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Clear cell sarcoma arising from the chest wall: a case report.

Clear cell sarcoma is a rare malignant soft tissue neoplasm that usually arises adjacent to tendons or aponeuroses. The clinical course is rather slow, with repeated local recurrences followed by late metastases and eventual death. The principal sites of this neoplasm are the extremities, but tumors do occur in the trunk on rare occasions. We report a case of clear cell sarcoma arising from the chest wall. The patient, a 20-year-old woman, had noticed a chest wall mass and pain for 2 years. Biopsy of the mass showed abundant nests of round cells with clear cytoplasm. On immunohistochemical examination, tumor cells were strongly immunoreactive for S-100 and HMB-45. A diagnosis of clear cell sarcoma was confirmed. There was no other lesion found in the patient through routine imaging studies. She was treated with two courses of chemotherapy using ifosfamide, carboplatin, and etoposide. Subsequently, the tumor, including adjacent tissue, the chest wall, and sternum, was resected with a wide margin; and the defect of the chest wall was covered with Marlex mesh fabric, regin, and a musculocutaneous flap. She has shown no symptoms or signs of recurrence during 2 years of follow-up.

Adult↗

Cytodiagnosis of malignant melanoma of soft tissue: report of a case with diagnosis by intraoperative cytology.

BACKGROUND: Malignant melanoma of soft tissue (MMST) is a rare tumor and consists of < 1% of all soft tissue neoplasms. There are few reports on its cytodiagnosis. CASE: A 14-year-old male attended the Department of Orthopedics, Juntendo University Urayasu Hospital, in August 1994 because of a painless tumor in the distal portion of the left thigh. Intraoperative imprint smear examination led to a diagnosis of malignant melanoma, and wide resection of the tumor, including the surrounding normal tissue, was performed. On cytologic examination, the background was relatively clean, with tumor cells distributing individually or in clusters. Under high magnification, the tumor cells were seen to contain a slightly enlarged, conspicuous nucleolus and large cell body. The cells varied in shape from polygonal to spindle shaped, with a few multinuclear giant cells. Melanin and glycogen were observed in varying degrees in the tumor cells. CONCLUSION: MMST can be diagnosed easily if melanin is observed in the cytoplasm. Even in the absence of melanin, the tumor has relatively characteristic cytomorphology. Intraoperative cytology is useful for an accurate diagnosis of the tumor.

Adolescent↗

From the archives of the AFIP: benign musculoskeletal lipomatous lesions.

Benign lipomatous lesions involving soft tissue are common musculoskeletal masses that are classified into nine distinct diagnoses: lipoma, lipomatosis, lipomatosis of nerve, lipoblastoma or lipoblastomatosis, angiolipoma, myolipoma of soft tissue, chondroid lipoma, spindle cell lipoma and pleomorphic lipoma, and hibernoma. Soft-tissue lipoma accounts for almost 50% of all soft-tissue tumors. Radiologic evaluation is diagnostic in up to 71% of cases. These lesions are identical to subcutaneous fat on computed tomographic (CT) and magnetic resonance (MR) images and may contain thin septa. Lipomatosis represents a diffuse overgrowth of mature fat affecting either subcutaneous tissue, muscle or nerve, and imaging is needed to evaluate lesion extent. Lipoblastoma is a tumor of immature fat occurring in young children, and imaging features may reveal a mixture of fat and nonadipose tissue. Angiolipoma, myolipoma, and chondroid lipoma are rare lipomatous lesions that are infrequently imaged. Spindle cell and pleomorphic lipoma appear as a subcutaneous lipomatous mass in the posterior neck or shoulder, with frequent nonadipose components. Hibernoma appears as a lipomatous mass with serpentine vascular elements. Benign lipomatous lesions affecting bone, joint, or tendon sheath include intraosseous lipoma, parosteal lipoma, liposclerosing myxofibrous tumor, discrete lipoma of joint or tendon sheath, and lipoma arborescens. Intraosseous and parosteal lipoma have a pathognomonic CT or MR appearance, with fat in the marrow space or on the bone surface, respectively. Liposclerosing myxofibrous tumor is a rare intermixed histologic lesion commonly located in the medullary canal of the intertrochanteric femur. Benign lipomatous lesions may occur focally in a joint or tendon sheath or with diffuse villonodular proliferation in the synovium (lipoma arborescens) and are diagnosed based on location and identification of fat. Understanding the spectrum of appearances of the various benign musculoskeletal lipomatous lesions improves radiologic assessment and is vital for optimal patient management.

