[Dermatofibrosarcoma protuberans. Study of 2 cases].
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Aged↗
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Dermatofibrosarcoma protuberans: a case report.
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Adult↗
[Clinico-morphological characteristics of Darier-Ferrand dermatofibrosarcoma].
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Adult↗
High proliferative activity excludes dermatofibroma: report of the utility of MIB-1 in the differential diagnosis of selected fibrohistiocytic tumors.
CONTEXT: Dermatofibroma is a benign fibrohistiocytic tumor composed of a mixture of fibroblastic and histiocytic cells. The diagnosis of this tumor is generally uncomplicated; however, rare variants may be difficult to distinguish from malignant fibrohistiocytic tumors. Deep penetrating dermatofibroma may be difficult to distinguish from dermatofibrosarcoma protuberans, and pseudosarcomatous dermatofibroma and dermatofibroma with monster giant cells share morphologic similarities with malignant fibrous histiocytoma and atypical fibroxanthoma. OBJECTIVE: To find an immunohistochemical marker or markers that differentiate between fibrohistiocytic lesions of skin. DESIGN: We evaluated the immunophenotypic characteristics of 83 fibrohistiocytic tumors (36 typical dermatofibromas, 16 cases of dermatofibrosarcoma protuberans, 16 malignant fibrous histiocytomas, and 15 atypical fibroxanthomas) using antibodies against MIB-1 (Ki-67), factor XIIIa, CD34 (HPCA-1), HHF35 (muscle-specific actin), 1A4 (smooth muscle actin), cytokeratin (AE1/AE3, CAM 5.2, and 34betaE12), S100 protein, and desmin. RESULTS: A high proliferative index detected by MIB-1 staining excluded the possibility of dermatofibroma and was diagnostically useful in separating this entity from dermatofibrosarcoma protuberans, malignant fibrous histiocytoma, and atypical fibroxanthoma. A low proliferative index, however, could not differentiate dermatofibroma from dermatofibrosarcoma protuberans. Factor XIIIa reactivity was not helpful for the diagnosis of dermatofibroma, whereas CD34 reactivity was statistically significant in the diagnosis of dermatofibrosarcoma protuberans. The sensitivity of these 2 markers is low and therefore of questionable practical diagnostic value. CONCLUSION: Evaluation of the proliferative index may further assist in distinguishing dermatofibroma from dermatofibrosarcoma protuberans, atypical fibroxanthoma, and malignant fibrous histiocytoma.
Biomarkers, Tumor↗
Immunophenotyping of dermal spindle cell tumors: diagnostic value of monocyte marker Ki-M1p and histogenetic considerations.
Various studies have reported the utility of anti-CD34 staining in the differential diagnosis of dermal spindle-cell tumors. To investigate whether monoclonal antibody Ki-M1p might add practical diagnostic information, we examined a total of 120 cutaneous spindle cell neoplasms using a panel of markers. Anti-CD34 antibody QBEnd/10 consistently stained dermatofibrosarcomas, Kaposi's sarcomas, neurofibromas, and, to a lesser extent, hemangiopericytomas. A positive reaction was also found in > 18% of the dermatofibromas. Ki-M1p staining showed an intense immunoreaction in all dermatofibromas, whereas no reactivity was observed in dermatofibrosarcomas. In addition, a subset of cells was labeled in atypical fibroxanthomas and Kaposi's sarcomas. Neurofibromas, spindle-cell hemangioendotheliomas, and hemangiopericytomas were negative. Dermatofibrosarcomas and atypical fibroxanthomas also moderately expressed smooth muscle-specific actin. Immunohistochemically, a discrimination between dermatofibrosarcomas and neurofibromas was possible only by means of an antibody against the nerve growth factor receptor. We conclude that the combination of several antibodies, in particular anti-CD34 and Ki-M1p, may improve the accuracy of diagnostic immunohistochemistry in the field of cutaneous spindle cell tumors. We speculate that dermatofibroma is primarily a macrophage-rich inflammatory lesion in which cytokine secretion induces a secondary proliferation of fibroblasts, whereas dermatofibrosarcoma is likely to issue from primitive dermal cells of uncertain origin.
