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Incidence patterns of soft tissue sarcomas, regardless of primary site, in the surveillance, epidemiology and end results program, 1978-2001: An analysis of 26,758 cases.

Soft tissue sarcomas (STS) are a heterogeneous group of uncommon tumors that show a broad range of differentiation that may reflect etiologic distinction. Routine tabulations of STS are not morphology-specific. Further, the lack of inclusion of sarcomas arising in all organs in most standard evaluations underestimates the true rates. We analyzed the 1978-2001 Surveillance, Epidemiology and End Results program incidence rates of STS regardless of primary site, except bones and joints, using the 2002 criteria of the WHO classification. There were 26,758 cases available for analysis. Leiomyosarcomas accounted for 23.9%, malignant fibrous histiocytomas 17.1%, liposarcomas 11.5%, dermatofibrosarcomas 10.5%, rhabdomyosarcomas 4.6% and angiosarcomas 4.1%. Almost half (47.9%) of the sarcomas arose in the soft tissues, 14.0% in the skin and 7.0% in the uterus. Overall, incidence rates were highest among black women (6.26/100,000 woman-years) and the lowest among white women (4.60/100,000). Age-adjusted rates increased at 1.2% and 0.8% per year among white males and females, respectively, both trends statistically significant, while rates among blacks declined slightly. About 40% of leiomyosarcomas among women were uterine in origin, with a black/white rate ratio of 1.7. This rate ratio increased to 2.0 when we accounted for the lower prevalence of intact uteri among black compared to white women. Total STS rates rose exponentially with age. Rates for both uterine leiomyosarcoma and dermatofibrosarcoma increased rapidly during the childbearing years, peaking at about age 40 and 50, respectively. Incidence patterns of STS varied markedly by histologic type, supporting the notion that these tumors may be etiologically distinct.

Adolescent↗

Vulvar sarcoma: clinical implications.

This is a review of the histories of 47 patients with vulvar sarcoma, consisting of leiomyosarcoma (25), malignant fibrous histiocytoma (5), epithelioid sarcoma (8), dermatofibrosarcoma protuberans (9), including 7 from our own institute. When compared to the biological behaviour of sarcomas from other anatomic sites of the body, no essential differences were found. The prognosis after the appearance of regional or distant recurrence was poor and prevention of local recurrence by wide excision was the best way to improve the prognosis of leiomyosarcoma and dermatofibrosarcoma protuberans. However, the poor prognosis of epitheloid sarcoma did not change. Elective treatment of regional lymph nodes was not indicated in the four tumor types discussed and dissection of metastatic inguinal nodes was rarely beneficial. However, distressing local problems were prevented in a patient with epithelioid sarcoma and lasting benefit was seen in a patient with malignant fibrous histiocytoma who developed an inguinal metastasis after a 3-year disease-free interval. The beneficial effect of resection of pulmonary metastasis needs more attention.

Adolescent↗

CD34-reactive fibrous papule of the nose.

In human skin, the CD34 antigen is expressed on endothelium, periadnexal cells, and a population of reticular dermal interstitial cells. CD34 expression is characteristic of dermatofibrosarcoma protuberans and several other neoplasms, but not of typical fibrous papules of the nose. We describe a 16-year-old white girl with a slowly growing papule on the nose. Histopathology showed a dermal tumor with a superficial component of branched, thin-walled blood vessels and a deeper component of benign-appearing, spindle-shaped cells. These cells uniformly and strongly expressed CD34, but not factor XIIIa or markers of melanocytic, neural, muscular, vascular, or histiocytic differentiation. We consider this lesion a CD34-reactive fibrous papule. This benign tumor must be clearly distinguished from dermatofibrosarcoma protuberans, which also is composed of bundles of CD34-reactive spindle-shaped cells in most cases but has locally aggressive behavior.

Adolescent↗

Utility of rush paraffin-embedded tangential sections in the management of cutaneous neoplasms.

