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Dermatofibrosarcoma protuberans: radiologic appearance.

OBJECTIVE: Dermatofibrosarcoma protuberans is a distinct clinicopathologic entity characterized by fibroblasts with a prominent storiform pattern. It accounts for about 6% of all soft-tissue sarcomas. The lesion is typically superficial, and the diagnosis can be suspected on the basis of the tumor's clinical appearance. Consequently, the imaging appearance of this entity is essentially unreported. Large lesions, however, can infiltrate the deep soft tissue and be confused with higher-grade sarcomas. We report the radiologic appearance in 11 cases of histologically proved dermatofibrosarcoma protuberans. MATERIALS AND METHODS: The radiologic images of 11 patients who had histologically verified dermatofibrosarcoma protuberans were retrospectively studied. The patients included eight males and three females 9-47 years old (mean, 33 years). Each had a soft-tissue mass that had been present for a variable period, from 3 months to 23 years. CT scans were available for review in six cases, MR images in four, radiographs in nine, bone scintigrams in three, and arteriograms in three. RESULTS: All lesions were unmineralized nodular masses involving the skin and subcutaneous adipose tissue. The size, in greatest dimension on imaging studies, was 3-22 cm. CT scans (six cases) showed a well-defined lesion with a distinct lobular or nodular architecture and tissue attenuation approximately equal to or greater than that of skeletal muscle. Moderate enhancement was seen on CT scans obtained after injection of contrast material. Arteriograms (three cases) showed mild to moderate hypervascularity. The MR appearance (four cases) was nonspecific; the lesions had prolonged T1 and T2 relaxation times. In one case, MR images showed multiple nodular lesions. Radiographs (nine cases) showed a soft-tissue mass or soft-tissue swelling without evidence of bone involvement or calcification. Bone scintigrams (three cases) showed increased accumulation of tracer. CONCLUSION: The radiologic appearance of dermatofibrosarcoma protuberans is typically that of an unmineralized, nodular soft-tissue mass involving the skin and subcutaneous adipose tissue. CT scans or MR images are well suited to show this location, the relation of the lesion to underlying structures, and the distinct lobular or nodular architecture. Arteriograms will show mild to moderate hypervascularity and bone scintigrams will show increased accumulation of radiopharmaceutical.

Adipose Tissue↗

[New data on the histogenesis of connective tissue tumors].

The normal skin and adipose subcutaneous tissue, desmoid fibroma, lipomas with varying proliferative activity, fibrosarcoma, malignant fibrous histiocytoma, epithelioid leiomyoma are studied by means of electron microscopy and electron microscopical radioautography. It was found that the walls of the smallest vessels contain cells which are the source of a permanent physiological renewal of a different type of the connective tissue. In proportion to differentiation and gradual removal of these cells from the vascular wall their proliferative and metabolic activity is more and more decreasing. Thus, the vessels represent not only the transport-nutritive system but the central generative structure of the connective tissue as well. Pluripotential mesenchymal cell of the vascular wall which under physiological conditions is differentiating in the direction of fibro-, angio-, lipo- or osteogenesis, due to the influence of oncogenic factors gives rise to the development of benign and malignant tumours of fibrous, vascular (angiomas), smooth muscle, adipose and bone structure. This can be most demonstratively checked using electron-radioautography of tumourlike conditions and relatively slow growing tumours.

Adipose Tissue↗

Myxoid lipoma of oral soft tissue. A clinical and ultrastructural study.

Myxoid lipoma is an unusual histologic type of lipoma. This article reports four cases of myxoid lipoma that arose in the mouth. In frozen sections stained with oil red O, the cytoplasm of tumor cells stained strongly positive. Intercellular mucoid material was PAS-positive and diastase-resistant. It also stained positively with alcian blue. Electron microscopic observations revealed that adipocytes were in different stages of differentiation, with lipid globules of variable size observed in the cytoplasm. Many pinocytotic vesicles appeared at the plasma membrane. Mature adipocytes were surrounded by a basal lamina. Fibroblasts, collagen fibers, and mucoid material were scattered among adipocytes. It appears possible that, in the histogenesis of myxoid lipoma, multipotent mesenchymal cells undergo multidirectional differentiation. Some cells differentiate to produce mucosubstance, while others differentiate to become adipocytes.

