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Hibernomas are benign tumors derived from brown fat that most often present as painless, slowly enlarging masses in the interscapular region, the neck, the inguinal region and the mediastinum. We report one case of hibernoma of the axillary region and we review the clinical presentation, diagnostic and therapeutic of this kind of soft tissue neoplasm.
An extraskeletal osteosarcoma histologically mimicking parosteal osteosarcoma arising in a 63-year-old female is described. The intramuscular lesion in the left lower thigh was a 7 x 4 cm, well-defined mass with marked central calcification and ossification. The tumor was composed predominately of a centrally located osteocartilaginous component and a peripheral, non-osseous fibrous component. The former was characterized by a complex interanastomosing pattern of woven-bone trabeculae with small osteocytes and a minor cartilaginous element. The intratrabecular stroma consisted of benign-appearing fibroblasts. The peripheral fibrous part was characterized by fascicular and interlacing proliferations of spindle cells with mild atypia, rare mitosis and low to moderate cellularity. There were a few scattered foci of proliferating pleomorphic cells, constituting 5% of the tumor, indicating high-grade transformation. There was no evidence of zoning phenomena, cortical erosion, periosteal reaction or intramedullary involvement. The patient is well without recurrence or metastasis at 12 months follow up. This soft tissue neoplasm, mimicking parosteal osteosarcoma, should be distinguished from other bone-forming tumors or tumor-like lesions with which they may be confused. Pathologists and physicians should know the existence of this type of extraskeletal osteosarcoma and treat it as a high-grade extraskeletal osteosarcoma.
Bcl-2 is one of the many proteins that regulate programmed cell death and is overexpressed in B-cell lymphomas. The expression of bcl-2 in mesenchymal cells and soft tissue tumours was the subject of this study. Normal mesenchymal tissue and representative cases of soft tissue tumours of different types (n>200) were investigated immunohistochemically for bcl-2 expression. Although bcl-2 expression was normally relatively restricted to some smooth muscle cells and neural cells, bcl-2 immunoreactivity was widespread in different types of soft tissue neoplasms, both benign and malignant. Consistently positive tumours included solitary fibrous tumour, haemangiopericytoma, schwannoma and synovial sarcoma. The few soft tissue tumours that were consistently negative for bcl-2 included nodular fasciitis and desmoid tumour. Leiomyomas and leiomyosarcomas were heterogeneous; all uterine leiomyomas were bcl-2 positive, but all oesophageal leiomyomas were negative, paralleling the reactivity observed in the smooth muscle at those sites. Gastrointestinal stromal tumours showed bcl-2 reactivity; this was less consistent in malignant tumours. Along the malignancy gradient, there was no consistent trend in the bcl-2 reactivity. Dermatofibrosarcomas showed increase of bcl-2 expression with fibrosarcomatous transformation, whereas smooth muscle sarcomas and malignant peripheral nerve sheath sarcomas were less consistently positive than the corresponding benign neoplasms. We conclude that bcl-2 expression is widespread in soft tissue tumours, but shows constitutional expression patterns that are often parallel to the normal tissue counterparts. Compared with benign soft tissue tumours, bcl-2 expression is often reduced in sarcomas, but it cannot be used as a prognostic marker without correlation of the data to its phenotypic expression patterns.
We describe a case of a novel soft tissue neoplasm, composed of a monotonous population of cells. Their cytoplasm was parked with mitochondria, and had no immunohistochemical or ultrastructural evidence of differentiation. The neoplasm, located within the thigh of a 50-year old man, was well-circumscribed but unencapsulated. The patient was clinically free of neoplasm at 1 year follow-up, after complete local excision. We propose the term "soft tissue oncocytoma" for this lesion, because of the similarity of this neoplasm to oncocytomas of other reported sites.
The primitive neuroectodermal tumor (PNET) is an extremely aggressive soft tissue neoplasm that occurs in children and adolescents. We retrospectively reviewed our therapeutic experience with a multidisciplinary approach, combining surgery, chemotherapy, and radiation therapy. Treatment of PNET was carried out in compliance with the soft tissue protocol (CWS) from the German Society of Pediatric Oncology. Biopsy-proven diagnosis was followed by chemotherapy, which in all cases led to partial remission, allowing excision of the remainder of the tumor without mutilation. After excision, irradiation of the tumor site and two further sequences of chemotherapy were performed. When PNET of the paravertebral region caused symptoms of paralysis and immediate surgery was required, postoperative chemotherapy, a second-look operation, and irradiation were undertaken. Between 1986 and 1998 we treated 13 patients (median age 15 years). In five patients the PNET originated from the chest wall and in eight patients from the paravertebral and retroperitoneal region. Five patients died after 20 months on average, and the remaining eight patients are in full remission after 7, 16, 46, 55, 70, 74, 75, and 115 months, respectively. Close cooperation between surgeons and their pediatric and radiotherapy colleagues is obligatory when treating PNET. Chemotherapy as the first stage is mandatory to avoid a mutilating surgical procedure and intraoperative tumor cell dissemination.