Adolescent↗

Expression of receptor tyrosine kinases epidermal growth factor receptor and HER-2/neu in synovial sarcoma.

BACKGROUND: Synovial sarcomas are high-grade soft tissue neoplasms often characterized by a biphasic spindle and epithelioid cell morphology. The majority of synovial sarcomas harbor a specific chromosomal translocation in which the proximal portion of the SYT gene at chromosome 18q11 is fused to the distal portion of one of several duplicated SSX genes (most notably SSX1 and SSX2) at chromosome Xp11. SYT/SSX1 translocations are seen in nearly three times as many synovial sarcomas as SYT/SSX2 translocations. Although the SYT/SSX2 fusion is usually associated with the monophasic disease pattern, the SYT/SSX1 fusion is present in tumors with biphasic or monophasic patterns. The SYT/SSX1 fusion gene is associated with more aggressive tumor growth and poor outcome. Despite advances in the therapy of local disease, distant metastasis remains the predominant cause of death. Accordingly, there is a need for alternate therapies, such as those recently developed against the receptor tyrosine kinases, such as epidermal growth factor receptor (EGFR) and HER-2/neu. METHODS: Archival specimens of synovial sarcoma (n=38) representing 30 patients were assessed for EGFR and HER-2/neu protein expression by standard immunohistochemical techniques. To validate the immunohistochemistry results, quantitative real-time polymerase chain reaction (Q-PCR) assays using either fresh and/or archival material was performed. The presence of gene amplification was determined by chromogenic in-situ hybridization. RESULTS: EGFR and HER-2/neu protein were detected by immunohistochemistry in 21 of 38 (55.3%) and 20 of 38 (52.6%) synovial specimens, respectively. EGFR immunoreactivity showed a granular and membranous pattern, whereas HER-2/neu immunoreactivity demonstrated only a membrane pattern. Coexpression was observed in 13 of 38 specimens (34.2%). HER-2/neu expression by immunohistochemistry in synovial sarcomas was restricted to tumors with the SYT/SSX1 translocations. Of 6 specimens with SSX2 translocation, none (0%) showed HER-2/neu immunoreactivity and 1 (17%) demonstrated EGFR expression. Q-PCR demonstrated the presence of mRNA for EGFR and HER-2/neu in 19 of 30 specimens (63.3%) and 22 of 30 specimens (73.3%), respectively. EGFR and HER-2/neu were expressed at low concentrations compared with the expression of glyceraldehyde 3-phosphate dehydrogenase (GAPDH). No evidence of gene amplification was observed. CONCLUSIONS: EGFR and HER-2/neu are expressed in the majority of patients with SYT/SSX1 synovial sarcomas, albeit at low levels. Treatment with tyrosine kinase inhibitors may represent appropriate alternate therapy for these patients.

Adolescent↗

[Clinicopathologic features of dendritic fibromyxolipoma].

OBJECTIVE: To study the clinicopathologic features of dendritic fibromyxolipoma for the purpose of differentiating it from other confusable soft tissue neoplasms. METHODS: Eight cases of dendritic fibromyxolipoma were obtained and their clinicopathologic features were studied. Immunohistochemistry stains for CD34, bcl-2, vimentin, cytokeratin, EMA, S-100, HHF35 and smooth muscle actin were performed with labeled streptavidin-biotin (SLAB) system on DAKO auto-immunohistochemical stainer. RESULTS: Dendritic fibromyxolipoma usually occurrs in middle-aged to elderly men, developing primarily in the subcutis or muscular fascia of the head and neck region, shoulder, back, calf and foot. Grossly, it is well-circumscribed, partly thinly encapsulated lesion with focal mucinous or gelatinous cutting surface. Histologically, all these tumors showed similar histological features., and were characterized by an admixture of mature adipose tissue, spindle and stellate cells, and abundant myxoid stroma with prominent collagenization. The proportion of above elements varied in different tumors or in different areas within the same tumor. One of the pathologic characteristics was the proliferation of spindle cells and stellate cells showing thin cytoplasmic dendritic prolongations. No cytological atypia or mitotic activity could be identified. Furthermore, the lesions were well-vascularized and the vascular elements consisted of small to median-sized and capillary-sized plexiform vessels. Spindle cells were strongly positive for CD34, bcl-2 and vimentin but negative for S-100 protein and epithelial and muscle markers. CONCLUSIONS: Dendritic fibromyxolipoma is characterized by clinicopathologic and immunohistochemical features. Based on those features of this tumor, this entity appears to represent a transitional form between spindle cell lipoma and solitary fibrous tumor, and should be distinguished from myxiod liposarcoma and myxoid malignant fibrous histiocytoma. Due to the benign nature of this lesion, simple local excision is curative.