Antibodies, Monoclonal↗
COL1A1-PDGFB fusion in a pediatric Bednar tumor with 2 copies of a der(22)t(17;22).
We present a 10-year-old girl with a pure Bednar tumor (pigmented dermatofibrosarcoma protuberans) of the right shoulder. Cytogenetic analysis demonstrated 47 chromosomes with 2 copies of a derivative chromosome 22, der(22)t(17;22)(q22;q13). Fluorescence in situ hybridization (FISH) analysis demonstrated the COL1A1-PDGFB fusion on both der(22) chromosomes. By RT-PCR and sequencing, we observed a fusion of the COL1A1 exon 41 with PDGFB exon 2. This pure pediatric Bednar tumor in a child, like childhood dermatofibrosarcoma protuberans, had a linear structural abnormality rather than a ring chromosome that is more commonly encountered in adult Bednar and dermatofibrosarcoma protuberans tumors. The underlying molecular abnormality in this pediatric Bednar tumor is the same as in dermatofibrosarcoma protuberans.
Base Sequence↗
Fibrohistiocytic lipoma: twelve cases of a previously undescribed benign fatty tumor.
A lipoma with a spindled proliferation within it, resembling known (myo)fibroblastic lesions such as fibrous histiocytoma or dermatofibrosarcoma protuberans, (ie, fibrohistiocytic lipoma), has not been previously reported. This tumor varies from other classic lipoma variants, including spindle cell lipoma, myolipoma, angiolipoma, and fibrolipoma. We examine the clinicopathologic findings of this new lipoma variant. The Soft Tissue Pathology Registry of the Armed Forces Institute of Pathology was searched for patients with "lipoma with fibrohistiocytic proliferation." Lesions that were better classified as other entities were excluded. Patient slides and clinical history, including associated lesions, family history, duration of symptoms, history of trauma, natural progression, and treatments, were reviewed. Immunohistochemistry was performed on cases with available material (n = 6). Twelve patients with fibrohistiocytic lipoma were included. All tumors revealed a well-distributed quilt-like proliferation or solid focus of slightly plump to relatively bland spindled cells with collagenous stroma in short fascicular and storiform growth patterns. These spindled cells resembled those seen in either fibrous histiocytoma or dermatofibrosarcoma protuberans. However, the spindled proliferation was all within a well-circumscribed lipoma. The lesions lack the dermal involvement or plump pleomorphism of fibrous histiocytoma and the dermal involvement or infiltrative growth pattern of dermatofibrosarcoma protuberans. The fatty component demonstrated heterogeneously sized adipocytes, as those seen in other lipomas. Inflammation and hemosiderin were minimal. Mast cells were not identified. The tumors were typically found in the subcutis of the trunk of men (10 of 12; one each on the wrist and leg; mean age, 31 years). The average size of the lesions was 3.0 cm, and they were present for a mean duration of 10 months prior to surgical excision. One patient had two concurrent lesions; all others had solitary tumors. Another patient had a intracranial dermoid cyst removed during childhood. Four patients had a personal or family history of hypercholesterolemia, hypertension, or myocardial infarction. There was no history of antecedent trauma. Cases studied were positive for vimentin, calponin (5 of 5), CD34 (3 of 5), and occasionally KP-1 or lysozyme in the spindled component, and all cases studied were negative for the actins, caldesmon, S-100 protein, desmin, cytokeratins, and epithelial membrane antigen. Although the actins were negative in our laboratory, the more sensitive calponin positivity suggests myofibroblastic phenotype of the spindled component of this lesion. CD34-positive fibroblasts were present in three of five cases. Of eight patients with follow-up, there were no recurrences; all patients were alive and free of disease over a mean of 10 years (range, 2 months to 31 years). We have identified a lipoma variant, fibrohistiocytic lipoma, that has not been previously described. In our experience the morphology and calponin positivity suggest myofibroblastic phenotype for the spindled cells, within a lipoma. This entity can be distinguished from fibrous histiocytoma, fibromatosis, dermatofibrosarcoma protuberans, spindle cell lipoma and other lipoma, and liposarcoma variants.
