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Abstract Although dermatofibromas are not uncommon benign dermal nodules, palms are rarely involved. Herein, a rare case of palmar dermatofibroma was described, which occurred in a patient with porokeratosis.
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Abstract Introduction Benign fibrous histiocytomas are common lesions of the skin that rarely affect the tongue. Such cases are available in the literature exclusively as case reports. Similarly, malignant fibrous histiocytoma, now classified as undifferentiated pleomorphic sarcoma, is exceedingly rare in the tongue and not fully understood. Objectives This study systematically reviews the available literature discussing the clinical and pathological features of malignant and benign fibrous histiocytomas. Data Synthesis A total of 20 cases were included in this review. Patient-level data were extracted from cases to include clinical presentation, workup, treatment, and outcome. Conclusion Benign fibrous histiocytomas are consistent in clinical and histopathologic presentation. Surgical treatment provides excellent outcome, with no recurrence in all excised cases. Malignant tumors have a more aggressive clinical and pathological presentation. Surgical treatment with possible adjuvant radiotherapy resulted in recurrence in 40% of cases (follow-up of 24 months), and death due to disease in 47% of patients (follow-up of 19 months).
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Dermatofibromas most commonly occur on limbs and rarely occur on the face. Dermatofibroma occurring on the face is associated with unusual clinicopathologic features and a more aggressive clinical course in comparison to typical cases. Additionally, the most common subtype found in previous studies was benign fibrous histiocytoma, with the keloid type being very rare (about 1% of reported cases). The aim of this study was to present our experience with a keloidal dermatofibroma of the face, which is usually missed clinically, and to discuss the treatment of a keloidal dermatofibroma in this location.
Subject(s)
Diagnosis, Differential , Extremities , Forehead , Histiocytoma, Benign Fibrous , KeloidABSTRACT
El patrón en arcoiris ha sido un tema controvertido, dado que en un principio se planteó como un patrón dermatoscópico específico asociado al diagnóstico de Sarcoma de Kaposi. Sin embargo, esta asociación ha sido cuestionada por diversos reportes que han evidenciado la presencia de este patrón dermatoscópico en otro tipo de lesiones, tales como, otros tumores vaculares, cicatriz hipertrófica, angioqueratoma, dermatitis por estasis, pseudo-Kaposi (acroangiodermatitis), melanoma, liquen plano, dermatofibroma hemosiderótico, entre otros. Se ha propuesto que este efecto se genera por la absorción, difracción y difusión de la luz polarizada y su interferencia con diferentes componentes vasculares y fibrosos de la dermis más que por algún hallazgo histopatológico específico. Presentamos el primer caso con registro fotográfico de dermatofibroma hemosiderótico con presencia de un 'patrón irisado' en la dermatoscopía.
The dermoscopic 'rainbow pattern' has been a controversial issue; it was initially proposed as a specific dermatoscopic pattern associated with Kaposi's Sarcoma. However, this has been questioned by several reports that have shown the presence of this dermoscopic pattern in a wide variety of lesions such as other vascular tumors, hypertrophic scars, angiokeratoma, stasis dermatitis, pseudo-Kaposi acroandgiodermatitis), melanoma, lichen planus, and hemosiderotic dermatofibroma, among others. It has been proposed that this effect is generated by the absorption, diffraction and diffusion of polarized light and its interference with different vascular and fibrous components of the dermis, rather than being caused by a specific histopathologic finding. We present the first photographically recorded case of hemosiderotic dermatofibroma, characterized by the presence of a rainbow pattern in dermoscopy.
Subject(s)
Humans , Male , Adult , Skin Neoplasms/pathology , Histiocytoma, Benign Fibrous/pathology , Dermoscopy , Hemosiderosis/pathology , Skin Neoplasms/diagnosis , Histiocytoma, Benign Fibrous/diagnosis , HemosiderosisABSTRACT
El fibrohistiocitoma aneurismático, es una distintiva pero pobremente reconocida variante clínico-patológica, de los fibrohistiocitomas cutáneos. Podría resultar de la proliferación de vasos sanguíneos y hemorragia, dentro de un fibrohistiocitoma cutáneo común. Se reporta el caso de un paciente de 39 años de edad, con una lesión tumoral en cara externa del codo izquierdo. Discutiremos el reconocimiento clínico, la histopatología y la diferenciación histológica con otros tumores.