Infiltrating angiolipomas are rare benign soft-tissue neoplasms that are locally aggressive and require wide surgical excision. Of 10 patients studied, five showed serpiginous densities intermixed with fat on plain films. Six radionuclide bone scans showed uptake by the soft-tissue lesion. Eight angiograms demonstrated poorly marginated, very hypervascular lesions. Computed tomography (CT) in five patients yielded excellent visualization of predominantly low density lesions that invaded and replaced skeletal muscle. The combination of angiography and CT indicated the specific diagnosis and demonstrated the size, extent, and vascularity of the tumors.
Cyclin D3 immunohistochemical expression was investigated in normal, reactive, and neoplastic human embryonal and adult tissues. In the fetus, cyclin D3 was expressed in selected developmental phases of a limited number of cell systems. In normal adult tissues, cyclin D3 showed two patterns of distribution: in lymphoid tissues it was expressed in proliferative compartments, while in most other tissues it was expressed by terminally differentiated/quiescent cells. This dual role in proliferation and differentiation was partially conserved in neoplasms. In non-Hodgkin lymphomas, cyclin D3 immunolabelling was correlated with proliferative activity and progression; a significant exception was seen in cyclin D1-positive mantle cell lymphomas, which were cyclin D-negative. Benign endocrine tumours were frequently strongly cyclin D3-positive, while high-grade (small cell) neuroendocrine carcinomas were always negative. In most other epithelial neoplasms, cyclin D3 immunostaining was heterogeneous. In breast carcinomas, no relationship was seen between ER status and MIB1 labelling; cyclins D3 and D1 were frequently expressed in the same tumour, while occasional tumours showed an inverse quantitative relationship between cyclins D1 and D3, and rare tumours were negative for both. In soft tissue neoplasms, cyclin D3 was consistently expressed in some tumours, such as stromal tumours of the gastrointestinal tract and embryonal rhabdomyosarcomas. Our data suggest that cyclin D3 has a dual role in proliferation and differentiation in normal tissues and in some neoplastic conditions; that the cyclin D3 expression pattern is different from cyclin D1, suggesting non-redundant functions; that cyclin D3 expression is strong in endocrine cells secreting steroid hormones, and in their neoplastic counterparts; and that cyclin D3 deregulation may be of pathogenetic relevance in lymphomagenesis and could be diagnostically useful.
BACKGROUND: Synovial sarcomas are malignant high-grade, soft-tissue neoplasms that account for 7% to 8% of all malignant soft-tissue tumors and are the most common nonrhabdomyosarcoma soft-tissue sarcomas in pediatric patients. STUDY DESIGN: A retrospective review of the records of children younger than 17 years with synovial sarcoma treated at the University of Texas MD Anderson Cancer Center from 1966 until 1999 was undertaken. Primary site, tumor size, tumor margins, surgical treatment, adjuvant therapy, local and distant recurrence, and survival were recorded for 42 patients. Overall survival (OS) and progression-free survival (PFS) rates were calculated by the Kaplan-Meier method. The PFS and OS comparisons were performed using the log-rank test. RESULTS: Forty-four patients were identified, but two patients were excluded because of incomplete records. The median followup duration for the 42 patients was 8.8 years (range 0.2 to 22.4 years). The 5-year progression-free survival and overall survival rates were 75.6% and 87.7%, respectively. Eleven patients were dead and four others had progressed but were alive without evidence of disease after further therapy. Intergroup Rhabdomyosarcoma Study (IRS) grouping and tumor invasiveness were found to be significant prognostic indicators (p < 0.01 and p = 0.02, respectively). Patients with initial gross total resection (IRS Groups I and II) and noninvasive tumors (T1) were most likely to have prolonged PFS and OS. Patients with small tumors (<5 cm) (p = 0.09) had better PFS and OS. Adjuvant radiation therapy appeared to be of benefit, and chemotherapy did not seem to impact PFS or OS. Tumors > or = 5 cm are associated with increased risk of local recurrence and distant metastases. CONCLUSIONS: Complete resection with clear, yet not necessarily large, margins remains the treatment of choice for synovial sarcoma in children. Adjuvant radiation therapy should possibly be considered in patients with clear margins (IRS Group I) and in patients with microscopic residual tumor (IRS Group II). Chemotherapy did not seem to impact PFS or OS. Lymph nodes should be evaluated for local regional disease.