BACKGROUND: Mohs micrographic surgery (MMS) modified by the use of tangential, formalin-fixed, paraffin-embedded histologic specimens is advantageous in treating selected skin neoplasms. OBJECTIVE: To review the use of our experience with a modification of MMS to treat lentigo maligna melanoma (LMM), lentigo maligna (LM) and other melanoma in situ (MIS) lesions, dermatofibrosarcoma protuberans (DFSP), atypical fibroxanthoma (AFX), and angiosarcoma. METHODS: Our experience utilizing a modification of MMS in the treatment of 77 patients with LM or other MIS, 23 patients with LMM, 11 patients with DFSP, 1 patient with AFX, and 1 patient with angiosarcoma was reviewed. Length of follow-up and rate of recurrence were examined. A literature review of this pertinent modification of the Mohs technique was performed. RESULTS: One hundred fourteen patients underwent MMS for melanocytic (LM, MIS, LMM), spindle cell (DFSP, AFX), and vascular malignant neoplasms. One patient developed locally recurrent LM and one patient with LMM developed satellite metastasis. Regional lymph node metastasis occurred in one patient with LMM and in a patient with angiosarcoma. CONCLUSION: The use of Mohs micrographic surgery in conjunction with rush formalin-fixed, paraffin-embedded tangential histologic sections provides the accuracy and tissue conservation of the Mohs procedure while ensuring more confident interpretation of histology in cases of lentigo maligna, lentigo maligna melanoma, dermatofibrosarcoma protuberans, atypical fibroxanthoma, and angiosarcoma.

Aged↗

Expression of protein gene product 9.5 in epithelioid and conventional malignant peripheral nerve sheath tumors.

CONTEXT: Due to the frequent lack of S100 protein expression in malignant peripheral nerve sheath tumors (MPNSTs), especially the epithelioid variant, these tumors are difficult to diagnose without the aid of electron microscopy or a clinical history of neurofibromatosis. METHODS: Protein gene product 9.5 (PGP9.5), a broad neural marker, is expressed in nerve fibers and neurons of both the peripheral and central nervous systems. We compared its expression to that of S100 protein in 16 cases of MPNST. As controls, 6 monophasic synovial sarcomas, 9 leiomyosarcomas, and 5 dermatofibrosarcoma protuberans were included. RESULTS: Expression of PGP9.5 was seen in 15 MPNSTs, with 3 to 4+ positivity in the majority of the cases. Ten cases, 2 epithelioid and 8 conventional MPNSTs, were reactive with PGP9.5, but were negative for S100 protein. Five cases were immunoreactive for both S100 protein and PGP9.5. One case was negative for PGP9.5 but demonstrated focal S100 protein positivity. Expression of PGP9.5 was seen in 4 of 6 synovial sarcomas, 3 of 9 leiomyosarcomas, and none of 5 dermatofibrosarcoma protuberans. CONCLUSION: Although PGP9.5 is not a specific marker for MPNST, it is a more sensitive marker than S100 protein (94% vs 38%). When there is a lack of S100 protein expression and a broad panel of immunostains, such as cytokeratin, epithelial membrane antigen, and smooth muscle actin, yields only focal or equivocal staining, PGP9.5 is a useful diagnostic adjunct in confirming the neural origin of a spindle cell sarcoma.

Adult↗

Tyrosinase expression in malignant melanoma, desmoplastic melanoma, and peripheral nerve tumors.

CONTEXT: Pathologists may encounter problems in the differential diagnosis of malignant melanoma, spindle and epithelioid neoplasms of peripheral nerves, and fibrohistiocytic tumors. Tyrosinase has been demonstrated to be a sensitive marker for melanoma. OBJECTIVE: To determine the specificity of tyrosinase expression in the differential diagnosis of melanoma, desmoplastic melanoma, and peripheral nerve sheath tumors. DESIGN: Immunoreactivity for tyrosinase, HMB-45 (anti-gp100 protein), S100 protein, CD34, and vimentin was studied in 70 tumors, including 15 melanomas (5 desmoplastic, 4 amelanotic, 6 melanotic), 13 malignant peripheral nerve sheath tumors; 10 schwannomas (1 pigmented), 12 neurofibromas (4 pigmented), and 20 fibrohistiocytic tumors (10 dermatofibrosarcoma protuberans and 10 dermatofibromas). Microwave-based antigen retrieval was performed in 10mM citrate buffer, pH 6.0, for 20 minutes at 121 degrees C. RESULTS: All melanomas demonstrated positive immunostaining for tyrosinase, HMB-45, and S100 protein. Immunoreactivity for HMB-45 was generally stronger than that for tyrosinase in amelanotic lesions and significantly stronger in 1 of the desmoplastic lesions. The 4 pigmented neurofibromas were focally positive for tyrosinase, but did not stain for HMB-45. The pigmented schwannoma was focally positive for both tyrosinase and HMB-45. The malignant peripheral nerve sheath tumors, dermatofibrosarcoma protuberans, and dermatofibromas were nonreactive for tyrosinase and HMB-45. CONCLUSIONS: Our results support the sensitivity of tyrosinase expression and demonstrate the relative specificity of tyrosinase as a marker for melanocytic lesions, including desmoplastic melanoma, although pigmented peripheral nerve tumors may demonstrate focal positive staining. Immunoreactivity for tyrosinase and HMB-45 may have been enhanced by the microwave-based antigen-retrieval technique used in this study.

