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A case of pigmented storiform neurofibroma (PSN) is presented. The lesion was present in the shoulder of a 7-year-old black girl and recurred after 10 years. The characteristic storiform pattern and melanin production are the relevant features for histologic diagnosis. The histogenesis and taxonomic position of this lesion is discussed with emphasis on its relationship with dermatofibrosarcoma protuberans (DFSP) and cellular blue nervus.
Nucleolar organizer regions (NORs) are loops of DNA which are present within the nucleoli of cells that possess ribosomal RNA (rRNA) genes. NORs are associated with proteins which can be visualized by a simple silver staining technique. As the number of NORs appears to reflect cell and nuclear activity its determination in benign, intermediate and malignant conditions could be of help in their differential diagnosis. In this study, we investigated 35 fibrohistiocytic tumors of the skin of benign, intermediate and malignant potential from 32 patients: 15 fibrous histiocytomas (FH), 5 FH with atypia (AFH), 7 atypical fibroxanthomas (AFX), 5 dermatofibrosarcoma protuberans (DFSP) and 3 malignant FH (MFH). A one-step silver technique was used on formalin or Bouin fixed specimens. Ag-NOR counts from benign conditions (FH, AFH) significantly differed from that of their intermediate-risk (AFX, DFSP) and malignant (MFH) counterparts. Furthermore, in 2 cases (one AFX; one MFH) which twice recurred, Ag-NOR counts steadily increased with time. However, a distinction could not be achieved between AFX and MFH. Although Ag-NOR is a simple and valuable technique, it does not allow a definite distinction between malignant and intermediate processes, at least as far as fibrohistiocytic tumors of the skin are concerned.
The histogenetic relationship amongst various dendritic cells of the dermis which may express markers including factor XIIIa (FXIIIa) or CD34 remains unclear. In this study we utilized a sensitive indirect immunoperoxidase staining technique to identify CD34 and FXIIIa, as well the monocyte/macrophage markers KP-1 and MAC 387 expression in a variety of cutaneous dermal tumors of mesenchymal origin to see if differential expression of CD34 vs FXIIIa exists. Tumors studied included dermatofibroma (DF) (N = 10), keloid (N = 9), atypical fibroxanthoma (AFX) (N = 3), and dermatofibrosarcoma protuberans (DFSP) (N = 7). DF were all composed of FXIIIa+ spindle-shaped and stellate tumor cells (mean score = 4.9 or > or = 75% FXIIIa+) as previously reported, but these cells rarely (< 10%) expressed CD34. Six of 7 DFSP were found to be > 75% CD34+ and FXIIIa negative, while one DFSP was negative for both CD34 and FXIIIa. In all DFSP, there were trapped FXIIIa+ cells which were distinct from the spindle-shaped tumor cells. AFX showed sparse populations of FXIIIa+ cells in the stroma (mean score = 1.33 or 10-25% positive), which were distinct from the atypical giant cells characteristic of these tumors. Keloid similarly contained trapped FXIIIa+ cells (mean score = 0.44 or < 5% positive) that were distinct from the spindle-shaped fibroblasts of the tumor mass. Dendritic and spindle-shaped cells within these tumors were consistently both KP-1 and Mac-387 negative, while all lesions studied were characterized by scattered round, histiocytic cells which were KP-1+ and/or Mac-387+ irrespective of tumor cell type.(ABSTRACT TRUNCATED AT 250 WORDS)
The human hematopoietic progenitor cell antigen is known as CD34. This antigen is present on normal bone marrow progenitor cells and vascular endothelial cells. We used the monoclonal antibody anti-CD34 and immunoperoxidase staining techniques to evaluate the expression of CD34 in benign and malignant vascular and spindle cell tumors. All of the 42 vascular lesions, except two of three lesions of intravascular papillary endothelial hyperplasia, demonstrated diffuse membraneous staining of moderate to strong intensity of their endothelial cells. Also, normal placentas (five) showed similar staining. All neurofibromas (12), three of five neuromas, and one of four neurilemmomas revealed moderate to strong, diffuse, membraneous staining. Five of eight piloleiomyomas, two of seven angioleiomyomas, and one of five uterine leiomyomas showed focal to diffuse, and weak to moderate, membraneous staining in the smooth muscle component. Six dermatofibrosarcoma