No abstract available.
Diagnosis, Differential ; Histiocytoma, Benign Fibrous ; Xanthogranuloma, Juvenile

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.
Diagnosis, Differential ; Extremities ; Forehead* ; Histiocytoma, Benign Fibrous* ; Keloid*

BACKGROUND: Angiokeratomas are vascular malformations that usually appear as multiple or solitary cutaneous papules, nodules, or plaques. Several clinical variants of angiokeratoma exist. The differential diagnosis of angiokeratoma can be difficult and some cases that are clinically suggestive of angiokeratoma are found to be caused by other diseases following skin biopsy. OBJECTIVE: The purpose of this study was to examine the diagnostic yield following analysis of clinically diagnosed angiokeratomas, which presented as multiple or solitary cutaneous papules, nodules, or plaques. METHODS: We retrospectively reviewed 36 patients who had visited the department of dermatology between January 2004 and December 2013, and who, following biopsy, had a clinical diagnosis of angiokeratoma or a differential diagnosis of angiokeratoma. We compared the clinical and histopathologic diagnoses, and analyzed the rate of concurrence and clinical features, including age, sex, location, and duration. RESULTS: The angiokeratoma patients accounted for 61.1% of all new patients and their mean age was 32.2 years. The most common subtype was solitary angiokeratoma (11 cases, 50%). Histopathologic analysis showed that 38.9% of patients had a different type of disease and their mean age was 31.4 years. The most common causes of disease for this latter group were pyogenic granuloma (21.4%) and hemangioma (21.4%), followed by calcinosis cutis, dermatofibroma, neurofibroma, pilomatricoma, verruca vulgaris, and herpes viral infection. CONCLUSION: Lesions suggestive of angiokeratoma need further examination and a biopsy is useful to determine the correct differential diagnosis between angiokeratoma and other diseases, to avoid erroneous management.
Angiokeratoma* ; Biopsy ; Calcinosis ; Dermatology ; Diagnosis ; Diagnosis, Differential ; Granuloma, Pyogenic ; Hemangioma ; Histiocytoma, Benign Fibrous ; Humans ; Neurofibroma ; Pilomatrixoma ; Retrospective Studies ; Skin ; Vascular Malformations ; Warts

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.
Antibodies ; Dermatofibrosarcoma ; Diagnosis* ; Diagnosis, Differential ; Factor XIIIa ; Fibroblasts ; Histiocytes ; Histiocytoma, Benign Fibrous* ; Langerhans Cells ; Monocytes

OBJECTIVETo study the clinical and pathologic features of primary cardiac inflammatory myofibroblastic tumor.

METHODSA total of 4 patients with primary cardiac inflammatory myofibroblastic tumor were encountered during the period from 1993 to 2013 in National Center for Cardiovascular Disease. The clinical features, imaging findings and outcomes of the 4 patients were evaluated. ALK protein expression and ALK gene status were studied using the archival tumor tissues.

RESULTSThere were 1 female and 3 male patients. The age of patients ranged from 5 months to 30 years (mean = 16 years). The tumor was located in right ventricle (n = 2), right atrium (n = 1) or pericardium (n = 1). Histologic patterns included 2 cases of fibrous histiocytoma type, 1 case of granulomatous type and 1 case of sclerosing type. Immunohistochemical study showed that 2 cases expressed ALK protein. Fluorescence in-situ hybridization however did not reveal any ALK gene rearrangement.

CONCLUSIONSInflammatory myofibroblastic tumor of the heart is rarely encountered and easily misdiagnosed. It carries distinctive clinical and pathologic features. ALK protein expression is helpful in arriving at the correct diagnosis.

Adolescent ; Adult ; Biomarkers, Tumor ; genetics ; metabolism ; Child ; Diagnosis, Differential ; Female ; Granuloma, Plasma Cell ; enzymology ; pathology ; Heart Neoplasms ; enzymology ; pathology ; Histiocytoma, Benign Fibrous ; enzymology ; pathology ; Humans ; Immunohistochemistry ; In Situ Hybridization, Fluorescence ; Infant ; Male ; Receptor Protein-Tyrosine Kinases ; genetics ; metabolism

Alveolar adenoma is a very rare benign intraparenchymal lung tumor originating from type II pneumocytes. It can be mistaken for other benign tumors or lung cancer in radiological images. It is especially difficult to distinguish alveolar adenoma from sclerosing hemangioma. A small aspiration biopsy specimen, such as with percutaneous needle aspiration, is insufficient for a pathological diagnosis. Surgical resection is the only method by which a pathological diagnosis can be made and the disease treated. An alveolar adenoma presenting as multiple nodules is very rare and has to our knowledge not been reported in Korea previously. Here, we report a case of alveolar adenoma in multiple nodules in a 57-year-old female and review the literature.
Adenoma* ; Biopsy, Needle ; Diagnosis ; Female ; Histiocytoma, Benign Fibrous ; Humans ; Korea ; Lung Neoplasms ; Lung* ; Middle Aged ; Needles ; Pneumocytes

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.
Dermoscopy ; Diagnosis ; Erythema ; Histiocytoma, Benign Fibrous* ; Humans ; Telangiectasis

Actins ; metabolism ; Angiomyoma ; metabolism ; pathology ; Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Forearm ; Hemangioma ; metabolism ; pathology ; surgery ; Histiocytoma, Benign Fibrous ; metabolism ; pathology ; Humans ; Middle Aged ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Skin Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

OBJECTIVETo study the clinicopathologic characteristics of atypical fibrous histiocytoma (AFH), with emphasis on differential diagnosis.

