Pseudomyogenic hemangioendothelioma (PHE) is a rare angiogenic tumor. Histologically, the morphological characteristics of neoplastic vessels and endothelial differentiation are not obvious, and it is easy to be confused with epithelioid sarcoma, epithelioid hemangioendothelioma and myogenic tumor. PHE usually occurs in arms and legs in young people and has a significant male predominance. The tumor has a predilection for the distal extremities and its typical manifestation is multiple center invasion of a single limb, which can involve all layers of skin and subcutaneous tissues,and is often accompanied by abvious pain. Histologically, PHE is characterized by infiltrative growth of tumor. Most tumor lesions are composed of sheets and loose fascicles of plump spindle or epithelioid cells within a background of variably prominent inflammatory infiltration, which was commonly composed of neutrophils. Some cells may resemble rhabdomyoblasts, and nuclear atypia and mitosis were rare. The tumor cells generally expressed positive cytokeratin (CK), ETS-related gene (ERG), Friend leukemia virus integration 1 (FLI1) and integrase interactor 1(INI1). In some cases, the tumor cells expressed CD31. A case of a young woman was reported in this paper, who presented with a subcutaneous mass with severe pain and was chronologically misdiagnosed with herpes zoster, low-grade malignant fibrous histiocytoma and epithelioid hemangioendothelioma. In this study, the clinical and pathological features, differential diagnosis and the latest progress in therapy of PHE were analyzed based on relevant literature.
Adolescent ; Adult ; Biomarkers, Tumor ; Child ; Diagnosis, Differential ; Diagnostic Errors ; Female ; Hemangioendothelioma, Epithelioid/pathology* ; Hemangioma ; Histiocytoma, Malignant Fibrous/diagnosis* ; Humans ; Male ; Pain ; Precancerous Conditions/diagnosis*

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*

Pleomorphic dermal sarcoma (PDS) is a rare mesenchymal neoplasm sharing histopathological features with atypical fibroxanthoma (AFX), but has additional features of deep invasion of the superficial subcutis, tumor necrosis and vascular/perineural invasion. It is not well documented in the literature because of its rarity, and its clinical course has been debated due to the lack of homogenous criteria. We describe here the case of a 91-year-old female with a 6-month history of a solitary, asymptomatic, well-defined, 3.4-cm-sized, reddish, hard, protruding mass on the lateral aspect of the right upper eyelid. On the basis of initial punch biopsy results, storiform cellular infiltrate of pleomorphic spindle and polygonal cells with frequent atypical mitoses, the lesion was identified as AFX. Following the initial biopsy, micrographic surgery was performed and a tumor-free margin was confirmed. Considering the conservation of the periocular function and the advanced age of the patient, we planned secondary intention healing rather than primary suturing. After surgery, skeletal muscle infiltration was found and the diagnosis was revised to PDS by a pathologist based on the currently accepted criteria for PDS. There has been no evidence of recurrence or periocular functional defects during a 2-year follow-up without adjuvant therapy. Although the PDS is highly malignant, complete excision under micrographic surgery can prevent recurrence without adjuvant therapy. Also, the secondary intention healing is an effective method for closure of large defects on the face.
Biopsy ; Diagnosis ; Eyelids* ; Female ; Follow-Up Studies ; Histiocytic Sarcoma ; Histiocytoma, Malignant Fibrous ; Humans ; Intention* ; Methods ; Mitosis ; Muscle, Skeletal ; Necrosis ; Recurrence ; Sarcoma*

Pleomorphic dermal sarcoma (PDS) is a rare mesenchymal neoplasm sharing histopathological features with atypical fibroxanthoma (AFX), but has additional features of deep invasion of the superficial subcutis, tumor necrosis and vascular/perineural invasion. It is not well documented in the literature because of its rarity, and its clinical course has been debated due to the lack of homogenous criteria. We describe here the case of a 91-year-old female with a 6-month history of a solitary, asymptomatic, well-defined, 3.4-cm-sized, reddish, hard, protruding mass on the lateral aspect of the right upper eyelid. On the basis of initial punch biopsy results, storiform cellular infiltrate of pleomorphic spindle and polygonal cells with frequent atypical mitoses, the lesion was identified as AFX. Following the initial biopsy, micrographic surgery was performed and a tumor-free margin was confirmed. Considering the conservation of the periocular function and the advanced age of the patient, we planned secondary intention healing rather than primary suturing. After surgery, skeletal muscle infiltration was found and the diagnosis was revised to PDS by a pathologist based on the currently accepted criteria for PDS. There has been no evidence of recurrence or periocular functional defects during a 2-year follow-up without adjuvant therapy. Although the PDS is highly malignant, complete excision under micrographic surgery can prevent recurrence without adjuvant therapy. Also, the secondary intention healing is an effective method for closure of large defects on the face.
Biopsy ; Diagnosis ; Eyelids* ; Female ; Follow-Up Studies ; Histiocytic Sarcoma ; Histiocytoma, Malignant Fibrous ; Humans ; Intention* ; Methods ; Mitosis ; Muscle, Skeletal ; Necrosis ; Recurrence ; Sarcoma*

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

Undifferentiated sarcomas are rarely identified in the intracranial region. A 23-year-old man was admitted with a chief complaint of headache. Initial magnetic resonance images showed signs of low-grade glioma in the frontal lobe. Stereotactic biopsy was performed, and a diagnosis of diffuse astrocytoma was confirmed. Three months later, the patient presented with a high-grade tumor as seen on imaging studies. He underwent total resection of the tumor and histopathological tests identified an undifferentiated sarcoma. The patient died eight months later due to massive tumor bleeding. To the best of our knowledge, this is the first report of undifferentiated sarcoma arising from low-grade glioma without any chemotherapy or radiotherapy.
Astrocytoma ; Biopsy ; Diagnosis ; Drug Therapy ; Frontal Lobe ; Glioma* ; Headache ; Hemorrhage ; Histiocytoma, Malignant Fibrous ; Humans ; Radiotherapy ; Sarcoma* ; Young Adult

Undifferentiated pleomorphic sarcoma, known as malignant fibrous histiocytoma, is a malignant neoplasm that arises in both soft tissue and bones. In 2002, the World Health Organization declassified malignant fibrous histocytoma as a formal diagnostic entity and renamed it 'undifferentiated pleomorphic sarcoma not otherwise specified.' It most commonly occurs in the lower extremities and rarely metastasizes cutaneously. We report a case of cutaneous metastatic undifferentiated pleomorphic sarcoma of the buttocks occurring in a 73-year-old man diagnosed with mediastinal sarcoma 4 years previously. He first noticed the mass approximately 2 months previously. Histological findings with immunomarkers led to a final diagnosis of cutaneous metastatic sarcoma from mediastinal undifferentiated pleomorphic sarcoma.
Aged ; Buttocks ; Diagnosis ; Histiocytoma, Malignant Fibrous ; Humans ; Lower Extremity ; Sarcoma* ; World Health Organization

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

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