protuberans were studied: generalized, strongly positive membraneous staining was present in four. All specimens showed staining of the normal endothelial cells and the cells surrounding the hair follicles (bulge area), sebaceous glands, and eccrine glands. No staining was demonstrated in any of the following fibrohistiocytic tumors: atypical fibroxanthomas (two), fibrous dermatofibromas (23), giant cell tumor of tendon sheath (one), and hemosiderotic dermatofibromas (18). Melanocytic tumors [Spitz nevi (three) and spindle cell superficial spreading malignant melanoma (one)], Merkel cell carcinomas (six), and spindle cell squamous cell carcinomas (two) did not stain with anti-CD34. Glomus tumors (two) and a hemangiopericytoma were also negative except for their vascular channels.(ABSTRACT TRUNCATED AT 250 WORDS)
Dermatomyofibroma is a recently described, benign acquired cutaneous proliferation of fibroblasts and myofibroblasts. We report 2 additional cases, both in young women. The lesions measured 0.5 and 1.0 cm and were located on the breast and upper back. Follow-up at 8 months and 3 years revealed no evidence of recurrence. Both lesions exhibited a uniform population of spindle cells forming intersecting long fascicles with a tendency to horizontal orientation in the reticular dermis. Cytologic atypia was absent and mitotic figures were minimal or absent. Collagen was present as thin fibers separating individual cells and as thicker bundles between the fascicles. A fine reticulin network surrounded the spindle cells. Elastic fibers were mostly preserved and appeared thicker or even increased in number. The spindle cells were immunoreactive for vimentin and muscle actin and negative for desmin, S-100 protein, factor XIIIa, and CD34. Electron microscopic studies confirmed the presence of myofibroblasts. The differential diagnosis was made with cellular and fibrous dermatofibroma, piloleiomyoma, hypertrophic scar, and dermatofibrosarcoma protuberans, both classic and plaque type. The data presented supports the histologic distinction of dermatomyofibroma from other fibroblastic proliferations of the skin.
Spindle cell lipoma (SCL) is an uncommon soft tissue neoplasm that may provide diagnostic difficulty to the histopathologist. Four retrospectively identified SCLs were evaluated immunohistochemically with a broad panel of antibodies (CD34, factor XIIIa, S-100, actin and factor VIII). All four SCLs were strongly CD34 positive (95 to 100% of cells) and focally factor XIIIa positive (average 16% of cells). Tumor cells were S-100, actin and factor VIII negative. Dermatofibrosarcoma protuberans, neurofibroma, schwannoma and angiolipoma are CD34 positive tumors that have some similar histologic features to SCL and may be considered in the differential diagnosis. Differentiation is possible using a broad panel of immunostains and routine diagnostic criteria.
A commonly recognized feature of chronic radiation dermatitis is the presence of mesenchymal cells with large atypical nuclei known as radiation fibroblasts. Little is known about their lineage or potential for neoplastic transformation. To investigate these properties, we examined 16 biopsy specimens in which radiation fibroblasts were present with antisera to mesenchymal determinants (FXIIIa, CD34, HHF-35), a proliferation marker (Ki-67), and a tumor-suppressor protein that is overexpressed in many cancers (p53). Radiation fibroblasts were largely negative for the markers of lineage that we employed - only 2 of 16 specimens showed strong expression of FXIIIa, with weak expression in another case. Scattered radiation fibroblasts expressed CD34 in one case. HHF-35 (muscle specific actin) stained small, dendritic cells in the superficial dermis, but not radiation fibroblasts. P53 was not detected within radiation fibroblasts in any of our cases, but was overexpressed by endothelial cells in 2 cases. Ki-67 stained rare endothelial and interstitial cells but not radiation fibroblasts. Radiation fibroblasts are immunophenotypically distinct from dermal dendrocytes and myofibroblasts. They appear to be non-cycling cells, and do not express high levels of p53 despite their marked nuclear atypia. Their phenotype argues against their possible role as progenitors of atypical fibroxanthoma (AFX) and dermatofibrosarcoma protuberans (DFSP) which are associated with ionizing radiation-induced skin damage.