Adipose Tissue↗

A case of juvenile hyaline fibromatosis.

Juvenile hyaline fibromatosis (JHF) is a rare, autosomally-recessive disease characterized by papulonodular skin lesions, soft tissue masses, joint contractures, gingival hypertrophy and osteolytic bone lesions. Its onset is in infancy or early childhood. The most commonly affected sites are the nose, chin, ears, scalp, back and knees. The accumulation of an amorphous, hyaline material is typical in the skin and the other organs. Herein, we report a 14-month-old boy who presented with confluent pink papules on the paranasal folds and the chin, and nodular lesions on the periauricular and perianal regions. He had gingival hypertrophy and contractures of the shoulders, knees and elbows. He also had third-degree consanguineous parents. Histopathological studies confirmed the diagnosis of JHF with the presence of increased numbers of fibroblasts embedded in a hyalinized connective tissue stroma.

Fibroma↗

Spindle cell lipomas. A report of two cases: one with multiple lesions.

BACKGROUND: Spindle cell lipoma is an unusual and histologically distinctive form of lipoma occurring primarily in older individuals. OBJECTIVE: The dermatologic surgeon and pathologist should be familiar with this variant of lipoma. METHODS: We are reporting two cases of spindle cell lipomas with the results of histologic examination with special stains and immunohistochemical studies. RESULTS: The neoplasms consisted of lobulated masses of mature adipose tissue with areas of spindle cell proliferation. There was strong positive staining with antibodies for vimentin within the areas of spindle cells proliferation. One of the cases is unusual in that multiple lesions are occurring in a young patient. CONCLUSION: Recognition of the histologic pattern is important in distinguishing the benign spindle cell lipoma from the malignant liposarcoma.

Adipose Tissue↗

Lymphoma of bone, muscle, and skin: CT findings.

Lymphoma can involve any part of the musculoskeletal system. Primary musculoskeletal lymphoma is rare but can occur in bone (reticulum cell sarcoma) or in the skin and subcutaneous tissues (mycosis fungoides). Secondary involvement in the musculoskeletal system is more common and can have a variety of radiologic findings. The definitive diagnosis of musculoskeletal lymphoma, however, is difficult to make by using imaging criteria alone. Any part of the musculoskeletal system can be involved and, therefore, a wide variety of primary and secondary neoplasms or inflammatory processes may have similar radiologic findings. The main differential diagnostic considerations, depending on the age of the patient and the clinical presentation, include osteosarcoma, Ewing's tumor, metastatic disease (from breast, lung, thyroid, or renal primary lesions), as well as chronic osteomyelitis or myositis. Primary and secondary bone lymphoma can be indistinguishable radiologically and histologically, but modern imaging techniques allow more accurate differentiation of primary from secondary bone involvement. This pictorial essay illustrates the CT findings of primary and secondary lymphoma involving bone, muscle, and skin and subcutaneous tissues.

Bone Neoplasms↗

Radiographic imaging of musculoskeletal neoplasia.

BACKGROUND: Imaging is an integral part of the diagnosis, staging and evaluation of outcomes for bone and soft-tissue neoplasms. Each of the available imaging tools has a different role. METHODS: The authors reviewed the efficacy of the current imaging modalities in the diagnosis, staging, and follow-up of patients with musculoskeletal neoplasia. RESULTS: Plain-film radiography remains the gold standard in the differential diagnosis of bone lesions. Bone scintigraphy is an excellent screening modality, and computed tomography is especially useful in evaluating lesions of the axial skeleton. The superior soft-tissue resolution and multiplanar capabilities achieved with magnetic resonance imaging, however, has replaced the need for CT scans in many cases. CONCLUSIONS: The technological advances seen in recent years in all areas of imaging have improved the capabilities of these modalities to assist in the diagnosis, definition of tumor extent, and accurate staging of musculoskeletal tumors.

Bone Neoplasms↗

Radiology and pathology of fat.