Antigens, CD34↗

Expression of the CD10 antigen (neutral endopeptidase) by mesenchymal tumors of the skin.

BACKGROUND: The CD10 (or CALLA) antigen is a neutral endopeptidase expressed by a variety of non-hemopoietic cells, including fibroblasts in various tissues. So far little or conflicting data exist concerning the expression of CD10 in normal and pathologic skin. METHODS: We investigated the immunohistochemical expression of the CD10 antigen on formalin-fixed, paraffin-embedded tissue specimens of normal skin and 162 cutaneous proliferative lesions, mostly mesenchymal tumors. RESULTS: In normal skin CD10 immunoreactivity was found in periadnexal mesenchymal cells. In skin tumors, CD10+ cells were found mainly in dermatofibromas and, to a lesser extent, in Dermatofibrosarcoma protuberans and neurofibromas. Metastatic (and to a lesser extent primary) melanomas also occasionally expressed CD10. CONCLUSION: Our results show that the CD10 antigen is expressed by mesenchymal cells of normal human dermis, presumably encompassing a subset of CD34+ dermal dendrocytes and/or periadnexal fibroblasts. The CD10 antigen could be used as an adjunct to the diagnosis of some spindle-cell cutaneous tumors such as dermatofibromas and Dermatofibrosarcoma protuberans, but its value as a single marker is rather limited. The possible role of CD10 as a progression marker in melanoma is interesting and merits further study.

Antibodies, Neoplasm↗

[Treatment of Darier-Ferrand tumors of the head and neck. Retrospective analysis of 20 cases].

Dermatofibrosarcoma protuberans is an unusual cutaneous neoplasm. Because of its high propensity for local recurrence, a wide and deep excision is recommended as the only satisfactory treatment. This tumour can raise difficult problems of reconstruction to plastic surgeons particularly when located in the face. The authors present a retrospective study of twenty cases of dermatofibrosarcoma protuberans of the head and neck treated at the Saint-Louis hospital over a period of twelve years.

Adolescent↗

Gain of 17q24-qter detected by comparative genomic hybridization in malignant tumors from patients with von Recklinghausen's neurofibromatosis.

The genetic changes leading to the development of malignant peripheral nerve sheath tumors (MPNSTs) are largely unknown. The few tumors that have been investigated cytogenetically had highly complex karyotypes and no consistent rearrangements, and the attempts to pinpoint consistent DNA-level changes have met with only limited success. We used comparative genomic hybridization to analyze seven MPNSTs and one dermatofibrosarcoma protuberans from eight patients with von Recklinghausen's disease (neurofibromatosis type 1), as well as three sporadic MPNSTs. Gains and losses of DNA sequences were found in all tumors, with an average of four losses (range, 0-14) and two gains (range, 0-5) per tumor. Two striking observations were made: (a) an increase in copy number of the distal part of the long arm of chromosome 17, with the smallest region of overlap 17q24-qter, was seen in five of seven MPNSTs and in the only dermatofibrosarcoma protuberans, all of which were from patients with neurofibromatosis, whereas none of the three sporadic MPNSTs had this alteration; and (b) loss of 13q, with the smallest region of overlap 13q14-q21, was found in 6 of 10 MPNSTs. The consistent involvement of these two chromosomal regions probably reflects two different pathogenetic mechanisms for MPNSTs.

Chromosome Deletion↗

Accuracy of cytological grading of spindle-cell sarcomas.