The fibrohistiocytic tumors of the skin are a heterogeneous group of dermal/subcutaneous mesenchymal neoplasms which show fibroblastic, myofibroblastic and histiocytic (macrophage-like) differentiation, often one beside the other in the same tumor. "Fibrohistiocytic" means in this context the morphologic similarity of the cells with fibroblasts and histiocytes. The WHO classification of 2005 includes the following entities as fibrohistiocytic tumors of the skin: BENIGN: 1. Fibrous histiocytoma (FH)/(synonymous: Dermatofibroma. Variants of FH: 1a. cellular fibrous histiocytoma, 1b. atypical (pseudosarcomatous) fibrous histiocytoma, 1c. aneurysmatic fibrous histiocytoma, 1d. epithelioid fibrous histiocytoma; 2. dermatomyofibroma; 3. (juvenile) xanthogranuloma. INTERMEDIATE: 4. plexiform fibrohistiocytic tumor; 5. dermatofibrosarcoma protuberans; 6. atypical Fibroxanthoma. MALIGNANT: 7. malignant fibrous histiocytoma. All these entities are reviewed in this paper with particular attention devoted to differential diagnostic considerations.
BACKGROUND: Allograft recipients are at increased risk for skin cancer. The incidence of cutaneous squamous cell carcinoma is 50-250 times higher than in the age-matched control population, and basal cell carcinoma is about 10 times more frequent. The incidence of Kaposi's sarcoma is increased 400 to 500 times over that in a control population of the same ethnic origin. However, the incidence of other types of cutaneous sarcoma in organ allograft recipients is largely unknown. CLINICAL OBSERVATION: Within a 2-year-period, we observed 2 patients with cutaneous malignant fibrous histiocytoma and 1 patient with atypical fibroxanthoma among a cohort of 642 renal transplant recipients. For comparison, the incidence for dermatofibrosarcoma protuberans which is the commonest type of cutaneous sarcoma, is 0.45/100,000 persons/year in the non-immunocompromised population. Our observation represents an incidence of 156/100,000/ year (95% confidence interval Cl 28-489/100,000/year) for cutaneous malignant fibrous histiocytoma and of 78/100,000/year (95% CI 4-368/ 100,000/year) for atypical fibroxanthoma. CONCLUSION: To our knowledge, this is the first report on an elevated incidence of cutaneous malignant fibrous histiocytoma and of atypical fibroxanthoma in renal transplant recipients. Future cohort studies on malignancies in organ allograft recipients should aim at defining this risk more exactly.
BACKGROUND: Dermatofibroma (DF) is a common benign histiocytic tumor, which has several clinicopathological variants. Myxoid DF is one of these variants, which is characterized by a stromal mucin deposition. CD44 is a polymorphic transmembrane glycoprotein and the principal cell surface receptor of hyaluronate (HA), the major component of the extracellular matrix. In a recent study, we have observed an abnormal accumulation of HA in the superficial dermis of transgenic mice with a keratinocyte-specific CD44 expression defect. We have also shown that HA was accumulated in large amounts in the superficial dermis of lichen sclerosus et atrophicus (LSA) lesions and that the epidermal CD44 expression of LSA skin was significantly decreased or lost. In an another study, we have suggested that a decrease in CD44 expression in follicular epithelial proliferations might be correlated with an abnormal HA accumulation in perifollicular solitary cutaneous myxoma. Recently we have also demonstrated that classical DF lesions displayed a strong CD44 expression in tumor cells and a weak HA positivity in tumor stroma whereas CD44 expression was significantly reduced or absent in dermatofibrosarcoma lesions and the tumor stroma showed strong HA staining. OBJECTIVE AND METHODS: Here we present 3 cases of myxoid DF, in which we explored the nature of the mucinous material in myxoid stroma by colloidal iron and hyaluronic acid binding protein stainings, as well as the expression of CD44 in the tumor cells by immunohistochemistry. RESULTS: We show that HA is accumulated in the stroma of all myxoid DF lesions with a significant decrease in CD44 expression in the tumor cells. CONCLUSION: Our results suggest that a decrease in CD44 expression in the tumor cells may result in stromal myxoid changes characterized by an abnormal HA accumulation in myxoid DF.