Adult↗
[Soft tissue sarcoma in the head and neck--analysis of 87 patients].
From 1964 to 1984, 87 patients with soft tissue sarcoma (excluding lymphreticulum system sarcoma) in the head and neck were treated in our hospital. All were proved by pathology. The incidence rate of fibrosarcoma was the highest (36.8%), hemangiosarcoma the lowest (5.8%). There was no liposarcoma or synovial sarcoma in this group. The most frequently involved site was the area near the nasal cavity and maxillary sinus for fibrosarcoma (66%), the base of tongue for hemangiosarcoma (60%), scalp for dermatofibrosarcoma protuberans (54%), parapharyngeal space and soft tissue of the neck for neurogenic sarcoma (45%). Metastasis rate to the lymph nodes was 16-20% for rhabdomyosarcoma, hemangiosarcoma and malignant fibrohistiocyte tumor. No distant metastasis was found in dermatofibrosarcoma protuberans and malignant fibrohistiocyte tumor. Distant metastasis rate was 10-20% for the other types. Local recurrence rate was 9.1% for dermatofibrosarcoma protuberans, 88.9% for malignant fibrohistiocyte tumor, 30-66.7% for the other sarcomas. The 5- and 10-year survival rates were 20% and 0%, 37.5% and 0%, 91.6% and 91.6% for rhabdomyosarcoma, malignant fibrohistiocyte tumor and dermatofibrosarcoma protuberans. There were differences in clinical behavior for soft tissue sarcomas in the head, neck and in the trunk. Highly malignant sarcomas, such as liposarcoma, synovial sarcoma and rhabdomyosarcoma were rare in the head and neck but the low malignant sarcomas in the head and neck were 3-10 times as common as those in the trunk. Distant metastasis rate of the fibrosarcoma was 20% in head and neck and 6.7%in trunk, the 5- and 10-year survival rates were 36% and 77.9%, 25% and 73.6%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
LN-2 (CD74). A marker to distinguish atypical fibroxanthoma from malignant fibrous histiocytoma.
BACKGROUND: In this study, the authors examined the expression of LN-2, an antigen expressed by B cells, macrophages, and Reed-Sternberg cells, in a variety of spindle cell lesions of the skin to determine whether LN-2 immunoreactivity can be used to differentiate among these tumors. For comparison, they examined CD34 antigen expression in these lesions, which has been shown to be a useful marker in differentiating dermatofibrosarcoma protuberans from dermatofibroma. METHODS: Immunocytochemistry with anti-LN-2 and anti-CD34 monoclonal antibodies on formalin fixed, paraffin embedded material was performed on 102 spindle cell lesions, including dermatofibroma, dermatofibrosarcoma protuberans, atypical fibroxanthoma, malignant fibrous histiocytoma, leiomyoma, and neurofibroma. RESULTS: LN-2 immunoreactivity did not distinguish between dermatofibroma and dermatofibrosarcoma protuberans, both of which showed weak immunoreactivity. In marked contrast, 90% of cases of malignant fibrous histiocytoma showed strong staining for LN-2, whereas the vast majority (90%) of cases of atypical fibroxanthoma were negative or stained only weakly with anti-LN-2 antibodies. Of the two cases of atypical fibroxanthoma that stained strongly for LN-2, both lesions were > 2 cm in size and extended deep into the subcutaneous fat. CONCLUSIONS: Differential expression of the LN-2 antigen by atypical fibroxanthoma and malignant fibrous histiocytoma distinguishes these two lesions and suggests that acquisition of LN-2 positivity may be a marker of tumor progression.
Antibodies, Monoclonal↗
Giant cell fibroblastoma. New histological observations.
