplasms.

Base Sequence↗

PubMed1995

Hyaline-cell rich chondroid syringoma. A tumor mimicking malignancy.

We report three cases of chondroid syringoma composed almost entirely of hyaline cells and occurring in the soft tissues. These lesions were confused with a variety of malignant neoplasms, including malignant melanoma, myxoid chondrosarcoma, and alveolar soft part sarcoma. The tumors occurred in two men and one woman aged 37, 75, and 71 years, respectively, and involved the heel, great toe, and face. All cases arose in the subcutaneous tissue or fascial layer and ranged in size from 0.8 to 2.0 cm. The neoplasms were composed of plasmacytoid hyaline cells with variable amounts of myxoid stroma. Two of the chondroid syringomas contained rare duct structures. One contained cartilage lobules but no ducts. All were well circumscribed. Immunohistochemistry showed positive staining for keratin, S-100, and vimentin in all cases. Muscle markers, including desmin, smooth-muscle actin, and muscle-specific actin, were negative. Follow-up has showed no evidence of recurrence or metastasis.

Adenoma, Pleomorphic↗

PubMed1990

Detailed investigation of the diagnostic value in tumour histopathology of ICR.2, a new monoclonal antibody to epithelial membrane antigen.

The production and detailed immunostaining properties of a new rat monoclonal antibody (ICR.2) to epithelial membrane antigen are reported. The antibody was selected for its ability to compete with the polyclonal antiserum (M7), used in the original immunohistological studies, in order that it might serve as a direct replacement in diagnosing epithelial tumours. Most of the staining reactions on normal tissues were identical to those previously reported with M7 but there were some important differences. They included: positivity of renal and adrenal capsular fibroblasts, perineurium, some myoepithelial and smooth muscle cells, occasional osteoblasts and squamous and thyroid follicular epithelium in the normal state. The intercellular canaliculi of sweat glands and secretory canaliculi of gastric oxyntic cells were clearly demonstrated. These staining reactions could be obtained with M7 when a sensitive detection system was used although the results were usually weak and inconsistent. Nearly all adenosquamous and transitional carcinomas were positive. The remaining tumours fell into three major groups: (1) those which were consistently or nearly consistently negative--melanoma, seminoma, rhabdomyosarcoma, alveolar soft part sarcoma, adrenal cortical carcinoma, granulocytic sarcoma, paraganglioma, non-Hodgkin's lymphoma. Hodgkin's disease and embryonal carcinoma: (2) those which were either negative or positive with distinctive patterns of staining--basal cell carcinoma, embryonal tumours: and (3) non-epithelial tumours that were consistently positive--epithelioid sarcoma, synovial sarcoma, osteosarcoma, chordoma and myeloma--or positive in a significant minority of cases--leiomyosarcoma, malignant fibrous histiocytoma, clear cell sarcoma of tendon sheath, various neuroectodermal tumours.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

PubMed1979

A clinicopathological study on soft tissue tumors of the head and neck.

The present study consists of 651 cases of soft tissue tumors originating in the head and neck area. Among these tumors 628 were benign (96%), while there were only 23 malignant cases (4%). In the benign group hemangiomas showed the highest incidence (47%) followed by tumors of adipose tissue (19%) and those of peripheral nerves (12%). Rare tumors consisted of nodular fascitis, hibernoma, lipoblastomatosis, glomus tumor, granular cell tumor, and chondroma. The majority of cases in the malignant group were either those of muscle origin or blood vessel origin. Excepting for two cases of rhabdomyosarcoma of the embryonal type and two cases of angiosarcoma, all showed recurrence and/or metastasis and terminated in death. Three cases of liposarcoma of either well-differentiated or myxoid type had a good prognosis with all three living for 3 or more years without any recurrence. Other malignant tumors consisted of malignant schwannoma, fibrosarcoma, malignant fibrous histiocytoma, alveolar soft part sarcoma, and chordoma. The age, sex, anatomical location, and differential diagnosis have also been described.

Adolescent↗

PubMed1984

Distribution of laminin, fibronectin, and interstitial collagen type III in soft tissue tumours.