Aged↗

Ossifying fibromyxoid tumor of soft parts: a clinicopathologic study of 70 cases with emphasis on atypical and malignant variants.

The ossifying fibromyxoid tumor of soft parts (OFMT) is an uncommon soft tissue neoplasm of uncertain lineage. Approximately 100 cases of OFMT have been reported. Although the majority behave in a benign fashion, very rare tumors with histologic and clinical evidence of malignancy have been reported. Criteria for malignancy in OFMT have not as yet been defined. Seventy cases of OFMT were retrieved from our consultation archives and studied with respect to patient age and sex, tumor site and size, growth pattern, percentage of typical OFMT, cellularity and nuclear grade, mitotic figures (MF)/50 high power fields (HPF), atypical mitoses, necrosis, vascular invasion, and the presence of bone. Immunohistochemistry for pancytokeratin, S-100 protein, smooth muscle actin, desmin, and collagen II was performed on a subset of cases. Follow-up information was obtained from the submitting pathologists and clinicians. The Fisher exact test was used for statistical analysis. Patients (39 male, 31 female) ranged in age from 14 to 83 years (median 49 years). The tumors occurred primarily as subcutaneous or deeply seated masses in the trunk and proximal extremities and ranged from 1 to 14 cm (median 4.0 cm). The percentage of typical OFMT present in each case ranged from 0% to 100% (median 70%), and bone was present in 44 cases (63%). Mitotic activity ranged from 0 to 40 MF/50 HPF (median 2 MF/50 HPF), necrosis was present in 12 cases (17%), and vascular invasion was seen in 8 cases (11%). High cellularity or high nuclear grade was seen in 14 and 13 cases, respectively. Immunohistochemical results were as follows: S-100 protein (33 of 55, 60%), pancytokeratin (5 of 48, 10%), smooth muscle actin (2 of 44, 5%), desmin (5 of 39, 13%), and collagen II (1 of 26, 4%). Follow-up (51 cases, mean 54 months, median 36 months, range 5-151 months) showed local recurrences in nine patients and metastases in eight patients. Currently, 41 patients are disease free, 6 are alive with disease, 4 are dead of disease, and 1 died of other causes. The presence of high cellularity (p = 0.002), high nuclear grade (p = 0.001), or >2 MF/50 HPF (p = 0.004) were significantly associated with the development of metastatic disease and local recurrence. Infiltrative growth was also associated with increased risk of local recurrence (p = 0.03). We conclude that the histologic spectrum of OFMT is broader than previously appreciated, as many clinically benign cases display moderate cellularity, nuclear enlargement, or have identifiable mitotic figures. Our results strongly suggest that OFMT with 1) high nuclear grade or 2) high cellularity and mitotic activity of >2 MF/50 HPF should be regarded as sarcomas with significant potential for metastasis and untoward outcome ("malignant OFMT"). The remainder can be considered within the spectrum of OFMT, recognizing that even these lesions possess a risk, albeit very low, of metastasis. Consequently, OFMT should be considered tumors of intermediate malignancy. Their line of differentiation remains unclear, although we suggest they may belong to the category of translocation-associated sarcomas, not all of which recapitulate a normal line of differentiation.

Adolescent↗

Molecular genetic characterization of the EWS/CHN and RBP56/CHN fusion genes in extraskeletal myxoid chondrosarcoma.