Giant cell fibroblastoma is a rare mesenchymal neoplasm of unknown origin and uncertain clinical course. The neoplasm has been considered by some authors as a juvenile variant of dermatofibrosarcoma protuberans. We report a patient with giant cell fibroblastoma, now 28 months following surgical removal, in which the neoplasm was characterized histologically by a proliferation of spindle-shaped cells intermixed with pseudovascular channels called "angiectoid spaces." The spaces were lined by large cells with pleomorphic nuclei intermixed with multinucleated cells. Immunohistochemically, the tumor stained diffusely for vimentin and CD34, a surface glycoprotein expressed by some mesenchymal neoplasms including dermatofibrosarcoma protuberans. We postulate a mechanism of formation of the angiectoid spaces based on histopathological findings in serially sectioned portions of the neoplasm. Positive staining of tumor cells for CD34 supports a possible relationship of the neoplasms with dermatofibrosarcoma protuberans.
Adolescent↗
Tenascin expression in cutaneous fibrohistiocytic tumors. Immunohistochemical investigation of 24 cases.
We studied the immunolocalization of the extracellular matrix glycoprotein tenascin in a series of 24 cutaneous fibrohistiocytic tumors, including seven benign lesions (benign fibrous histiocytoma/dermatofibroma), six intermediate malignancy lesions (dermatofibrosarcoma protuberans), and 11 malignant lesions (three atypical fibroxantomas and eight malignant fibrous histiocytomas). The results of the immunohistochemical staining were evaluated semiquantitatively. All lesions expressed tenascin in the extracellular matrix, with some differences in the distribution of the immunoreactivity. In benign fibrous histiocytoma and in dermatofibrosarcoma protuberans, there was a homogeneous, intense, and diffuse staining of the extracellular matrix (++); the only exception was the homogenized, sclerotic collagen present in late, regressing benign fibrous histiocytoma, which showed a weak and patchy reactivity (+). In atypical fibroxantomas and in malignant fibrous histiocytomas, there was an irregular distribution of the positivity within the tumor matrix (+). Prominent staining of the cytoplasm of several neoplastic cells was observed in atypical fibroxantoma and malignant fibrous histiocytoma (++), focal cytoplasmic staining of scattered cells was found in dermatofibrosarcoma protuberans (+), and cytoplasmic staining was absent from benign fibrous histiocytoma (-). These findings indicate a relationship between cytoplasmic and extracellular matrix expression of tenascin in these lesions, with an increase in cytoplasmic staining and a decrease in extracellular matrix staining in the malignant forms. Based on these different staining patterns, tenascin immunolocalization could furnish some help in the differential diagnosis among benign, intermediate malignancy, and malignant cutaneous fibrohistiocytic tumors. Moreover, the intense tenascin staining at the edge of the lesion could be helpful in defining its extent and therefore provide an additional criterion to evaluate the radicality of the surgical procedure.
Dermatofibrosarcoma↗
Soft tissue sarcomas in dermatology.
BACKGROUND: Dermatofibrosarcoma protuberans, atypical fibroxanthoma, and cutaneous leiomyosarcoma are uncommon soft tissue sarcomas that tend to have a high recurrence rate when removed with standard excisional surgery, and in some cases have been reported to metastasize. These tumors have been reported in the surgical, medical, and pathology literature. The diagnosis and treatment of these tumors is still evolving. Wide excision and surgical techniques involving meticulous surgical margin control appear to be the most efficacious treatment for these problematic tumors. OBJECTIVE: The surgical, medical, and pathology literature was extensively reviewed to collate the observations of multiple investigators, and to summarize their findings. Literature from medical journals of multiple subspecialties as well as textbooks were reviewed with particular emphasis on diagnosis, histologic features including immunohistochemistry, and useful surgical techniques that facilitate the removal of these difficult tumors. RESULTS: Dermatofibrosarcoma protuberans is a soft tissue neoplasm of intermediate malignancy that shows a high recurrence rate when treated with standard excision. Wide surgical excision using a 3-cm margin greatly reduces the risk of recurrence, but is not practical in some cases. Evidence is continuing to accumulate that Mohs micrographic surgery may be the treatment of choice for this tumor. Atypical fibroxanthoma is best thought of as a superficial form of malignant fibrous histiocytoma. It is histologically indistinguishable from certain forms of malignant fibrous histiocytoma and differs primarily in its size and superficial location. These tumors are best removed surgically. For most smaller tumors a 1-cm margin, which is carried down to the subcutaneous tissue, provides adequate treatment. In cases where tissue conservation is important, Mohs micrographic surgery has been shown to be very effective. Cutaneous leiomyosarcoma is a rare superficial soft tissue sarcoma that has a high incidence of recurrence after excision and can metastasize. Because of the small number of patients treated, treatment recommendations are still evolving. Currently, wide local excision is recommended with a 3-5 cm margin and removal of the underlying subcutaneous tissue. Mohs micrographic surgery has been used in a small number of cases with good success. CONCLUSION: Dermatofibrosarcoma protuberans, atypical fibroxanthoma, and cutaneous leiomyosarcoma are soft tissue sarcomas that have a high rate of recurrence when treated with standard surgical techniques and may occasionally metastasize. When removing these tumors it is very important to take adequate margins. Mohs micrographic surgery is proving to be very useful in treating these difficult neoplasms.