The distributions of laminin, fibronectin, and interstitial collagen type III have been investigated in a series of 60 soft tissue tumours by immunochemistry. Positive laminin staining was seen in sites predicted by the distribution of ultrastructurally visible basal lamina. Pericellular laminin was present in all benign tumours of Schwann cell and smooth muscle origin examined, in the two malignant Schwannomas examined, and in six of 13 leiomyosarcomas. It was also evident around nests of cells in an alveolar soft part sarcoma and around malignant endothelial cells in an angiosarcoma. In fibroblastic and fibrohistiocytic tumours it was found only in blood vessel walls. The results of laminin staining led to revision of the original histopathological diagnosis in seven of the 60 cases studied. Fibronectin was abundant in the stroma of most neoplasms, both benign and malignant. It was also found in a distribution parallel to that of laminin. In some tumours this was clearly distinguishable from the distribution of interstitial collagen. Intracellular fibronectin was shown consistently only in mast cell granules. Its demonstration in synovial cells, fibroblasts, and histiocytes was more variable. Interstitial collagen type II had the most irregular distribution of the three proteins. It was as plentiful in tumours of smooth muscle origin as in tumours of fibroblastic origin, but was scanty in fibrous histiocytomas. Its distribution appeared similar to that of laminin and fibronectin in leiomyomas, but differed from these two proteins in Schwann cell tumours and other neoplasms. In one leiomyosarcoma fibronectin, laminin, and type III collagen appeared to be lost concomitantly from tumour cell peripheries.

Collagen↗

PubMed2004

Melanocytes and the microphthalmia transcription factor network.

The first mouse microphthalmia transcription factor (Mitf ) mutation was discovered over 60 years ago, and since then over 24 spontaneous and induced mutations have been identified at the locus. Mitf encodes a member of the Myc supergene family of basic helix-loop-helix zipper (bHLH-Zip) transcription factors. Like Myc, Mitf regulates gene expression by binding to DNA as a homodimer or as a heterodimer with another related family member, in the case of Mitf the Tfe3, Tfeb, and Tfec proteins. The study of Mitf has provided many insights into the biology of melanocytes and helped to explain how melanocyte-specific gene expression and signaling is regulated. The human homologue of MITF is mutated in patients with the pigmentary and deafness disorder Waardenburg Syndrome Type 2A (WS2A). The mouse Mitf mutations therefore serve as a model for the study of this human disease. Mutations and/or aberrant expression of several MITF family member genes have also been reported in human cancer, including melanoma (MITF), papillary renal cell carcinoma (TFE3, TFEB), and alveolar soft part sarcoma (TFE3). Genes in the MITF/TFE pathway may therefore also represent valuable therapeutic targets for the treatment of human cancer. Here we review recent developments in the analysis of Mitf function in vivo and in vitro and show how traditional genetics, modern forward genetics and in vitro biochemical analyses have combined to produce an intriguing story on the role and actions of a gene family in a living organism.

Alleles↗

PubMed1997

Skeletal metastases from soft-tissue sarcomas. Incidence, patterns, and radiological features.

We reviewed 277 patients with soft-tissue sarcoma (STS) treated between 1975 and 1995 to study the incidence, distribution, time of appearance, and radiological findings of skeletal metastases. Of these, 28 (10.1%) had metastases within a mean period of 18.6 months after admission. The incidence of skeletal metastases differed among the histological subtypes of sarcoma; alveolar soft-part sarcoma, dedifferentiated liposarcoma, angiosarcoma, and rhabdomyosarcoma tended to show higher incidences. The regional bones close to the primary tumour were affected in 13 (46.4%) of the 28 patients, and the axial bones in 18 (64.3%). Radiologically, the metastatic bony lesions predominantly showed osteolytic changes, and there were pathological fractures in 21 of 44 lesions.

Adolescent↗

PubMed1986

The ribosome-lamellar complex; is it a normal structure?

Ribosome-lamellar complexes were seen in mast cells in the iris of a patient with Usher's syndrome (retinitis pigmentosa associated with deafness) and in the neoplastic cells of an alveolar soft part sarcoma of the thigh. These 'organelles', originally described and associated with haematopoietic malignancy, where they have been described as the hallmark, are being increasingly often described in neoplastic and nonneoplastic cells. We believe that their usefulness in diagnosis should be questioned and the concept of their association with malignancy abandoned.

Adult↗

PubMedDate not supplied

Crystalline inclusions in a subependymal giant cell tumor in a patient with tuberous sclerosis.