Aneurysmal fibrohistiocytoma is a distinctive but poorly recognized clinical-pathological variant of cutaneous fibrohistiocytomas. It could result from the proliferation of blood vessels and hemorrhage within a common cutaneous fibrohistiocytoma. We report a case of a 39-year-old patient with a tumor lesion on the left elbow. We will discuss clinical recognition, histopathology and histological differentiation with other tumors.
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Some morphological variants of benign fibrous histiocytoma (dermatofibroma) present with distinct clinical features. In particular, atypical, aneurysmal, and cellular fibrous histiocytoma are associated with a significant risk of local recurrence. Furthermore, very rarely, cutaneous fibrous histiocytoma can lead to metastatic disease. Deep benign fibrous histiocytoma is an uncommon and poorly recognized clinical subtype that arises in subcutaneous or deep soft tissue. Deep benign fibrous histiocytoma has many histologic features in common with cellular fibrous histiocytoma. Deep benign fibrous histiocytoma recurs in approximately 20% of cases and may rarely metastasize. We report a rare case of deep benign fibrous histiocytoma with metastatic potential in a 38-year-old man who presented with a 2-year history of a recurrent hard mass on his left shoulder.
Subject(s)
Adult , Humans , Aneurysm , Histiocytoma, Benign Fibrous , Neoplasm Metastasis , Recurrence , ShoulderABSTRACT
BACKGROUND: Dermatofibrosarcoma protuberance (DFSP) must be differentiated from dermatofibroma (DF). However, especially in cases of superficial biopsy and cellular dermatofibroma, this is difficult by using histopathology alone since both are composed of neoplastic spindle cells. Although a panel of immunostains is useful, the expressions of conventional markers often overlap. A previous study showed that novel D2-40 immunostain may be useful for differentiating between DF and DFSP. OBJECTIVE: To evaluate the usefulness of D2-40 immunohistochemical staining for differentiating DFSP from DF and compare the results with other commonly used immunostains (CD34 and factor XIIIa). METHODS: Twenty-eight cases of DF and 15 cases of DFSP were selected from clinicopathologically proven cases reviewed by the Department of Dermatology at our medical center and Daegu Catholic University Medical Center. D2-40, CD34, and factor XIIIa immunohistochemical staining was performed. The immunopositivity was measured throughout the entire lesion. RESULTS: Seventeen cases (60.7%) of DF and no cases of DFSP showed immunoreactivity to D2-40 in the spindle cells. Three (10.7%) cases of DF and 13 (86.7%) cases of DFSP showed immunoreactivity to CD34 in the spindle cells. Twenty-five (89.3%) cases of DF and four (26.7%) cases of DFSP showed immunoreactivity to factor XIIIa in the spindle cells. A total of 60.7% of cases of DF were positive on D2-40 staining, 89.3% were negative on CD34 staining, and 89.3% were positive on factor XIIIa staining. All cases (100%) of DFSP were negative by D2-40 staining, 86.7% were positive by CD34 staining, and 73.3% were negative by factor XIIIa staining. CONCLUSION: D2-40 immunostaining may be useful for distinguishing between DF and DFSP since the immunoreactivity of DF was significantly higher than that of DFSP (p=0.001). However, the results of our study were not as useful as those of a previous study. Therefore, further studies are needed to address this issue.
Subject(s)
Academic Medical Centers , Biopsy , Dermatofibrosarcoma , Dermatology , Factor XIIIa , Histiocytoma, Benign FibrousABSTRACT
El fibrohistiocitoma benigno es un tumor raro en niños y más frecuente en adultos, que afecta principalmente la piel de las extremidades; su tamaño es usualmente pequeño. Según datos de la Organización Mundial de la Salud, menos del 1 % de los fibrohistiocitomas benignos son profundos. No se ha informado previamente su presencia en el peritoneo de niños. Por su gran interés, presentamos el caso de un niño de 14 meses con fibrohistiocitoma benigno peritoneal irresecable.