Dermatofibrosarcoma↗
Bednar tumor of the foot: a case report.
Pigmented dermatofibrosarcoma protuberans (Bednar tumor) is a rare neoplasm accounting for approximately 1-5% of all cases of dermatofibrosarcoma protuberans. The majority occurs on the trunk, and the remainders are more or less equally distributed in the upper and lower extremities and the head and neck. Microscopically it is characterized by spindled cells arranged in a tight storiform pattern and admixed with a small population of melanin-containing dendritic cells. The dendritic cells are the primary feature that distinguish this lesion from conventional dermatofibrosarcoma protuberans. We report here a case of Bednar tumor occurring on the dorsal aspect of the foot in a young female.
Adult↗
Pediatric penile tumors of mesenchymal origin.
OBJECTIVES: To describe 2 cases of mesenchymal pediatric penile tumors in a 13-year-old boy and an 11-month-old infant. Mesenchymal tumors are rare in the male external genitalia. METHODS: Two patients, one aged 13 years and one 11 months, presented with painless penile masses at the penoscrotal junction. The evaluation included serial clinical examinations and ultrasonography. Both lesions were surgically excised after increasing in size. Because of malignancy, the first patient underwent wide reexcision of the tumor site. RESULTS: The pathologic findings of the first case were combined dermatofibrosarcoma protuberans, giant cell fibroblastoma, and fibrosarcoma, a malignant tumor. Immunohistochemical staining showed strong and diffuse CD34 cytoplasmic positivity in the giant cell fibroblastoma and dermatofibrosarcoma protuberans components; the dedifferentiated fibrosarcoma tumor cells were negative for this antibody. The second case was myofibroma, a benign fibroblastic tumor with a prominent myofibroblastic component that is congenital and tends to occur within the first few months after birth. Neither patient had recurrence at 16 months and 3 years of follow-up. To our knowledge, this is the first reported case of combined dermatofibrosarcoma protuberans, giant cell fibroblastoma, and fibrosarcoma of the penis and the second reported case of isolated penile myofibroma. CONCLUSIONS: Penile tumors should be included in the differential diagnosis of pediatric penile masses. Surgical resection is often curative, but patients with malignant tumors should receive careful follow-up to monitor for recurrence.
Adolescent↗
Flow cytometric DNA analysis of malignant fibrous histiocytoma and related fibrohistiocytic tumors.