Unusual crystalline cytoplasmic inclusions were encountered in tumor cells in a subependymal giant cell tumor (SEGT) in a 16-year-old girl with tuberous sclerosis. By electron microscopy, the tumor cells demonstrated typical features previously described in SEGT, including abundant dense bodies, prominent Golgi complexes, abundant mitochondria, rough and smooth endoplasmic reticulum, scattered intermediate filaments and microtubules, glycogen, and rare synaptic contacts as well as primitive intercellular junctions. The dense bodies were bound by a single membrane and were round, ovoid, irregular, or cylindric in appearance with electron-dense homogenous content or fingerprint profiles. Of note was the presence of numerous cytoplasmic rhomboidal or rectangular crystalline inclusions akin to those seen in alveolar soft part sarcoma. These inclusions measured as much as 8 microns in length and had 7-nm periodicities, often with intersecting lamellae. Rarely, the membrane-bound dense bodies showed areas of similar periodicities, indicating that the crystalline inclusions are related to and might originate from the dense bodies. While crystalline inclusions have previously been described in one patient with SEGT (Bender and Yunis, Ultrastruct Pathol 1980; 1:287-299), the inclusions in the present case were a striking feature and add to the spectrum of the ultrastructural pathology of SEGT.

Adolescent↗

PubMedDate not supplied

Intracisternal collagen fibrils in proliferative fasciitis and myositis of childhood.

During ultrastructural examination of a difficult to diagnose tumor, short spacing collagen fibrils (periodicity of banding, approximately 43.2 nm) and native collagen fibrils (periodicity of banding, approximately 53 nm) were found in dilated and vacuolated cisternae of rough endoplasmic reticulum and the perinuclear cistern. Original diagnoses from several histopathologists included alveolar soft part sarcoma, malignant fibrous histiocytoma, atypical fibroxanthoma, and myogenic tumor. The finding of intracisternal collagen (which is but a variety of intracellular collagen) led to the conclusion that this was a fibroblastic neoplasm. This, plus a review of the histologic findings, led to the diagnosis of proliferative fasciitis and myositis of childhood.

Adolescent↗

PubMed2000

Granular cell traumatic neuroma: a lesion occurring in mastectomy scars.

BACKGROUND: Granular cell changes can be observed in a variety of benign and malignant tumors, and are seen more commonly in granular cell tumors, which in about 5% of cases develop in the breast. Granular cells also have been observed in sites of previous trauma, such as surgery, and are found to be inflammatory reactions of histiocytic origin. METHODS AND RESULTS: We investigated, morphologically and immunohistochemically, 2 granular cell lesions occurring in mastectomy scars after surgery for carcinoma. Both lesions were composed of strands and nests of large granular cells, haphazardly set in a background of fibrous tissue, with sparse inflammatory infiltrates. Several tortuous hypertrophic nerve bundles were also embedded in the fibrous tissue. A few of these nerve bundles showed degenerative changes and contained granular cells. Immunohistochemically, granular cells were positive for S100 protein, neuron-specific enolase, vimentin, and CD68 antigen. CONCLUSIONS: We consider these proliferative lesions of peripheral nerves to have the features of both granular cell tumor and traumatic neuroma. These cases indicate that traumatic neuroma can undergo extensive granular cell changes and constitute a previously unrecognized entity, which we provisionally label granular cell traumatic neuroma. Granular cell traumatic neuroma has to be taken into consideration when evaluating lesions occurring at mastectomy scars and should be differentiated from malignant tumors with granular cells, such as apocrine carcinoma and alveolar soft part sarcoma.

Aged↗

PubMedDate not supplied

Jejunoileal alveolar rhabdomyosarcoma. A case report.

Intestinal localization of rhabdomyosarcoma is exceptional, this case is the first to be published in the world literature. A 35-year-old patient with abdominal pain, fever, was found to have an infiltrative white-grey tumour, involving 20 cm the jejunoileal wall and also the surrounding mesenterium up to the origin of upper mesenterical vessels and lymph nodes. Histologic examination showed an alveolar type of rhabdomyosarcoma intricated with solid undifferentiated tumoral cells. The presence of multinucleated giant cells and the positivity of protein S 100 reaction was important for differential diagnosis, given the alveolar soft part sarcoma, malignant mesothelioma, malignant melanoma or papillary carcinoma.

Adult↗

PubMed1992

Crystalloids in angiomyolipoma. 1. A previously unnoticed phenomenon of renal angiomyolipoma occurring at a high frequency.