Extraskeletal myxoid chondrosarcoma (EMC) is a soft-tissue neoplasm cytogenetically characterized by the translocations t(9;22)(q22;q11-12) or t(9;17)(q22;q11), generating EWS/CHN or RBP56/CHN fusion genes, respectively. In the present study, 18 EMCs were studied both cytogenetically and at the molecular level. Chromosomal aberrations were detected in 16 samples: 13 with involvement of 9q22 and 22q11-12, and three with rearrangements of 9q22 and 17q11. Fifteen cases had an EWS/CHN fusion transcript and three had an RBP56/CHN transcript. The most frequent EWS/CHN transcript (type 1; 10 tumors), involved fusion of EWS exon 12 with CHN exon 3, and the second most common (type 5; two cases) was fusion of EWS exon 13 with CHN exon 3. In all tumors with RBP56/CHN fusion, exon 6 of RBP56 was fused to exon 3 of CHN. By genomic XL PCR and sequence analyses, the breakpoints from 14 cases were mapped in the EWS, RBP56, and CHN genes. In CHN, 12 breakpoints were found in intron 2 and only two in intron 1. In EWS, the breaks occurred in introns 7 (one break), 12 (eight breaks), and 13 (one break), and in RBP56 in intron 6. Repetitive elements such as Alu and LINE sequences seem to have limited, if any, importance in the genesis of EWS/CHN and RBP56/CHN chimeras. Furthermore, there were no chi, chi-like, topoisomerase II, or translin consensus sequences in the introns harboring the translocation breakpoints, nor could the number of topo I sites in EWS, RBP56, and CHN introns explain the uneven distribution of the breakpoints among EWS or CHN introns. Additional genetic events, such as nucleotide insertions, homologies at the junction, deletions, duplications, and inversions, were found to accompany the translocations, indicating that the chromosomal translocations do not require sequence-specific recombinases or extensive homology between the recombined sequences.

Adult↗

Primitive neuroectodermal tumor of the kidney--another enigma: a pathologic, immunohistochemical, and molecular diagnostic study.

Primitive neuroectodermal tumor (PNET), the second most common type of sarcoma in the first two decades of life, rarely presents as an organ-based neoplasm. Rather, it is seen typically in the soft tissues of the chest wall and paraspinal region. We report a case of primary PNET of the kidney in a 17-year-old girl who presented with abdominal pain, hematuria, and an abdominal mass. Nodules and sheets of monotonous-appearing primitive round cells and the formation of rosettes focally were the principal microscopic features. The tumor cells were uniformly immunoreactive for vimentin, cytokeratin, neuron-specific enolase, and 013 (CD99). In addition, the characteristic translocation of PNET and Ewing sarcoma, t(11;22)(q24;q12), was detected by polymerase chain reaction (PCR). Eight previous examples of renal PNET have been reported in the literature in the past 2 years, but only three of these cases have had complete immunohistochemical evaluation with the demonstration of 013 positivity. To our knowledge the present case is the only one to date demonstrating the recurrent translocation t(11;22)(q24;q12) by PCR. Assuming that the previous cases in the literature are bona fide examples of PNET, the kidney may be another site of predilection for this usual soft-tissue neoplasm. We are once again confronted with the dilemma about the nature of the progenitor cell.

12E7 Antigen↗

Chest wall tumors: radiologic findings and pathologic correlation: part 1. Benign tumors.

Benign chest wall tumors are uncommon lesions that originate from blood vessels, nerves, bone, cartilage, or fat. Chest radiography is an important technique for evaluation of such tumors, especially those that originate from bone, because it can depict mineralization and thus indicate the diagnosis. Computed tomography (CT) and magnetic resonance (MR) imaging are helpful in further delineating the location and extent of the tumor and in identifying tumor tissues and types. Although the radiologic manifestations of benign and malignant chest wall tumors frequently overlap, differences in characteristic location and appearance occasionally allow a differential diagnosis to be made with confidence. Such features include the presence of mature fat tissue with little or no septation (lipoma), the presence of phleboliths and characteristic vascular enhancement (cavernous hemangioma), evidence of neural origin combined with a targetlike appearance on MR images (neurofibroma), well-defined continuity of cortical and medullary bone with the site of origin (osteochondroma), or fusiform expansion and ground-glass matrix (fibrous dysplasia). Both aneurysmal bone cysts and giant cell tumors typically manifest as expansile osteolytic lesions and occasionally show fluid-fluid levels suggestive of diagnosis.

Adult↗

Undifferentiated primary malignant soft tissue tumor.

A rare case of a malignant, undifferentiated, soft tissue neoplasm is presented. A thorough podiatric and medical evaluation is essential in the proper diagnosis and treatment of soft tissue tumors. This evaluation includes appropriate consultation with the various medical specialists.

Algorithms↗

[Cryotherapy of benign neoplasms of the soft tissues in the maxillofacial area].

In the light of the relevant literature and of personal studies, the author deals with the possibilities of treating benign neoformations in the maxillofacial region by cryotherapy. The results are discussed from the aspects of cryophysical and cryobiological prerequisites. The extension in depth of the changes is the decisive criterion for cold application.

Cryosurgery↗

Tissue response to mechanical vibrations for "sonoelasticity imaging".

The goal of "sonoelasticity imaging" is to differentiate between normal soft tissues and hard lesions. This is done by measuring and then displaying the ultrasound Doppler spectrum of regions within tissues which are mechanically forced with low frequency (20-1000 Hz) vibrations. The resolution and sensitivity of the technique ultimately rest on the spatial resolution of ultrasound Doppler detection, the low frequency mechanical properties of tissues, and the vibration response of layered, inhomogeneous regions with hard tumor inclusions and complicated boundary conditions set by the presence of skin, bones and other regions. An initial investigation has measured some tissue stiffness parameters, and applied these in a NASTRAN finite element analysis to simulate a prostate tumor in the pelvic cavity. The measurements show a wide separation between the elastic modulus of tumors and soft tissues such as muscle and prostate. NASTRAN analyses show the ability to delineate regions of different elasticity based on the pattern of vibration amplitudes. The ability to change vibration frequency within the 100-300 Hz band seems particularly helpful in simulations and experiments which visualize small stiff inclusions in tissues. Preliminary results support the postulate that sonoelasticity imaging can provide useful information concerning tissue properties that are not otherwise obtainable.

Computer Simulation↗

Granular cell tumor treated with Mohs micrographic surgery: report of a case and review of the literature.

BACKGROUND: Granular cell tumors are uncommon soft tissue neoplasms of neural origin that most often arise in the oral cavity. Penile lesions are distinctly uncommon. Mohs micrographic surgery (MMS) is a highly effective treatment for several cutaneous neoplasms when tissue conservation is crucial. OBJECTIVE: To examine the use of MMS for this soft tissue malignancy. METHODS: We report only the eighth granular cell tumor of the penis. The literature was reviewed regarding the use of MMS for these neoplasms. RESULTS: MMS was performed to minimize the destruction of normal tissue in treating this patient's granular cell tumor. Only one previous article documented the use of MMS for this soft tissue tumor. CONCLUSION: Although utilized infrequently to treat granular cell tumors, MMS may prove beneficial when lesion location or size render tissue conservation or assuredness of cure paramount.

Granular Cell Tumor↗

Hydatid disease of the soft tissues of the lower limb: findings in three cases.

Three cases of hydatid disease are reported, all presenting as soft tissue lesions in the lower extremities. All three cases were studied with ultrasound (US), two with computed tomography (CT), and two with magnetic resonance (MR) imaging techniques. Two patients presented with multivesicular lesions which were considered diagnostic of hydatid disease. The third patient showed a lesion with a predominantly solid pattern, closely mimicking a soft-tissue neoplasm. US was not diagnostic, but MR outlined vesicular structures and a fibrous pericyst. Hydatid disease presenting in the soft tissues can therefore be diagnosed with confidence when it shows multivesicular lesions but MR may be the most useful imaging technique when a complex or solid pattern is present.

Adult↗

Imaging of tumours of the foot and ankle.

The general presentation of osseous and soft-tissue neoplasms of the foot and ankle is not different from lesions encountered in other parts of the body and imaging features may be nonspecific. Some lesions, however, have a predilection for the foot and ankle and may have typical imaging features, such as plantar fibromatosis or intraosseous lipoma. This presentation aims to give an overview of bone and soft-tissue tumours of the foot and ankle and to discuss the strength of each imaging modality in the diagnosis.

Ankle↗

[Benign neoplasms of the soft tissues of the oral cavity].

The Authors, starting from a statistical study of Gennari et al., point out the frequency of the benign neoformations in the soft tissue of the oral cavity. They describe then the most frequently findable histological forms and the problems posed to the clinic and the surgeon by some histological forms such as the mixed tumours of the minor salivary glands and the fibropapillomatosis of the vestibular fornices. The Authors conclude stating that they not always are harmless neoformations and easily surgically excisable.

Fibroma↗

Carcinosarcoma and sarcomatoid carcinoma of the bladder: clinicopathological study of 41 cases.

PURPOSE: Carcinosarcoma of the bladder is a rare neoplasm characterized by an intimate admixture of carcinoma and malignant soft tissue neoplasm. The clinical usefulness of separating carcinosarcoma (carcinoma with sarcomatous component) from sarcomatoid carcinoma (carcinoma with spindle cell carcinomatous component) is uncertain, and it comprises the subject of this report. MATERIALS AND METHODS: We reviewed the clinical and pathological records of 10 men and 5 women a mean of 66 years old with carcinosarcoma, and 21 men and 5 women a mean of 66.5 years old with sarcomatoid carcinoma of the bladder, as documented in the files of the Mayo Clinic between 1936 and 1995. RESULTS: Of the 15 patients in the carcinosarcoma group 9 had urothelial carcinoma, small cell carcinoma, 3 had squamous cell carcinoma and 2 had more than 1 type. The sarcomatous component included chondrosarcoma in 3 cases, leiomyosarcoma in 3, malignant fibrous histiocytoma in 3, osteosarcoma in 2, fibrosarcoma in 1, rhabdomyosarcoma in 1 and more than 1 type in 2. All disease was high stage at presentation. Treatment included cystectomy in 11 patients with (4) and without (7) radiation therapy, and transurethral resection in 4 with (1) and without (3) radiation therapy. Mean followup available in 14 cases was 34 months (range 1 to 144). A total of 11 patients died of cancer at 1 to 48 months (mean 17.2) and 2 survived for 8 to 131 months. Of the 26 patients in the sarcomatoid carcinoma group 18 had urothelial carcinoma, 1 had squamous carcinoma, 2 had urothelial carcinoma combined with squamous cell carcinoma and 5 had spindle cells only with no recognizable epithelium. All but 1 case was high stage at diagnosis. Treatment included transurethral resection in 17 patients with (7) and without (10) radiation therapy, including 1 who also received chemotherapy, and only cystectomy in 5, including 2 who also underwent radiation therapy and 1 who also received chemotherapy. Mean followup available in 21 cases was 49 months (range 1 to 420). A total of 17 patients died of cancer at 1 to 73 months (mean 9.8), 1 was alive at 140 months and 3 died of unrelated causes. CONCLUSIONS: Carcinosarcoma and sarcomatoid carcinoma of the bladder are highly aggressive malignancies with a similar outcome regardless of histological findings and treatment. Pathological stage is the best predictor of survival.

Aged↗

Chest wall tumors: radiologic findings and pathologic correlation: part 2. Malignant tumors.

Malignant chest wall tumors are classified into eight main diagnostic categories: muscular, vascular, fibrous and fibrohistiocytic, peripheral nerve, osseous and cartilaginous, adipose, hematologic, and cutaneous. However, there are malignant tumors that arise in the chest wall and that do not fit well in any of these categories (eg, Ewing sarcoma and synovial sarcoma). Malignant chest wall tumors typically manifest as painful, rapidly growing, large palpable masses. Chest radiography, the technique most often used for initial evaluation, can be helpful for detecting cortical destruction. However, computed tomography is more sensitive than chest radiography for detecting calcified tumor matrix and cortical destruction. Magnetic resonance imaging often allows more accurate delineation and localization of the tumor and is helpful for determining the presence and extent of tumor invasion and for tissue characterization. Although the imaging features of many malignant chest wall tumors are nonspecific, knowledge of the typical radiologic manifestations of these tumors often enables their differentiation from benign chest wall tumors and occasionally allows a specific diagnosis to be suggested. The article reviews the clinical and imaging features of the most common malignant chest wall tumors and presents images collected at a single cancer referral center.

Adult↗