Benign fibrous histiocytoma is very rare in children, and more frequent in adults. It is commonly found in the skin, especially in the limbs. Usually it is small. According to the World Health Organization, less than 1 % of benign fibrous histiocytomas are found in deep tissues. Peritoneal location of this kind of tumor has not been previously reported in children. Due to its great interest, we report the case of a 14 month-old male with a peritoneal benign fibrous histiocytoma. No resection of the lesion was feasible.
O fibrohistiocitoma benigno é raro em crianças e mais frequente em adultos. Afeta mais a pele das extremidades e seu tamanho é pequeno. Segundo dados da Organização Mundial da Saúde, menos de 1 % dos fibrohistiocitomas benignos são profundos. Não se informou previamente sua presença no peritônio de crianças. Por seu grande interesse, apresentamos o caso de uma criança de 14 meses com fibrohistiocitoma benigno peritoneal irressecável.
Subject(s)
Infant , Peritoneum , Histiocytoma, Benign Fibrous , NeoplasmsABSTRACT
Backg round: Dermatofibroma sometimes clinically presents as a nodular lesion without gross skin surface change. Clinicopathologic features of this variant of dermatofi broma have not been evaluated. Aims: To assess clinicopathologic features of dermatofi broma presenting as a subcutaneous nodule. Methods: This study reviewed the clinical and histological features of 42 cases of subcutaneous dermatofibromas and compared them with 95 cases of conventional dermatofi broma. Results: Dermatofi broma without gross skin surface change was associated with a shorter pre-diagnosis duration than conventional dermatofi broma. Increase in size during the pre-diagnosis period was signifi cantly more frequent in the conventional type. In addition, these dermatofi bromas were more likely than the conventional type to occur in the head and neck region. Although tumor depth was deeper than in the conventional type, less than half of the dermatofi bromas without gross skin surface change were found histologically to be “subcutaneous” or “deep-penetrating dermatofi broma”. Subcutaneous extension was more frequent in these dermatofi bromas while focal stromal hyalinization and hemosiderin deposits were more common in the conventional type. Limitations: This study is a retrospective, single center design. Conclusion: The present study suggests that dermatofi broma without gross skin surface change is a variant type with distinct clinical and histological features that distinguish them from conventional dermatofi broma.
Subject(s)
Adult , Female , Histiocytoma, Benign Fibrous/anatomy & histology , Histiocytoma, Benign Fibrous/diagnosis , Histiocytoma, Benign Fibrous/epidemiology , Histiocytoma, Benign Fibrous/genetics , Histiocytoma, Benign Fibrous/pathology , Male , Skin/anatomy & histology , Surface PropertiesABSTRACT
BACKGROUND: Dermatofibroma (DF) comprises a heterogeneous group of mesenchymal tumors, with fibroblastic and histiocytic elements present in varying proportions. The cell of origin of DF has been investigated, but remains unclear. OBJECTIVE: The present study attempted to investigate the expression of leukocyte-specific protein 1 (LSP1), a marker of fibrocytes, in DF. Additionally, we evaluated the effectiveness of LSP1 in the differential diagnosis of DF from dermatofibrosarcoma protuberans (DFSP). METHODS: Immunohistochemical staining was performed on 20 cases of DF using antibodies against LSP1, CD68, and factor XIIIa (FXIIIa). In addition, the expression of LSP1 and FXIIIa was evaluated in 20 cases of DFSP. RESULTS: Eighteen of 20 cases (90%) of DF stained positive for LSP1, with variation in the intensity of expression. CD68 was positive in 10 cases (50%), and FXIIIa was expressed in all cases of DF. There were differences between the regional expression patterns of the three markers in individual tumors. In contrast, only 2 of 20 cases of DFSP expressed LSP1, and none of DFSP cases stained positive for FXIIIa. CONCLUSION: The LSP1-positive cells in DF could potentially be fibrocyte-like cells. FXIIIa and CD68 expression suggests that dermal dendritic cells and histiocytes are constituent cells of DF. It is known that fibrocytes, dermal dendritic cells and histiocytes are all derived from CD14+ monocytes. Therefore, we suggest that DF may originate from CD14+ monocytes. Additionally, the LSP1 immunohistochemical stain could be useful in distinguishing between DF and DFSP.