This is the first case report of atypical fibroxanthoma in the otolaryngology literature. Atypical fibroxanthoma is a rare fibrohistiocytic tumor that occurs on actinically damaged skin. The typical presentation is a nodular lesion in sun-exposed areas of the head and neck in an elderly patient. Differentiating atypical fibroxanthoma from other soft tissue sarcomas, such as dermatofibrosarcoma protuberans and malignant fibrous histiocytoma, can be difficult. Treatment should be wide local excision with a 1-cm margin. In cosmetically important regions in which tissue preservation is important, Mohs' surgery should be considered.
Skin cancer from 775 patients in the savannah showed a preponderance of squamous cell carcinoma (SCC) of the leg related to neglected, poorly managed and chronic ulcers or scars from burns or injuries. SCC of the head and neck had no predisposing factor. Malignant melanoma overwhelmingly affected the feet, dermatofibrosarcoma affected the trunk and Kaposi sarcoma affected the limbs. Basal cell carcinoma (BCC) comprised only 2% of all skin cancers. The 18 albino patients had a higher frequency of both SCC and BCC mostly on the head and neck. Excisional surgery was generally effective, the wounds being closed primarily or by means of flaps and skin grafts. However, 98 patients required amputation below the knee for tibial involvement by SCC. The prognosis and health care costs of skin cancer will only be improved if burns and injuries are adequately treated.
Giant congenital melanocytic nevus (CMN) might be complicated by the development of several tumor types, mainly melanoma and rhabdomyosarcoma, but also poorly differentiated neoplasms. Striking polypoid exophytic masses occasionally result from neuroectodermal and neuromesenchymal hamartomatous overgrowths, which lends support to the concept of pluripotential melanoblastomatous and neurocristic origin of these lesions. The present report describes what appears to be a unique example of dermal hamartomatous polypoid CD34(+) fibrogenic proliferation devoid of melanin, namely a dendrocytoma, surrounded by the melanocytes from the nevus, located in the skin of the scrotum against a background of giant CMN. The differential diagnosis included dermatofibrosarcoma protuberans, giant-cell fibroblastoma, angiofibroma, Bednar tumor, other types of dermal dendrocytic hamartoma, and neurocristic cutaneous hamartoma. This case lends support to the proposed neurocristic origin of dermal fibrogenic cells.
Between June 1975 and April 1981, 61 of the 177 eligible patients whose nonosseous sarcomas of extremity or trunk origin had been completely excised primarily or after local recurrences agreed to participate in a randomized study of adjuvant chemotherapy. Dermatofibrosarcoma, lymphomas, myeloma, Kaposi's sarcoma, and embryonal rhabdomyosarcoma were excluded as were patients with significant second primary cancers and those who received either preoperative or postoperative radiation therapy. After stratification by anatomic status of disease, site of origin, and histologic grade, a random one half of the 61 participants began alternating courses of vincristine/cyclophosphamide/dactinomycin, and vincristine/doxorubicin/dacarbazine at six-week intervals for one year. The control group was evaluated at six-week intervals without adjuvant chemotherapy, but these patients were offered this chemotherapy later if they had progressive disease excised. Although 30% of the 61 patients experienced local recurrence of disease within the first five years after randomization, and only 54% were continuously disease free for five or more years, 82% were surviving at five years (Kaplan-Meier calculations) with a median follow-up of 64.3 months. Partial suppression of distant metastasis by adjuvant chemotherapy was apparent in the overall study, in the extremity tumor category, and in the subgroup of patients who had received limb-sparing surgery; however, no survival advantage for chemotherapy-treated patients was demonstrated. The 30 adjuvant chemotherapy-treated patients received a total of three thoracotomies as compared with 17 salvage thoracotomies for the 31 control patients; however, salvage surgery for local recurrences has been similar in the two groups. Recent improvement in the survival of patients with soft-tissue sarcomas is not necessarily a result of adjuvant chemotherapy or radiation therapy.
OBJECTIVE AND IMPORTANCE: Melanocytic colonization of nonpigmented extracranial tumors has been reported in adenocarcinomas, squamous cell carcinomas, skin appendage tumors, and dermatofibrosarcoma protuberans. To our knowledge, melanocytic colonization of a meningioma has not previously been described. CLINICAL PRESENTATION: We report an unusual case of a 70-year-old African-American woman who presented with a large frontoparietal meningioma that extended through the calvarium. INTERVENTION: Craniotomy with gross total resection of the tumor was performed. Histochemistry, immunocytochemistry, ultrastructural analysis, and molecular genetic study via fluorescence in situ hybridization confirmed melanocytic colonization of a meningothelial meningioma. CONCLUSION: With the inclusion of meningothelial meningioma, the spectrum of tumors affected by melanocytic colonization continues to expand.
Solitary fibrous tumors (SFTs) represent a distinct neoplasm that should be included in the differential diagnosis of spindle-cell neoplasms of the soft tissue. Basic fibroblast growth factor (bFGF or FGF-2) is a mitogenic and angiogenic polypeptide produced by diverse cell types, including the cells derived from normal tissue and neoplastic lesions. In this study, the expression of bFGF, vimentin, CD 34, c-kit (or CD 117), desmin, S-100 protein, and alpha-smooth muscle actin (alpha-SMA) in SFTs, hemangiopericytomas (HPC), gastrointestinal stromal tumors (GIST), and dermatofibrosarcoma protuberans (DFSP) were evaluated to assess their usefulness in the differential diagnosis of these lesions. The expression of bFGF mRNA was also examined in SFTs by in situ hybridization (ISH) using a digoxigenin-labeled bFGF oligonucleotide probe. All the SFTs, GISTs and DFSPs exhibited strong and diffuse immunoreactivity for CD34 and vimentin, and were completely negative for desmin, S-100 protein and alpha-SMA. The HPCs were positive for vimentin, but negative for CD34. In all the SFTs, strong and diffuse nuclear immunostaining was observed with bFGF antibody, contrasting with the negative staining observed in the majority of the HPCs, GISTs, and DFSPs. The bFGF mRNA was also expressed in the SFT cells. The constitutive expression of the bFGF in the SFT widens the spectrum of available markers for these tumors, providing a useful addition to their differential diagnosis in difficult cases, and contributing to the understanding of their histogenesis and molecular pathogenesis.
A review of 118 archival cases originally diagnosed as fibrosarcoma of the head and neck is presented. Sites of involvement were the sinonasal region, upper aerodigestive tract, skin, and soft tissues. Review of the pathologic specimens applying strict histopathologic criteria including immunohistochemical techniques reclassified many of the tumors as malignant fibrous histiocytoma, monophasic synovial sarcoma-like neoplasm, or dermatofibrosarcoma protuberans. Improved clinical outcome correlated significantly with lesser tumor size, lower histologic grade, and negative surgical margins. Location in the skin or soft tissue tended to convey improved survival, although not to a level of statistical significance.
We performed immunohistochemical analysis for KIT in 365 soft tissue sarcomas. Most tumors evaluated were completely negative for KIT, including all cases of leiomyosarcoma, rhabdomyosarcoma, myxofibrosarcoma, liposarcoma, solitary fibrous tumor, synovial sarcoma, dermatofibrosarcoma protuberans, schwannoma, malignant peripheral nerve sheath tumor, clear cell sarcoma, low-grade endometrial stromal sarcoma, and follicular dendritic cell sarcoma. Tumors showing occasional immunoreactivity for KIT included extraskeletal myxoid chondrosarcoma (2/20), Ewing sarcoma/malignant primitive peripheral neuroectodermal tumor (4/20), melanotic schwannoma (3/5), metastatic melanoma (4/20), and angiosarcoma (5/20). In most cases, staining for KIT was focal. Rare tumor cells showing KIT positivity were identified in a small number of other tumors. This study demonstrates very limited expression of KIT in soft tissue tumors other than gastrointestinal stromal tumors and underscores the discriminatory value of KIT immunohistochemical analysis for differential diagnosis. As some of these findings differ markedly from previous reports, it is evident again that variations in immunohistochemical technique can lead to major discrepancies in positive staining. Since treatment eligibility for selective tyrosine kinase inhibitors such as STI571 hinges on positive immunostaining, standardization and reproducibility of meaningful results are critically important.