Fine needle aspiration specimens from 29 patients with bone and soft tissue neoplasms were analyzed by flow cytometry for DNA index and cell cycle analysis to determine whether such studies were helpful in cytologic diagnosis. Of 15 cases initially cytologically diagnosed as benign, 14 had a DNA index of 1.0, indicating a diploid population. The remaining case diagnosed as cytologically benign had a DNA index of 1.3. Further tissue from this tumor revealed an osteogenic sarcoma. Of the 14 cases initially diagnosed as malignant, 12 were hyperdiploid. Cell cycle analysis showed that malignant tumors had a higher proportion of cells in S phase (15.2% +/- 8.7%) than benign tumors (6.9% +/- 1.6%). Furthermore, high-grade malignancies had a significantly greater number of cells in S phase (18.5% +/- 1.5%) than low-grade tumors (9.9% +/- 6.3%).
Tumours of soft tissue may manifest cartilaginous differentiation as a primary phenomenon (as in chondromas or extraskeletal myxoid and mesenchymal chondrosarcomas) or as a secondary metaplastic feature (most notably in extraskeletal osteosarcomas or malignant nerve sheath tumours). The literature regarding primary cartilaginous tumours is reviewed and their differential diagnosis discussed. Soft tissue chondromas are noteworthy for their tendency to show significant nuclear pleomorphism. Extraskeletal chondrosarcoma is much rarer than its osseous counterpart, accounting for only 1-2% of soft tissue sarcomas. Virtually all such sarcomas can be classified into the myxoid or mesenchymal subtypes, of which myxoid carries a better prognosis. Soft tissue neoplasms which may show focal cartilaginous metaplasia are covered more briefly.
OBJECTIVE: The purpose of this study is to delineate the magnetic resonance (MR) appearance of a granular cell tumor (GrCT) of the extremity and to correlate the imaging appearance with the microscopic findings. DESIGN AND PATIENTS: A retrospective review of five patients with a histopathologic diagnosis of GrCT and pre-operative MR imaging of the neoplasm was done. The images were reviewed by two musculoskeletal radiologists in a consensus fashion. Lesion location, size, shape, margination, and signal intensity characteristics were assessed. MR findings were correlated with histopathological examination. RESULTS: The benign subtype of GrCT is usually isointense or brighter than muscle on T1-weighted sequences, round or oval in shape, superficial in location, and 4 cm or less in size. On T2-weighted sequences, benign lesions may demonstrate a high peripheral signal, as well as a central signal intensity that is isointense to muscle or suppressed fat. A significant stromal component in the tumor and, hypothetically, a ribbon-like arrangement of tumor cells may influence the signal intensity demonstrated on the T1 and T2-weighted sequences. The malignant subtype may demonstrate signal intensity characteristics and invasion of adjacent structures often seen with other aggressive neoplasms; sizes larger than 4 cm and association with major nerve trunks can be seen. CONCLUSION: Benign GrCT has imaging characteristics which may distinguish this tumor from other soft tissue neoplasms, as well as the malignant type of this tumor.
OBJECTIVE: To determine if the MR features of elastofibroma are sufficient for diagnosis of this neoplasm. MATERIALS AND METHODS: The MR studies of two patients with pathologically proven bilateral elastofibromas were reviewed retrospectively. RESULTS: In each patient, bilateral semilunar soft-tissue masses demonstrating signal intensities isointense to that of muscle were identified in the periscapular regions deep to the posterolateral musculature of the chest. Focal linear areas of fat were present. The biopsy specimens of both patients demonstrated a positive reaction to the elastin stains. CONCLUSION: A periscapular soft-tissue neoplasm demonstrating a pattern of linear alternating regions of high and intermediate signal intensity on T1- and T2-weighted spin-echo MRI should be sufficient to allow the diagnosis of elastofibroma.