Subject(s)
Antibodies , Dermatofibrosarcoma , Diagnosis , Diagnosis, Differential , Factor XIIIa , Fibroblasts , Histiocytes , Histiocytoma, Benign Fibrous , Langerhans Cells , MonocytesABSTRACT
Objective:To investigate the clinical features, therapeutic method, and prognostic factor of fibrosarcomatous dermato-fibrosarcoma protuberans (FS-DFSP). Methods: Data of 18 FS-DFSP cases treated in the Department of General Surgery, Tianjin Huanghe Hospital between June 2004 and June 2014 were retrospectively analyzed. Research indexes included age, sex, pathogenic site, number of previous unplanned surgeries, tumor size, depth, incisal margin of the last surgical procedure, chemotherapy, relapse and metastasis, and survival time. Univariate and multivariate analyses of the research indicators were conducted in these cases. Results:All 18 patients underwent surgery, and the incisal margins were R0 in 17 cases and R1 in 1 case, with local recurrence in 2 of the 18 cas-es. The patient with R1 incisal margin underwent postoperative radiotherapy. Among all the patients, 12 with tumor base depth and/or largest tumor diameter of>5 cm accepted the chemotherapy of mesna, adriamycin, ifosfamide, and dacarbazine, also known as MAID regimen, after surgery. No progression of disease occurred during chemotherapy. After chemotherapy, pulmonary metastasis occurred in 3 of the 12 cases, and pulmonary metastasis with local recurrence existed in 1 case. Two of the 3 patients with pulmonary metastasis were treated with imatinib mesylate, and the therapeutic effect stabilized the disease. Two-year survival rate was 93%, and 5-year sur-vival rate was 79%in total patients. The results of mono-factorial analysis indicated that clinical factors, such as age, sex, pathogenic site, tumor size, depth, recurrence, incisal margin of surgical operation, and chemotherapy, were unrelated to the overall survival (OS) time. The number of previous unplanned surgeries and metastasis are related to OS. The results of multiple factor analysis showed that none of the clinical factors were independent prognostic factors for OS. Conclusion:A thorough removal of tumor can reduce the recur-rence rate, which is the key point in FS-DFSP treatment. Recurrence and metastasis of tumor are significant factors affecting prognosis.
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No abstract available.
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Humans , Histiocytoma, Benign Fibrous , Lymphoma, Large-Cell, Anaplastic , Lymphoma, Primary Cutaneous Anaplastic Large CellABSTRACT
Comunicamos el caso de una paciente de 48 años, con una infrecuente variante clínica de dermatofibroma, el gigante, siendo además polipoideo y localizado en palma. Enfatizamos el carácter inflamatorio del dermatofibroma y la importancia de conocer las etapas cronológicas de la lesión.
A 48-years old woman with an unusual clinical variant of dermatofibroma, the giant type is reported, the clinical aspect showed a polipoid lesion, firm and asymptomatic. The inflammatory nature of the dermatofibroma and the faces of the live of the lesions are emphasized.
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BACKGROUND: Dermoscopic features may be useful in verifying the clinical diagnosis and increasing the diagnostic accuracy of dermatofibroma. There have been no systematic analyses of the dermoscopic features of dermatofibroma in Koreans or studies regarding the correlation between dermoscopic features and histologic subtypes. OBJECTIVE: To investigate the dermoscopic features of dermatofibroma in Koreans and to explore the relationship between the dermoscopic features and histologic subtypes of dermatofibroma. METHODS: The study included 83 lesions from 61 patients with dermatofibroma. Dermoscopic examination was performed via hand-held polarized dermoscopy at 10x magnification. Images were documented using a digital camera with a 3-fold optical zoom. RESULTS: The main dermoscopic structures were pigment network (63.9%), white scar-like patch (53.0%), and homogeneous area (50.6%). The frequency of homogeneous area (50.6%), scale (50.6%), ring-like structure (36.1%), exophytic papillary structure (7.2%), and telangiectasia (6.0%) was significantly higher, and globule-like structure (20.5%) and erythema (8.4%) was significantly lower in Koreans than in Caucasians (p<0.05). Eleven dermoscopic patterns including a peripheral delicate pigment network and central white scar-like patch (28.9%) and total delicate pigment network (15.7%) were observed and there was no significant difference compared to Caucasians. Seven histologic types were observed; however, the correlation between dermoscopic features and histologic subtypes was not significant. CONCLUSION: The most common pattern of dermatofibroma was pigment network and central white scar-like patch in both Koreans and Caucasians. A correlation between dermoscopic features and histologic subtypes in dermatofibroma was not found. These results may be helpful in clinically diagnosing dermatofibroma, especially in Koreans.
Subject(s)
Humans , Dermoscopy , Diagnosis , Erythema , Histiocytoma, Benign Fibrous , TelangiectasisABSTRACT
Dermatofibrosarcoma Protuberans (DFS-P) es una neoplasia de partes blandas y cutánea poco frecuente, de crecimiento superficial con tendencia a recurrir localmente y bajo potencial metastásico. Ocurre en personas de edad media, preferentemente en tronco y zonas proximales de extremidades. Requiere un alto grado de sospecha clínica, ya que inicialmente puede ser similar a una cicatriz hipertrófica o a un dermatofibroma. A continuación se realiza una revisión de la literatura sobre este tumor poco frecuente, con énfasis en las características clínicas y las opciones terapéuticas actualizadas según las guías 2010 de la National Comprehensive Cancer Network(NCCN).
Dermatofibrosarcoma protuberans (DFS-P) is a soft cutaneous neoplasm, pretty rare, with superficial growing, that tends to local recurrence. Usually it present at medium age, in trunk and the beginning of extremities. It is very difficult to diagnose at the beginning because its similarity with hypertrophic scar or dermatofibroma. We present a review in the literature of DFS-P, with emphasizing the early clinical manifestations and the different therapeutic agents recently approved by National Comprehensive Cancer Network (NCCN).
Subject(s)
Humans , Dermatofibrosarcoma/diagnosis , Dermatofibrosarcoma/pathology , Skin Neoplasms/diagnosis , Skin Neoplasms/pathology , Clinical Evolution , Dermatofibrosarcoma/epidemiology , Dermatofibrosarcoma/therapy , Immunohistochemistry , Skin Neoplasms/epidemiology , Skin Neoplasms/therapyABSTRACT
Sarcoidosis is a multisystem granulomatous disease of unknown cause. The lung is the most commonly affected organ, but the skin is frequently involved. Specific sardoidal lesions are most often found on the head and neck area. The most common specific presentation is multiple papular lesions. The present case shows solitary cutaneous sarcoidosis resembling dermatofibroma, located on the thigh. Dermoscopy can help in the differential diagnosis of cutaneous sarcoidosis. Dermoscopic findings are orange globular-like structures and linear vessels, and areas of scar-like depigmentation. Histopathologic findings show non-caseating granulomas with sparse inflammatory cells. We report a case of cutaneous sarcoidosis resembling dermatofibroma, which was diagnosed using dermoscopy.
Subject(s)
Citrus sinensis , Dermoscopy , Diagnosis, Differential , Granuloma , Head , Histiocytoma, Benign Fibrous , Lung , Neck , Sarcoidosis , Skin , ThighABSTRACT
PURPOSE: Dermatofibroma is a common benign dermal tumor characterized by a proliferation of fibroblast-like spindle cells. It is commonly localized on the skin of extremities and presents as a slow growing solitary nodule. To our knowledge, the occurrence of dermatofibroma in the oral cavity is rare. Herein, we report a rare case of dermatofibroma on the lower lip. METHODS: A 60-year-old woman presented with a slow growing mass that measured 1 x 0.8 cm in diameter on the lower lip. The mass was surgically excised with clear margins. RESULTS: Histologically, the mass was characterized by a nodular tumor composed of collagen bundles, fibroblasts, and histiocytes, which were findings consistent with dermatofibroma. The postoperative course was uneventful without any complications. CONCLUSION: When evaluating nodular tumors of the oral area, dermatofibroma should be considered in the differential diagnosis.