METHODSThe clinical and pathologic features were reviewed in 24 cases of AFH (from 2007 to 2012). The follow-up data were analyzed. Immunohistochemical study using EnVision method was carried out.

RESULTSThere were 10 males and 14 females with age at presentation ranging from 8 to 67 years (mean = 41 years and median = 39 years). The tumor occurred in the extremities (number = 14), trunk (number = 8) or head and neck region (number = 2). Apart from one case, all were located in the dermis. The clinical appearance was similar to those of classic fibrous histiocytoma. Histologically, the tumor was characterized by various number of hyperchromatic bizarre cells scattered in the background. Mitotic figures including atypical ones were noted, especially in the more cellular areas. Immunohistochemical study showed that the tumor cells expressed vimentin. Focal positivity for alpha-smooth muscle actin was demonstrated in some cases. Follow-up information was available in 14 cases. Three of them suffered local recurrence. None of these cases had distant metastasis.

CONCLUSIONSAtypical fibrous histiocytoma represents a pleomorphic variant of fibrous histiocytoma. Although the tumor exhibits worrisome features, it usually pursuits a relatively benign course. Nevertheless, rare cases may recur, especially after incomplete excision. AFH is sometimes mistaken as atypical fibroxanthoma. A distinction between the two entities is warranted as they represent two different entities.

Actins ; metabolism ; Adolescent ; Adult ; Aged ; Back ; pathology ; Child ; Diagnosis, Differential ; Extremities ; pathology ; Female ; Follow-Up Studies ; Histiocytoma, Benign Fibrous ; metabolism ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Retrospective Studies ; Skin Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism ; Xanthomatosis ; pathology ; Young Adult

OBJECTIVETo study the clinicopathologic characteristics of cellular fibrous histiocytoma (CFH) with emphasis on diagnosis and differential diagnosis.

METHODSClinical and pathologic features were reviewed in 27 cases of CFH (encountered during the period from 2008 to 2012) along with outcome analysis. Immunophenotyping was performed with EnVision method.

RESULTSThe patients included 13 males and 14 females. The age at presentation ranged from 15 to 61 years (mean, 34 years; median, 32 years). The tumor occurred in the extremities (n = 14), head and neck (n = 7), and trunk (n = 6). Histologically, the tumors were located in the dermis. Some cases showed wedge like extension into the subcutaneous adipose tissue. On high power, they consisted of dense fibroblasts and myofibroblasts. Other cell components such as psammoma-like histiocytes, hemosiderin-containing macrophages or touton-type giant cells were rare. The spindled tumor cells were arranged mostly in intersecting fascicles. Focal storiform architecture was not uncommon. In addition, a few cases showed prominent hemangiopericytoma-like pattern. There was no prominent cellular atypia but increased mitotic figures were not difficult to find. Two cases exhibited necrosis. By immunohistochemistry, the tumor cells showed variable expression of alpha smooth muscle actin. CD34 positive cells were present in some cases, but were distributed mostly in the periphery or bottom of the lesions. They were all negative for desmin, h-caldesmon, S-100 protein and cytokeratin. Follow-up in 19 cases revealed local recurrences in 5 cases and bilateral pulmonary metastases in 1 case after repeated recurrences.

CONCLUSIONSCFH is a cellular form of benign fibrous histiocytoma which has a risk for local recurrence after incomplete excision. Distant metastasis can occur in rare examples. However, there were no morphological parameters predicting the risk of recurrence or metastasis. Increased awareness of the clinocopathological features and immunophenotypes of CFH is helpful in avoiding misdiagnosing the disease as malignant tumors, especially dermatofibrosarcoma protuberans.

Actins ; metabolism ; Adolescent ; Adult ; Antigens, CD34 ; metabolism ; Dermatofibrosarcoma ; metabolism ; pathology ; Diagnosis, Differential ; Extremities ; Female ; Follow-Up Studies ; Head and Neck Neoplasms ; metabolism ; pathology ; surgery ; Histiocytoma, Benign Fibrous ; metabolism ; pathology ; secondary ; surgery ; Humans ; Lung Neoplasms ; secondary ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Retrospective Studies ; Skin Neoplasms ; metabolism ; pathology ; surgery ; Young Adult