Dermatofibroma (DF) is a common, benign fibrohistiocytic tumor with unknown pathogenesis. There are multiple uncommon histologic variants of DF reported in literature, one of which is hemosiderotic DF. It can variably present as a pigmented papule or nodule commonly located on the lower extremities. Hemosiderotic DF remains to be underreported in the Philippines despite the widely available dermatopathology services in the country.
Human ; dermatofibroma ; histiocytoma, benign fibrous

Histiocytoma, Benign Fibrous/surgery* ; Humans ; Splenectomy ; Splenic Diseases/pathology* ; Splenic Neoplasms/surgery*

Humans ; RNA-Binding Protein EWS/genetics* ; Histiocytoma, Malignant Fibrous/pathology* ; Oncogene Proteins, Fusion/genetics* ; Biomarkers, Tumor ; Cyclic AMP Response Element-Binding Protein/genetics*

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*

Arm ; Fibroma ; Histiocytoma, Malignant Fibrous ; Humans ; Soft Tissue Neoplasms

OBJECTIVE: To investigate ultrasound and MRI features of malignant fibrous histiocytoma (MFH) of soft tissue. METHODS: Ultrasound, MRI images and pathological data of 12 patients with malignant fibrous histiocytoma in soft tissue confirmed by operation and pathology were analyzed from January 2012 to August 2018, inlcuding 7 males and 5 females, aged from 36 to 69 years old with an average age of 53 years old; the courses of disease ranged from 4 to 49 months with an average of 28 months. Clinical manifestations were soft tissue masses and pain in the affected limbs. Ultrasound, MRI and contrast-enhanced examination were performed before operation. The lesions, morphology, echo/signal characteristics, color flow signals and enhancement features were observed and compared with pathology. RESULTS: In 12 patients with MFH, 9 patients were primary lesions and 3 patients were recurrent lesions after operation. There were 7 cases of bilateral thighs, 2 cases of calves, 1 case of upper arm, 1 case of buttocks and 1 case of posterior peritoneum. The size ranged from 5.1 to 17.1 cm with an average of 8.7 cm. Ultrasound feature showed lobulated or agglomerate, and focused on low echo; 5 cases had capsule and with clear border; 7 cases were unclear boundary with surrounding tissues; and 6 cases with irregular echo-free. The blood flow signals were around the CDFI, and the internal blood flow signals were different. MRI feature showed lobulated, agglomerate or irregular shape, T1WI showed slightly lower signal or equal signal, T2WI showed high signal and DWI signal increased. Six patients manifested mixed signal inside, 7 patients manifested low signal separation inside, 5 patients with false envelope, and 9 patients manifested infiltration and growth with peripheral edema. T1WI showed uneven strengthening after enhancement. Immunohistochemical expression of Vim, CD68 were positive. CONCLUSIONS The age, location and imaging features of soft tissue MFH are characteristic. The diagnosis of MFH should be considered when irregular mass occurred in soft tissues of limbs at middle-aged and old people. Echo and signal are homogeneous or mixed. Separation, necrosis and cystic degeneration could be seen in the mass. When the blood flow signals are abundant and solid components are obviously enhanced, the diagnosis of MFH should be considered.
Adult ; Aged ; Edema ; Extremities ; Female ; Histiocytoma, Malignant Fibrous ; diagnostic imaging ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Ultrasonography

Primary malignant fibrous histiocytoma (MFH) of the chest wall is extremely rare and is characterized by aggressive features, including a high incidence of local recurrence and distant metastasis. Surgical resection of the chest wall is the primary modality of management. However, surgical treatment is not generally recommended in patients with evidence of distant metastasis. Here, we present a case of chest wall MFH along with a schwannoma mimicking distant metastasis in the right upper arm. The patient was treated by radical en bloc resection and survived for more than 9 years without recurrence.
Arm ; Histiocytoma, Malignant Fibrous ; Humans ; Incidence ; Neoplasm Metastasis ; Neurilemmoma ; Positron-Emission Tomography ; Recurrence ; Thoracic Wall ; Thorax

BACKGROUND: This study investigated the clinical outcomes of surgical treatment of primary chest wall soft tissue sarcoma (CW-STS). METHODS: Thirty-one patients who underwent surgery for CW-STS between 2000 and 2015 were retrospectively reviewed. The disease-free and overall survival rates were estimated using the Kaplan-Meier method, and prognostic factors were analyzed using a Cox proportional hazards model. RESULTS: The median follow-up duration was 65.6 months. The most common histologic type of tumor was malignant fibrous histiocytoma (29%). The resection extended to the soft tissue in 14 patients, while it reached full thickness in 17 patients. Complete resection was achieved in 27 patients (87.1%). There were 5 cases of local recurrence, 3 cases of distant metastasis, and 5 cases of combined recurrence. The 5-year disease-free rate was 49%. Univariate analysis indicated that incomplete resection (p<0.001) and stage (p=0.062) were possible risk factors for recurrence. Multivariate analysis determined that incomplete resection (p=0.013) and stage (p=0.05) were significantly associated with recurrence. The overall 5- and 10-year survival rates were 86.8% and 64.3%, respectively. No prognostic factor for survival was identified. CONCLUSION: Long-term primary CW-STS surgery outcomes were found to be favorable. Incomplete microscopic resection and stage were risk factors for recurrence.
Follow-Up Studies ; Histiocytoma, Malignant Fibrous ; Humans ; Methods ; Multivariate Analysis ; Neoplasm Metastasis ; Proportional Hazards Models ; Recurrence ; Retrospective Studies ; Risk Factors ; Sarcoma ; Survival Rate ; Thoracic Wall ; Thorax

An undifferentiated pleomorphic sarcoma (UPS), also known as a malignant fibrous histiocytoma in the past, commonly involves the soft tissue of the extremities and the retroperitoneum. However, a primary UPS of the colon mesentery is very rare. A 69-year-old male patient visited our outpatient department for treatment of an enlarged, palpable mass in the right lower quadrant (RLQ). Computed tomography showed a 15-cm multilobulated, heterogeneous, enhanced mass in the RLQ, which we suspected originated from the colon. He underwent a right hemicolectomy, and the pathologic result was a colon mesenteric UPS. We report a rare case of a primary UPS of the colon mesentery and discuss the characteristics of this neoplasm in reference to the literature.
Aged ; Colon ; Extremities ; Histiocytoma, Malignant Fibrous ; