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[The radiographic report of soft tissue tumor on the legs in a case of cutaneous polyarteritis nodosa].

A 55-year-old male was admitted to our hospital because of arthralgia, rash, and painful tumor in the legs. On admission, skin lesions involving dark erythematous macules and a tender mass of 3 by 5 cm were present. Laboratory findings included hemoglobin of 11.2 g/dl, white blood cell count of 6200/microliter, erythrocyte sedimentation rate (ESR) of 88 mm/hour, and normal results of renal function tests. Hepatitis B surface antigen, anti-nuclear antibody (ANA), and perinuclear pattern antineutrophil cytoplasmic autoantibody (ANCA) were negative. A T 1-weighted magnetic resonance image (MRI) showed iso-intensity areas that increased in intensity on T 2-weighted images of the M. gastrocnemius. The angiographic appearance of tumor staining with some degree of luminal irregularity in the posterior tibial artery suggested a diagnosis of soft tissue neoplasm. Biopsy specimens of the right leg lesion were consistent with necrotizing arteritis of both small and medium-sized vessels. Treatment with prednisolone and cyclophosphamide resulted in the disappearance of the skin lesions and the improvement of laboratory data. At first, classical polyarteritis nodosa was considered histologically. However, lesions were limited to skin, muscles, and joints, and there has been no evidence of systemic disease for 1.5 years; consequently, cutaneous form of polyarteritis nodosa was diagnosed. Reports of soft tissue tumors on the legs of patients with polyarteritis nodosa are quite rare. The interesting radiographic findings of cutaneous polyarteritis nodosa were reported.

Cyclophosphamide↗

Gouty tophus presenting as a soft-tissue mass diagnosed by fine-needle aspiration: a case report.

We present a case in which a primary diagnosis of gout was made by fine-needle aspiration. The patient had a right distal ulnar mass, and the initial clinical and radiological diagnoses were that of giant cell tumor of tendon sheath. While tophi are the hallmark of gout, they rarely undergo aspiration because of the known clinical history. This case demonstrates that a tophus may mimic a soft-tissue neoplasm. Whenever an aspiration yields amorphous or granular material, the cytopathologist should be aware of and consider gouty tophus as a diagnostic possibility and perform compensated polarized microscopy on the specimen smears.

Aged↗

Elastofibroma: a rare soft tissue tumour with a pathognomonic anatomical location and clinical symptom.

BACKGROUND: Elastofibromas are rare benign soft tissue tumours that are almost always located at the inferior pole of the scapula, deep to the serratus anterior muscle. Their anatomical location and a distinctive clinical symptom distinguish them from malignant soft tissue tumours. PATIENTS AND METHODS: A prospective histo-pathological database of all soft tissue tumours referred for treatment to the Royal Marsden Hospital between 1995 and 2003 was searched. Seven patients treated in this institution with a histological diagnosis of elastofibroma were identified. RESULTS: The usual presenting symptoms were that of a mass at the angle of the scapula (7/7), and clunking of the scapula on shoulder abduction (5/7). Pain was an infrequent symptom (1/7). Imaging by CT or MR usually indicated an indeterminate soft tissue neoplasm deep to the peri-scapular muscles, but the histological diagnosis was confirmed pre-operatively by core biopsy in all patients. Subsequent surgical excision was only performed if symptoms were severe. CONCLUSIONS: The clinical presentation of these benign neoplasms is typical and the suspected clinical diagnosis can be confirmed easily by core biopsy. Surgery can be safely reserved for symptomatic patients.

Aged↗

[Intramuscular myxoma. Apropos of a case and review of the literature].

Having observed one case of intramuscular myxoma, the authors made a review of the literature of this rare localization. This benign soft tissue neoplasm, is well characterized by its usual latency. Physical examination is not very specific and must be completed by computed tomography scan. The surgical treatment realizes a wide excision with clear surgical margins. Diagnosis is based on histological examination. Neither metastatic spread, nor recurrence is observed.

Female↗

DNA content parameters of paraffin-embedded soft tissue sarcomas: optimization of retrieval technique and comparison to fresh tissue.

DNA content analysis of formalin fixed paraffin embedded (FFPE) tissue permits determination of the influence of DNA content on the prognosis in cohorts of patients for whom the clinical outcome is known. Of key importance in such an analysis is the accuracy of DNA content determination. Variations in the quality of DNA histograms from FFPE tissues of different types prompted a comparative evaluation of the preparative methodology of FFPE soft tissue sarcomas for DNA flow cytometry. Following deparaffination and rehydration of fixed tissue, and prior to fluorochrome staining, tissue blocks of 15 DNA aneuploid soft tissue sarcomas were subjected to repeated experimental (time x concentration) enzyme exposures. The goal of these studies was to define the optimal tissue specific retrieval technique with the coefficient of variation, maintenance of DNA aneuploidy, and DNA index as endpoints. After optimizing the technique, the DNA content of 50 soft tissue neoplasms derived from FFPE specimens was compared to the corresponding fresh surgical tissue. The observed 14 percent error rate in the determination of DNA ploidy status suggest limited utility for FFPE tissue in prospective therapeutic trials of soft tissue sarcoma.

Cell Count↗

An unusual presentation of extramedullary plasmacytoma occurring sequentially in the testis, subcutaneous tissue, and heart.

Extramedullary plasmacytoma (EMP) is a rare neoplasm of soft tissue that usually arises in the respiratory tract, nasal cavity, sinuses, and nasopharynx. It is even more uncommon for it to arise either in the testis or heart. We report the presentation of a case where plasmacytomas were found sequentially in the testis, subcutaneous tissue, and heart. EMP usually has a good prognosis except when it involves the heart. Our patient survived for only 15 months post autologous hematopoietic stem cell transplantation.

Antineoplastic Combined Chemotherapy Protocols↗

Extensively calcified synovial sarcoma.

Synovial sarcoma is an uncommon fibroblastic soft tissue neoplasm, commonly arising near, but not necessarily from, the synovium of joint capsules, bursae or tendon sheaths. The radiological diagnosis is difficult. We present a case of synovial sarcoma studied with plain film radiography, CT, and pathology that had an unusual extensive calcification, which complicated its radiological diagnosis.

Adult↗

Myxoid monophasic synovial sarcoma: case report of an unusual histological variant.

We report the case of a 61-year-old woman who presented a recurrent myxoid synovial sarcoma involving the right ankle and foot. This tumor, defined as a synovial sarcoma showing more than 50% myxoid change in the stroma, has only recently been described as a rare histological variant. The histological diagnosis is particularly difficult in such cases, as the one we are describing, where the tumor is entirely myxoid and monophasic raising the possibility of other myxoid soft tissue neoplasms, such as extraskeletal myxoid chondrosarcoma, malignant peripheral nerve sheath tumor or leiomyosarcoma. On the basis of morphological and immunophenotypical findings, the diagnosis of myxoid synovial sarcoma should be properly established, especially in view of its unusual clinical course and treatment. Furthermore, we will discuss the clinicopathological and immunohistochemical features observed in our case.

Female↗

Pleomorphic hyalinizing angiectatic tumor of soft parts: case report and literature review.

Pleomorphic hyalinizing angiectatic tumor (PHAT) of soft parts is a rare soft tissue neoplasm. Few cases have been reported in the literature thus far. PHAT is a spindle cell neoplasm with a prominent vascular component which displays some features of both neurilemoma and malignant fibrous histiocytoma. Lesions typically occur on the lower extremities of adults, however, lesions in other anatomic locations have been described. In this paper, we report a 60 years old female with PHAT of the right foot and review the current literature. El-Tal A-E-K, Mehregan D. Pleomorphic hyalinizing angiectatic tumor of soft parts: case report and literature review.

Diagnosis, Differential↗

Imaging of atypical lipomas of the extremities: report of three cases.

Atypical lipomas are soft tissue neoplasms which differ from simple lipomas in that they consist of mature fat cells interspersed with occasional areas containing a variable admixture of multinucleated cells, collagen bundles, and adipocytes with large, hyperchromic nuclei. Although these histopathologic features resemble those of liposarcoma, atypical lipomas occurring in the extremities differ from liposarcoma because they have no tendency for distant metastases. Three patients with atypical lipomas involving the posterior compartment of the thigh are presented. The lipomas were imaged with computed tomography (CT) and magnetic resonance imaging (MRI), using a 0.15-T whole body imager. Although all lesions appeared largely lipomatous, the three lesions simulated liposarcoma on both CT and MRI. On CT, each lesion demonstrated small areas of confluent soft tissue density; these areas were hyperintense with adipose tissue on strongly T2-weighted MRI. We conclude that atypical lipoma of the extremities may not be distinguishable from liposarcoma on imaging and that biopsy is necessary for differentiation.

Adult↗

Fine needle aspiration biopsy of soft tissue sarcomas: utility and diagnostic challenges.

The role of fine needle aspiration biopsy (FNAB) as the primary modality for the initial diagnosis of previously undiagnosed soft tissue sarcomas presents several important challenges. Most practicing pathologists are inexperienced with the wide array of soft tissue neoplasms and their morphologic heterogeneity, making them susceptible to misdiagnosis. However, in the hands of experienced cytopathologists, FNAB in conjunction with ancillary techniques has a diagnostic accuracy approaching 95% for the diagnosis of malignancy. FNAB has been shown to have a diagnostic yield nearly identical with core needle biopsy while avoiding significant clinical complications. Nevertheless, FNAB has certain limitations related to the accurate histologic grading and subtyping of certain subgroups of sarcomas. It may also be difficult to accurately distinguish between low-grade sarcomas and benign or borderline cellular lesions, especially in the spindle cell sarcoma subgroup. The aim of this review is to highlight the utility and limitations of FNAB in the primary diagnosis of soft tissue sarcomas, highlight diagnostically challenging lesions, and comment on the limitations of FNAB in providing a "definitive" diagnosis.

Biopsy, Fine-Needle↗

[Problems in the electron microscopic diagnosis of neurosarcomas].

Electron microscopical analysis of 113 cases of neurogenic tumours of the human soft tissues is presented. Ultrastructural signs of neurogenic differentiation are distinguished; neuronal and those of nerve sheaths. The comparison of the ultramorphology of neurosarcomas with that of carcinoids and granular cell tumours is performed. A considerable increase of the proportion of neurogenic tumours among soft tissue neoplasms is noted after the ultrastructural investigation. Differential diagnosis of neurosarcomas and malignant histiocytomas and other tumours at the electron microscopic level is discussed.

Diagnosis, Differential↗

MR imaging techniques for soft-tissue lesions.

MR imaging is the technique of choice for detection, characterization, and staging of soft-tissue neoplasms. MR examinations need to be tailored to each patient based upon the patient's size and anatomic region of interest so that optimal information is obtained. Technique is critical. Proper positioning to allow patient comfort and reduce motion artifacts is essential. Selection of the appropriate coils, image planes, and optimal pulse sequences to identify and determine the extent of lesions is critical for therapy planning.

Artifacts↗

Synovial sarcoma mimicking desmoplastic small round-cell tumor: critical role for molecular diagnosis.

BACKGROUND: The identification of recently described nonrandom chromosomal defects specific for various small round-cell and spindle-cell sarcomas can eliminate diagnostic uncertainty arising from the clinical and histopathologic overlap of soft tissue neoplasms. METHODS: A 26-year-old man presented with bulky abdominal-pelvic disease. Immunohistochemical and molecular studies on tumor were performed. Treatment was instituted using cycles of high-dose cyclophosphamide (4,200 mg/m2) with doxorubicin (75 mg/m2). RESULTS: Clinical findings pointed to desmoplastic small round-cell tumor. The tumor was histologically undifferentiated and immunoreactive for vimentin but negative for other markers. Reverse transcriptase-polymerase chain reaction revealed the SYT/SSX2 fusion transcript of the synovial sarcoma t(X;18) chromosomal rearrangement. The high-dose chemotherapy, plus surgery, achieved a complete remission, but recurrent disease emerged 13 months from diagnosis. CONCLUSIONS: This clinically unique case of synovial sarcoma highlights how the use of now readily available molecular techniques will allow more accurate appraisals of the incidence and anatomic distribution of soft tissue neoplasms-information that bears upon pathogenesis and treatment. This case confirms the utility of high-dose alkylator-based therapy for synovial sarcoma. It also demonstrates that with nonlocalized solid tumors, the eradication of minimal residual disease remains an elusive goal. One alternative involves immunologic attack against markers derived from tumor-specific chromosomal defects such as those found in our patient.

Abdominal Neoplasms↗

"Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series.

Eighteen examples of an unusual malignant soft-tissue neoplasm, the morphology of which ranged from that of "atypical" epithelioid sarcoma to that of a rhabdoid tumor or undifferentiated carcinoma (with transitional forms) are described. Patients included 11 males and seven females; their median age was 35.5 years with most patients aged 20 to 40 years. Development of a mass was the main presenting symptom. Six tumors developed in the pelvis and perineal region, four in the pubic region and vulva, three in the buttocks, one in the deep soft tissues of the left hip, one on the penis, one in left forearm, one in left axilla, and one on the occiput. Tumor size ranged from 1 to 20 cm (median, 4 cm). On microscopic examination, the tumor cells invaded the subcutaneous or deep soft tissues, had prominent epithelioid or rhabdoid features, had marked cytologic atypia, and grew in a multinodular pattern in half of the cases. Areas of necrosis were often seen. A granuloma-like pattern reminiscent of that observed in classic epithelioid sarcoma was observed in only two cases. Immunohistochemically, positivity for cytokeratin, epithelial membrane antigen, and vimentin was seen in all but one of the cases. Of 16 cases, 10 and eight tumors reacted with desmin and CD34, respectively; five of 15 reacted at least focally with smooth-muscle actin, whereas three of 13 and one of 10 reacted for HMB-45 and carcinoembryonic antigen, respectively. S-100 protein and CD31 yielded negative results. Seven tumors were investigated at the ultrastructural level, four of which showed prominent intracytoplasmic intermediate filament aggregates, often accumulating into paranuclear whorls, which is in keeping with the rhabdoid phenotype. Five tumors showed features of epithelial differentiation (i.e., tonofilament-like structures or desmosomes or both), whereas one tumor displayed features of myofibroblastic differentiation. Differential diagnoses include mainly conventional epithelioid sarcoma, extrarenal malignant rhabdoid tumor, epithelioid malignant peripheral nerve sheath tumor, melanoma, rhabdomyosarcoma, and undifferentiated carcinoma. Follow-up information on 14 patients (range, 4 months to 8 years; median, 19 months) revealed local recurrence in one case and metastatic dissemination in six patients, leading to death in five. In our opinion, the above-described neoplasms represent a usually "proximal-type" of epithelioid sarcoma. In contrast to the conventional, "distal-type" epithelioid sarcoma, the proximal variant is characterized by a predominantly large-cell, epithelioid cytomorphology, marked cytologic atypia, frequent occurrence of rhabdoid features, and lack of a granuloma-like pattern in most cases. It appears to be somewhat more aggressive (or at least metastasizes earlier) than usual epithelioid sarcoma.

Adolescent↗

[Primary neuroectodermal tumor (PNET): a rare highly malignant soft tissue tumor in children and young adults].

The PNET is an extremely malignant soft tissue neoplasm resulting from a balanced reciprocal translocation t (11; 22) (q24; q12). Treatment of the undifferentiated small cell tumor is carried out in compliance with the protocol of the soft tissue trail (CWS) from the German Society of Pediatric Oncology. Biopsy-proven diagnosis is followed by primary chemotherapy which leads in 95% of cases to remission. After excision of the remainder of the malignancy, an irradiation of the tumor site and two further sequences of chemotherapy are performed. Between 1986 and 1994, in cooperation with our pediatric and radiotherapeutical colleagues, we treated ten patients. In four patients (median age, 14 years) the PNET originated from the chest wall, in six patients from the paravertebral and retroperitoneal region. Five patients died after 20 months on average, while the remaining five patients are in full remission after 22, 37, 41, 42 and 82 months, respectively.

Adolescent↗

Parachordoma.

Parachordomas are rare soft-tissue neoplasms that are histologically similar to chordomas. They typically develop adjacent to tendon, synovium, or osseous structures within extremities and present as slowly growing, locally destructive, lobulated neoplasms that are prone to recurrence if incompletely excised. We report a parachordoma occurring in the right middle finger of a 25-year-old man. The neoplasm had been present for 5 years, and the patient had sustained repeated injuries to the area. Histologically, the lesion was characterized by well-circumscribed lobules composed of small cellular aggregates, some in "alveolar" distribution, and single, large, vacuolated (physalipherous) cells embedded within a hyalin and chondroid matrix divided by fibrous trabeculae. Immunohistochemically, the parachordoma stained positively for the S-100 protein, vimentin, and cytokeratin.

Adult↗