Spindle-cell sarcomas constitute an important component of soft-tissue sarcomas, where accurate grading may be more important than histologic subtype in order to plan treatment strategies and determine prognosis. To evaluate the applicability and accuracy of grading spindle-cell sarcomas on fine-needle aspiration cytology (FNAC) smears, 54 cases of histologically documented spindle-cell sarcomas, consisting of synovial sarcomas (20 cases), neurofibrosarcomas (12 cases), leiomyosarcomas (9 cases), dermatofibrosarcoma protuberans (DFSP; 6 cases), fibrosarcomas (3 cases), hemangiopericytomas (2 cases), and spindle-cell sarcomas, unclassified (2 cases), were graded according to a three-tier system proposed earlier for FNAC smears, while the histological sections were graded using the French Federation of National Cancer Centers (FNCLCC) grading system. The cytological grading was correlated with the histological grade. There was an overall cytologic and histologic concordance in 40/54 (74%) cases, and concordance in 9/13 (69%) grade I, 19/25 (76%) grade II, and 12/16 (75%) grade III cases. Analysis of grading of individual sarcomas revealed a concordance in 92% of neurofibrosarcomas, 78% of leiomyosarcomas, 70% of synovial sarcomas, 67% of dermatofibrosarcoma protuberans, 67% of fibrosarcomas, 50% of hemangiopericytomas, and 50% of cases of malignant mesenchymal tumors, spindle-cell type unclassified. Major noncorrelation was seen in 10/54 (18.5%) cases. Minor noncorrelation was seen in 4/54 (7.4%) cases. Thus it is possible to accurately predict the grade in 74% of cases of spindle-cell sarcomas. The cytological and histological concordance was better (75%) in high-grade (grades II and III) as compared to grade I sarcomas (69%). Sampling errors due to morphologic heterogeneity in sarcomas may cause noncorrelation in a few cases.

Adult↗

p53 immunohistochemistry in malignant fibrous histiocytomas and other mesenchymal tumours.

In this study we analysed by immunohistochemistry the expression of p53 protein in 14 malignant fibrous histocytomas (MFHs), 22 other types of sarcoma (eight leiomyosarcomas, four rhabdomyosarcomas, four liposarcomas, two fibrosarcomas, two chondrosarcomas, one malignant schwannoma, and one dermatofibrosarcoma protuberans), and 25 non-malignant mesenchymal lesions (eight dermatofibromas, four cases of nodular fasciitis, three leiomyomas, three fibromatoses, two epithelioid.leiomyomas, two neurofibromas, one schwannoma, one myositis ossificans, and one giant cell tumour of tendon sheath). Four MFHs and nine other types of sarcoma (four leiomyosarcomas, two chondrosarcomas, one liposarcoma, one fibrosarcoma, and one dermatofibrosarcoma protuberans) showed nuclear positivity for p53. Of the benign soft tissue lesions, p53 positivity was observed in two fibromatoses, one nodular fasciitis, and one dermatofibroma. The number of p53-positive cells in these benign lesions was considerably smaller than that in most of the p53-positive sarcomas. The p53 positivity in MFHs and other types of sarcoma indicates that p53 gene alterations may play a part in the neoplastic transformation of these tumours. The occurrence of p53 positivity in benign mesenchymal lesions suggests that sometimes p53 protein may accumulate in cells without an associated malignancy. Because of this, p53 immunoreactivity cannot, by itself, be used as a criterion of malignancy. According to our results, p53 positivity in over 1 per cent of tumour cells in mesenchymal lesions favours malignancy.

Genes, p53↗

Immunoblot analysis of CD34 expression in histologically diverse neoplasms.