We present a description of unique crystalloids in renal angiomyolipoma that have not previously been reported. The crystalloids cannot be identified by hematoxylin-and-eosin staining. Detailed observation after diastase treatment followed by PAS staining revealed needle- and rod-like crystalloids, which were clearly seen even by light microscopy, in 11 of 17 patients. Their appearance was characterized by the following phenomena: (a) They appeared mainly in large epithelioid smooth-muscle cells; (b) they appeared at a relatively high frequency at sites where smooth-muscle cells showed diffuse proliferation and where a hemangiopericytic pattern was observed; (c) they were often detected easily even at a site with a sarcomatous appearance; and (d) PAS-positive, diastase-resistant granules were often observed by light microscopy in the vicinity of crystalloids in all 17 patients. Electron-microscopic observation of one patient also revealed characteristic crystalloids. Prior to our study, only one patient had been reported to show crystalloids by electron microscopy, and the crystalloids were interpreted as renin. However, our study used Bowie's staining and immunohistochemistry to prove they were not renin. The nature of the crystalloids still needs to be elucidated. The fact that they closely resemble structures seen in alveolar soft part sarcoma provides one clue to their identification.

Adult↗

PubMed1977

[Neuroectodermal tumors of the skin (a normative study)].

A group of bioptically examined neuroectodermal tumours of the skin and soft tissues consisting, in particular, of 170 Schwannomas and 350 tumours has been reevaluated in a retrospective study. The following common classification of these tumours has been recommended: I. Tumours of Schwann's cells: 1. neurilemmoma [A and B], 2. neurofibroma [with the following variants v. Recklinghausen's type, plexiform, pigmented, Paccinian, with Meissner-Wagner's bodies and meningiomatous], 3. amputation neuroma, 4. neurosarcoma, 5. others. II. Melanogenic tumours: A. pigmented naevi (junction, mixed, intradermal, epithelioid, clear cell, halo, neurocutaneous, fibrous, blue, proliferating blue, melanotic progonoma, others). -B. praecancerous melanosis. -C. malignant melanoblastoma (common type, from praecancerosis). III. Tumours of ganglion cells: 1. ganglioneuroma, 2. neuroblastoma, 3. paragangliomas (with granules, without granules, alveolar soft part sarcoma).

Ganglioneuroma↗

PubMed1983

Proteins of intermediate filaments. An immunohistochemical and biochemical approach to the classification of soft tissue tumors.

The intermediate filament cytoskeleton of various types of human soft tissue tumors was analyzed by immunofluorescence microscopy with the use of specific antibodies against cytokeratins, vimentin, and desmin, as well as by one- and two-dimensional gel electrophoresis of high-salt buffer- and detergent-resistant cytoskeletal preparations. All leiomyomas as well as a leiomyosarcoma contained desmin. Leiomyomas of both gastrointestinal and uterine derivation and the retroperitoneal leiomyosarcoma showed strong reaction for desmin in the smooth muscle cells, but the latter two exhibited also vimentin staining. In embryonal rhabdomyosarcomas, desmin prevailed in the large, apparently well-differentiated rhabdomyoblasts; whereas the smaller, less differentiated tumor cells preferentially contained vimentin. Cells of malignant fibrous histiocytomas were characterized by their content of vimentin as the only intermediate filament protein present. In alveolar soft part sarcoma, a rare tumor of hitherto unknown histogenesis, vimentin and desmin co-existed within the same tumor cells, indicating, together with chemical determinations, the myogenic derivation of this neoplasm. The results show that immunologic and biochemical analysis of proteins associated with the intermediate filament cytoskeleton is a useful adjunct in the diagnosis of diverse neoplasms, particularly those with equivocal histologic features, and thus aids in the histogenetic classification of soft tissue tumors.

Desmin↗

PubMedDate not supplied

[A case of soft tissue alveolar sarcoma localized in the tongue].

Alveolar soft-part sarcoma is a rare form of malignant tumour. It is generally localized at the limb muscles, rarely in the head and neck. Its histogenesis is still uncertain but its specific cellular characters make the diagnosis easier. The case of a woman with an ASPS of the tongue is described. The morphological aspects and the immunohistochemical results seem, according to the data reported in literature, suitable with the diagnosis of alveolar soft-part sarcoma; however, it is difficult to explain the presence of local positivity to specific neuronal enolase.

Antineoplastic Agents↗