Conventional roentgenograms usually reveal a fatty tumor by its characteristic radiolucency. Exceptions to this presentation are the intrathoracic fatty tumors which are more often radiopaque than radiolucent. Obesity and steroids may cause focal, excessive accumulation of fat which can cause a mass effect and become symptomatic from pressure changes. Tubular widening of the superior mediastinum is characteristic mediastinal lipomatosis. Liposarcomas may or may not appear radiolucent. When a calcified retroperitoneal mass appears at least partially radiolucent, a liposarcoma must be suspected. Intrarenal fatty masses may be confused by overlapping bowel gas. Nephrotomograms, prior to the injection of contrast medium, will reveal the presence of fatty process. Obliteration of juxtavisceral or juxtamuscular fat is an important finding for localizing a disease process. CT is the most informative imaging method in establishing the presence of a fatty mass and for localizing a pathological process which infiltrates the juxtavisceral fat.

Adipose Tissue↗

Malignant schwannomas presenting as malignant fibrous histiocytomas.

Two soft tissue neoplasms considered to represent malignant fibrous histiocytomas by light microscopy showed typical findings indicative of Schwann cell origin when examined by electron microscopy. These findings included the presence of cells exhibiting long interdigitating cytoplasmic processes, which frequently contained collections of microtubules and were surrounded by thin, frequently interrupted, basal lamina material. In addition, pinocytotic activity at the cell surfaces and immature junctions joining apposing cellular membranes were identified. No evidence of fibroblastic, fibrohistiocytic, or myofibroblastic differentiation was identified ultrastructurally in the neoplastic cells. Our findings stress the need for ultrastructural examination to adequately classify soft tissue sarcomas. The 2 cases presented illustrate that there are some schwannomas that may be inaccurately classified as malignant fibrous histiocytomas if only light microscopy is used. It seems that only by ultrastructural means is it possible to accurately classify these peculiar neoplasms.

Cell Nucleus↗

[Significance of ultrastructure of peripheral nerve tumors in pathological and differential diagnoses].

Biopsy material fixed with formalin in 10 cases of peripheral nerve tumors and 5 soft tissue tumors were studied with special reference to the significance of the ultrastructure of peripheral nerve tumors in histopathological and differential diagnosis. The results demonstrated that electron microscopic examination of formalin-fixed tissue may still reveal their ultrastructural characteristics. This is of certain practical value in pathological and differential diagnosis in some difficult peripheral nerve tumors.

Diagnosis, Differential↗

The soft tissue myxoma of the head and neck region--report of a case and literature review.

A case of soft tissue myxoma in scalp is reported in a patient with a history of congenital cleft lip and palate. Review of the literature shows head and neck soft tissue myxoma occurs almost in every decade of life with peak occurrence in the fourth decade. Men are affected more than women. The most common location in the head and neck region is the palate, followed by the parotid area. The prognosis of this soft tissue tumor is good. Conservative surgical excision with adequate margins is the treatment of choice. Several theories concerning the pathogenesis of this tumor are discussed, with the theory that a basic error in tissue metabolism during initial growth is the most compatible to the elements of this case. Differential diagnosis regarding this type of lesion is also discussed.

Diagnosis, Differential↗

Analysis of chromosome 22q as an aid to the diagnosis of rhabdoid tumor: a case report.

Malignant rhabdoid tumor is a highly aggressive tumor of childhood that may present as a soft-tissue primary tumor. We report a soft-tissue neoplasm that was polyphenotypic by immunohistochemical expression of epithelial, mesenchymal, and neural markers and did not meet the criteria for any of the usual pediatric small round-cell tumors. The findings raised the diagnosis of rhabdoid tumor, leading to testing for WT1 mRNA and protein expression, which were positive, as has been reported for renal rhabdoid tumor. This tumor had the typical clinical behavior of rhabdoid tumor with therapy resistance and early tumor-related death. Multicolor spectral karyotyping of this neoplasm showed a balanced translocation between chromosomes 1 and 22 with breakpoints at 1p36 and 22q11-12. The latter region is commonly involved in rhabdoid tumor. This change was also identified by fluorescence in situ hybridization. This case suggests that studies of chromosome 22 may be required to distinguish rhabdoid tumor from other soft-tissue tumors.

Chromosomes, Human, Pair 22↗

Immunohistochemical detection of human herpes virus-8 latent nuclear antigen-1 is useful in the diagnosis of Kaposi sarcoma.