CD34 is a heavily glycosylated transmembrane protein of approximately 110 kd whose function is essentially uncharacterized. First identified in a myeloid leukemia cell line, immunohistological reactivity with anti-CD34 antibodies is also encountered in a histologically diverse subset of nonhematolymphoid neoplasms including angiosarcoma, solitary fibrous tumors, epithelioid sarcomas, spindle cell lipomas, dermatofibrosarcoma protuberans, and myofibroblastomas. Immunohistological reactivity for CD34 in hematopoietic stem cells and endothelial cells has been shown to correspond to the expression of the CD34 protein. With the exception of gastrointestinal stromal tumors, CD34 protein expression has not been investigated in other CD34 immunohistologically reactive nonhematolymphoid neoplasms. We undertook this study to examine whether the observed reactivity for anti-CD34 antibodies in apparently unrelated tumors is due to the expression of the same protein or whether shared epitopes elaborated by other proteins could account for this reactivity. Immunoblot analyses with anti-CD34 antibodies of six different CD34 immunohistologically reactive lesions show the same approximately 110-kd molecular weight protein. In addition, two cases of dermatofibrosarcoma protuberans show double bands at approximately 110 kd. Laser-capture microdissection of CD34 immunohistologically reactive epithelioid sarcoma and nonreactive epidermal cells illustrates that this reactivity is specific to tumor cells. These results show that the observed immunohistological reactivity with anti-CD34 antibodies is due to the expression of the CD34 protein and not to shared epitopes on unrelated proteins.

Adolescent↗

Immunohistochemical investigations of tumors of supposed fibroblastic-histiocytic origin.

The aim of this study was to localize alpha 1-antitrypsin, ferritin, and lysozyme by means of the indirect immunoperoxidase technique and to evaluate the significance of these antigens as markers of histiocytic differentiation in tumors of a supposed dual fibroblastic-histiocytic origin. The series comprised 31 malignant fibrous histiocytomas (MFH) of the pleomorphic, spindle cell, and myxoid types, four cutaneous fibrous histiocytomas, and four atypical fibroxanthomas, four dermatofibrosarcoma protuberans, and two osteoclastomas of bone. For comparison, 15 soft tissue sarcomas of various other types were examined. Of the MFHs of the pleomorphic type, 18 of 22 (82 per cent) were positively stained for alpha 1-antitrypsin and 12 of 22 (54 per cent) were positively stained for ferritin. Of the five MFHs of the spindle cell type, none was positively stained for alpha 1-antitrypsin, three were positive for ferritin, and one was positive for lysozyme. None of the myxoid variants (corresponding to grade I-II myxofibrosarcoma) was positively stained for either of the antigens. These results and the observations made on the cutaneous fibrous histiocytomas, atypical fibroxanthomas, dermatofibrosarcoma protuberans, and the various soft tissue sarcomas indicated that 1) alpha 1-antitrypsin is a valuable marker of histiocytic differentiation in both benign and malignant fibrous histiocytomas, 2) ferritin can be visualized in more than half of these fibroblastic-histiocytic tumors, and the presence of ferritin distinguishes the spindle cells of these tumors from fibroblasts of connective tissue and most fibrosarcomas, and 3) lysozyme, although a good marker of histiocytic differentiation in ordinary histiocytes and benign fibrous histiocytomas, is a poor marker of neoplastic histiocytes of malignant tumors. The results further support the concept that MFH is a tumor of a dual fibroblastic-histiocytic origin.

Ferritins↗

[Treatment of dermatofibroma protuberans with fixed Mohs' micrographic surgery].

INTRODUCTION: Dermatofibrosarcoma protuberans is a tumor of intermediate malignancy characterized by its aggressive local growth due to pseudopodium-like outgrowths and marked propensity to recur after surgical excision. To achieve complete cure with conventional surgery, surgical margins up to 5 cm are required, leading to wide scars. Mohs' micrographic surgery is used for the removal of certain malignant tumors, both ensuring complete excision by examination of all margins as well as minimizing normal tissue loss. However, differentiating minimal residual tumor from normal skin can be difficult on the frozen sections used in Mohs' micrographic surgery. Our aim was to develop a procedure of Mohs' micrographic surgery in conjunction with rush formalin-fixed, paraffin-embedded tangential sections. PATIENTS AND METHODS: Ten consecutive cases have been prospectively treated since 1998. Under local anesthesia, the tumor was first excised including lateral margin of 1 cm and a deep margin including the underlying muscle aponevrosis. A 2 to 3 mm thick horizontal section of the surgical bed was then removed, rush formalin-fixed, paraffin-embedded, tangentially sectioned, hematin-eosin stained, and eventually stained with an anti-CD34 monoclonal antibody. While waiting for pathology results, the surgical bed was not definitively closed. If excision was incomplete, an oriented complementary excision was performed. RESULTS: Excision was complete after the first stage in 7 patients and incomplete only deeply in 3. Lateral surgical margins were reduced to 1.3 cm in all patients, facilitating wound closure: direct suture (5 patients), controlled wound healing (3 patients) or flap coverage (2 patients). No recurrence has been observed after a mean follow-up of 26 months. DISCUSSION: The use of Mohs' micrographic surgery in conjunction with rush formalin-fixed, paraffin-embedded tangential sections reduce surgical margins in dermatofibrosarcoma protuberans. This procedure would be interesting in difficult sites, such as the genitalia, the breast, or the periarticular regions. Other cases, and longer follow-up are however necessary to validate this promising technique.