Fibrohistiocytic neoplasms with similar histologic characteristics may have vastly different biologic behaviors. We studied 23 fibrohistiocytic tumors to determine if cellular DNA content was correlated with clinical outcome. Archival paraffin blocks of 9 malignant fibrous histiocytomas (MFH), 3 dermatofibrosarcoma protuberans, 9 dermatofibromas, 1 juvenile xanthogranuloma, and 1 nodular fasciitis were processed, stained with propidium iodide, and analyzed by flow cytometry. Five of 9 (56%) MFH and 1 of 3 (33%) dermatofibrosarcoma protuberans were aneuploid. All 11 benign fibrohistiocytic tumors were diploid. Local recurrence occurred in 3 of 5 (60%) cases of aneuploid MFH, but in none with a diploid MFH tumor. No cases of dermatofibrosarcoma protuberans or benign tumors recurred. Aneuploidy was associated with decreased survival, as 2 of 5 patients with aneuploid MFH died within 1 year of diagnosis whereas all 4 patients with diploid MFH tumors are alive after an average follow-up of 4 years (range, 1 to 11). The diploid and aneuploid groups did not differ in clinical stage at the time of diagnosis. Our results indicate that retrospective DNA analysis can detect aneuploidy in both MFH as well as other fibrohistiocytic tumors, and that aneuploidy in MFH may place the patient at increased risk for local recurrence and mortality.
DNA↗
CD-34 is expressed by a distinctive cell population in peripheral nerve, nerve sheath tumors, and related lesions.
The pattern of CD-34 antigen (human progenitor cell antigen) immunoreactivity was studied within normal nerve, and a variety of nerve sheath and neuroectodermal tumors. Besides normal nerves, 111 soft tissue tumors were studied, including 17 neurofibromas, 10 neurilemomas, 12 malignant peripheral nerve sheath tumors, 1 melanocytic schwannoma, 21 fibroblastic lesions, 31 fibrohistiocytic lesions, seven neuroectodermal lesions, and 10 miscellaneous tumors. CD-34-positive dendritic cells were consistently identified within the endoneurium of normal nerve, all neurofibromas, dermatofibrosarcomas, and Antoni B (but not Antoni A) areas of neurilemomas. CD-34 was not expressed in the majority (eight of 10 cases) of malignant peripheral nerve sheath tumors. CD-34 was also lacking in all fibroblastic lesions (nodular fasciitis, fibromatosis, keloid, fibrosarcoma) and in neuroectodermal tumors that are not generally considered to show true nerve sheath differentiation (neurotropic melanoma, clear cell sarcoma, neuroepithelioma). We conclude that CD-34 (or a closely related epitope) defines a normally occurring nerve sheath cell that appears to be cytologically and immunophenotypically distinct from a fibroblast and conventional Schwann cell. The antigen can also be localized to benign nerve sheath tumors, but tends to be lost in malignant ones. The consistent presence of CD-34 within all 13 cases of dermatofibrosarcoma protuberans can be used as evidence in support of the view that these lesions are variants of nerve sheath tumors, and distinct from benign fibrous histiocytomas which consistently lack the antigen. Finally, expression of CD-34 by one of three giant cell fibroblastomas reinforces the close relationship between this tumor and dermatofibrosarcoma protuberans.
Antigens, CD↗
Bednar tumor: a report of two cases.
We have recently treated two Japanese with Bednar tumor, pigmented dermatofibrosarcoma protuberans. The tumors were multilobular with brown-pigmentation and an irregular surface. Firm nodules were growing into the deep subcutaneous tissue, but they were movable over the underlying tissue. Histopathological examination and immunohistochemical studies showed the typical features of Bednar tumor and positive staining for CD34, which is known to be expressed in dermatofibrosarcoma protuberans. Bednar tumor is thought to be a variant of dermatofibrosarcoma protuberans and to have intermediate malignancy.
Adult↗
[Malignant tumors of the abdominal wall. 10 years of experience at the Saint-Louis Hospital].
The authors present a 10-year retrospective study of 52 patients with dermatofibrosarcomas (33 cases), sarcomas (13 cases) and desmoid tumours (6 cases) operated in their department. All these tumours were located in the abdomen or adjacent regions. Resection margins were 5 cm for dermatofibrosarcomas and 2 cm for sarcomas and desmoid tumours, with a healthy deep anatomical barrier. Dermatofibrosarcomas were repaired by direct suture in 18% of cases, directed healing in 15%, healing and graft in 45% and flap in 31% of cases. 61% of sarcomas were sutured directly, 7% were treated by directed healing and 23% required cover by a flap. All desmoid tumours were closed by primary suture. The quality of the first surgical resection remains the predominant prognostic factor.
Abdominal Neoplasms↗