Kaposi sarcoma is a low-grade vascular neoplasm that has been shown by molecular analysis to uniformly express the latent nuclear antigen-1 of human herpes virus 8. Differentiating Kaposi sarcoma from other benign or malignant vascular tumors, as well as other nonvascular spindle cell soft-tissue neoplasms, can be challenging. Thus, detection of human herpes virus 8 in fixed tissues would be diagnostically useful. Recently, a monoclonal antibody to human herpes virus 8 latent nuclear antigen-1 has become commercially available for immunohistochemical analysis. We sought to study the sensitivity and specificity of this antibody in the detection of human herpes virus 8 latent nuclear antigen-1 in Kaposi sarcoma. Fixed, paraffin-embedded tissue sections from 21 cases of Kaposi sarcoma, nine cases of spindle cell hemangioma, five cases of cutaneous angiosarcoma, five cases of dermatofibrosarcoma protuberans, one case of vascular transformation of a lymph node, four cases of pilar leiomyoma, four cases of stasis dermatitis, four cases of pyogenic granuloma, and three cases of spindled melanoma were examined immunohistochemically using the rat monoclonal antibody to human herpes virus 8 latent nuclear antigen-1, open reading frame-73 (Advanced Biotechnologies Inc.). Tissue sections were stained with automated immunostainers (Ventana) using heat-induced epitope retrieval and a standard DAB detection kit (Ventana) modified to detect rat Ab. Strong, diffuse, nuclear staining in >10% of tumor cells was considered a positive result. In all, 21/21 cases of Kaposi sarcoma showed strong, diffuse, nuclear staining for human herpes virus 8 latent nuclear antigen-1 (100%), whereas all cases of spindle cell hemangioma, cutaneous angiosarcoma, dermatofibrosarcoma protuberans, vascular transformation of lymph node, pilar leiomyoma, stasis dermatitis, pyogenic granuloma, and spindled melanoma were negative for this antigen. The monoclonal antibody to human herpes virus 8 latent nuclear antigen-1, open reading frame-73, is a highly sensitive and specific marker of human herpes virus 8 infection in paraffin-embedded tissue sections of Kaposi sarcoma. As such, it is an extremely useful tool for differentiating between Kaposi sarcoma and other vascular and nonvascular spindle cell lesions, which do not express human herpes virus 8 latent nuclear antigen-1.

Antibodies, Monoclonal↗

The Amsterdam fast neutron therapy project: a final report.

In the period from February 1975 through September 1981 a total of 435 patients received radiotherapy with the 14 MeV d + T neutron generator, hospital based in the Netherlands Cancer Institute (the Antoni van Leeuwenhoek Hospital). Preliminary data on clinical results were published during the past few years. In this paper a final report is given of the program. The results can be summarized as follows: The neutron generator fulfilled the criteria for clinical use, that is it was reliable and had the required minimal output of 10(12) neutrons s-1. However, the dose distribution was more comparable with a 250 kV X-ray machine than with a modern accelerator. A number of physical parameters of importance for clinical neutron dosimetry have been determined for our therapy unit. These data, as well as the results of dosimetry intercomparisons in which our institute participated, contributed in the drafting of a European protocol for clinical neutron dosimetry. Pilot studies were carried out on different tumor sites, including head and neck, brain, pelvis, soft tissue and pulmonary metastases. In many patients local tumor control was seen, however, often concomitant with severe complications, especially in deep seated tumors. Randomized clinical trials were carried out for head and neck tumors (in collaboration with some other European centers) and for inoperable bladder and rectal tumors. No significant difference was observed in local tumor control or late morbidity between photon and neutron irradiation for the head and neck tumors. Also the results for pelvic tumors failed to demonstrate an advantage for neutron therapy. In this study two neutron arms were used with different dose schedules. As could be expected a higher local control rate was noticed for the higher neutron dose group, but concomitant with a higher complication rate. From our experience we have to conclude that treatment with our fast neutron treatment facility did not result in a benefit over photon irradiation. It seemed that the differential effect between tumor and normal tissues is smaller with fast neutrons than with photons.

Evaluation Studies as Topic↗

[NMR examination of soft tissue tumors].

Preliminary findings on NMR imaging of 30 soft tissue tumors demonstrated the indispensable value of this examination (particularly when a surface antenna is used) for preoperative investigation and diagnosis of tumoral recurrence when compared with other radiologic techniques. The possible potential of NMR imaging for characterization of tissues, apart from lipoma or liposarcoma, cannot be evaluated at the present time.

Abdominal Neoplasms↗