Adult↗

[Mohs' micrographic surgery: history, principles, critical analysis of its efficacy and indications].

OBJECTIVES: To systematically review the literature for studies reporting on the role of Mohs' micrographic (MMS) surgery in the treatment of skin tumors. To show how it is performed in France. DESIGN: We reviewed with a quality grid all studies indexed in MEDLINE before 2003/01/01 and published in English or French. Data were extracted by two independent reviewers. MAIN OUTCOME MEASURES: Quality of clinical studies, recurrence rates, number of patients lost to follow-up. RESULTS: No randomized study was found among the 493 references found. Studies of lower quality, on procedures similar to MMS, or previous systematic reviews were therefore selected. In tumors such as basal (BCC) or spinous (SCC) cell carcinoma, microcystic adnexal carcinoma, dermatofibrosarcoma protuberans, and Merkel cell carcinoma, MMS commonly induced lower recurrence rates than figures reported for conventional treatments and/or reduced surgical margins. Studies on melanoma were of low quality. CONCLUSIONS: Although no evidence-based guidelines could be developed, MMS should be used mainly for larger, morphea, micronodular or infiltrative-type, or recurrent BCCs located in danger zones, but also (sometimes with a slightly modified procedure) in microcystic adnexal carcinomas, dermatofibrosarcoma protuberans, Merkel cell carcinoma, and in aggressive forms of SCC. Randomized, controlled studies should be performed.

Humans↗

Congenital macronodular juvenile xanthogranuloma of the eyelid.

A large congenital eyelid mass prevented an infant from elevating his eyelid successfully. A small punch biopsy was initially interpreted as "consistent with a dermatofibrosarcoma protuberans." On the basis of this diagnosis, radical surgery was advised. Dermatofibrosarcoma protuberans, however, rarely affects children and seldom appears in the head and neck area. Because of the discrepancy between clinical and pathologic diagnoses, additional biopsies were performed, which disclosed the lesion to be a juvenile xanthogranuloma. The mass regressed after intralesional injections of corticosteroids. This is the first clinicopathologic description of congenital macronodular juvenile xanthogranuloma affecting the eyelids. The variability in the histologic composition of this large lesion may lead to a mistaken diagnosis of an aggressive condition that may provoke unnecessary radical surgery.

Adrenal Cortex Hormones↗

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↗

Mohs micrographic surgery for the treatment of spindle cell tumors of the skin.

BACKGROUND: Because of the uncommon nature of dermal spindle cell tumors, the effectiveness of various treatment modalities is difficult to assess. OBJECTIVE: Our purpose was to measure the effectiveness of treating dermatofibrosarcoma protuberans, atypical fibroxanthoma, malignant fibrous histiocytoma, and leiomyosarcoma by means of Mohs micrographic surgery (MMS). In addition, we attempted to determine whether MMS is useful in treating dermal spindle cell tumors when no definitive histopathologic diagnosis can be rendered. METHODS: In a retrospective chart review, demographic data, tumor data, treatment characteristics, recurrence, and follow-up data were tabulated. RESULTS: The recurrence rate for dermatofibrosarcoma protuberans treated by MMS was 3.0%, for atypical fibroxanthoma was 6.9%, for malignant fibrous histiocytoma was 43%, and for leiomyosarcoma was 14%. The recurrence rate for spindle cell tumors not otherwise specified was 0%. CONCLUSION: These data establish the effectiveness of MMS in the treatment of dermal spindle cell tumors, including those for which no definitive histopathologic diagnosis can be rendered.

Adolescent↗