In this article, the numerous proven applications of diagnostic ultrasound to the assessment of disease processes affecting the popliteal fossa and lower extremities have been discussed. Popliteal cysts and aneurysms, hematomas, cellulitis, abscesses, soft tissue neoplasms, and other space-occupying fluid collections are readily identified using this technique. In addition, post-traumatic and inflammatory disorders affecting tendons, synovial thickening and effusion, derangement of articular cartilage, meniscal tears, and alterations in soft tissue thickness as well as foreign bodies are amenable to detection by sonography. With future technologic improvements in transducer design and computerized image reconstruction, diagnostic capabilities of this method in the lower extremities should continue to expand.
Histological classification of soft tissue sarcomas can be made by light microscopy alone for those tumors with unique appearances, while a significant proportion of sarcomas require the implementation of electron microscopy or immunohistochemistry to obtain a final diagnostic classification. Immunostaining methods are particularly valuable in certain classes of sarcomas such as epithelioid sarcomas, malignant schwannomas, hemangiosarcomas, clear cell sarcomas, rhabdomyosarcomas, pleomorphic liposarcomas, leiomyosarcomas and synovial sarcomas through documentation of their cellular products. Immunohistochemistry is not a substitute for skilled interpretation of conventionally stained microscopic specimens; however, the availability of more specific determinants in the soft tissue neoplasms will expand the value of this approach in this area of diagnostic pathology.
Radiographic examination of soft tissue extremity masses is frequently inconclusive. In 18 patients with normal or nonspecifically abnormal radiographs, gray scale ultrasonography provided useful additional information. It was possible to distinguish fluid collections from solid masses, and recurrent venous thrombosis from hematoma in anticoagulated patients. Occasionally, specific diagnoses were suggested on the basis of ultrasonic morphologic characteristics. Diagnoses included soft tissue neoplasms, hematomas, aneurysms, synovial cysts, abscesses, and a lymphocele. Ultrasonically guided percutaneous needle aspiration was diagnostic to two cases. Features of differential diagnostic value relative to extremity solid masses and fluid collections are discussed. Ultrasonography is useful in evaluating these soft tissue masses.
Soft tissue tumors represent a frequent source of diagnostic difficulty for surgical pathologists. Over the past 20 years, immunohistochemical studies have emerged as a powerful and helpful tool for the assessment and characterization of these lesions. Although much progress has been made in this field, the continuous realization of the relative lack of specificity and broad overlap in reactivity for the various tumor markers among these lesions has contributed to limit somewhat their usefulness and reliability in this area of tumor diagnosis. However, with the advent of new markers has come the new promise of better and more accurate distinction and characterization for these tumors. Herein we will review the current status of immunohistochemical tumor markers in the diagnosis of soft tissue neoplasms and address some of the more recently described antibodies and their relative value and limitations for the diagnosis of these tumors.
OBJECTIVE: We report a rare case of chronic, neglected rupture of the distal biceps brachii which presented with gradually increasing swelling over the left lower arm. Fine-needle aspiration cytology was reported as indicative of a soft-tissue neoplasm. Computed tomography and magnetic resonance imaging were unable to rule out a neoplastic mass. Final histopathology after surgical excision revealed a reparative process. CONCLUSION: Such a presentation of ruptured biceps brachii wherein the clinicoradiological features are misleading and suggestive of a soft-tissue tumor is quite unusual.
Our objective was to describe the imaging features of lymphadenopathy at the medial epitrochlear region as frequently seen in cat-scratch disease (CSD) and to compare these features with those seen in non-CSD. Ten patients (four males and six females) presented with a subcutaneous soft tissue mass at the medial epitrochlear region. Three patients were younger than 15 years. Magnetic resonance imaging was performed in all patients. All lesions turned out to be regional lymphadenopathy instead of soft tissue neoplasm. They were caused by benign disorders in eight cases, including seven CSD and one other infection, and in two cases by metastatic disease. All lesions, except for three benign ones, were solitary. On MRI, high signal intensity (SI) strands in the surrounding soft tissue on T2-weighted images (WI) were present in all cases. Three cases of CSD showed homogeneous SI on both T1- and T2WI. After contrast administration, a moderate, homogeneous enhancement was seen. The other seven lesions, except for one metastasis, had both solid and liquid components. Liquid components showed high SI on T2WI without enhancement, whereas solid components had intermediate SI on T2WI and marked enhancement after contrast administration. Pathological findings in seven patients, including four with CSD, supported the tentative diagnosis made on MRI. One of two patients with CSD had positive serological test. Cat-scratch disease is a major cause of a soft tissue swelling at the medial epitrochlear region. Identification of lymphadenopathy and its presentation on MRI may be helpful in eliciting an animal-scratch history and achieving diagnostic and therapeutic aims in patients without primary cancer.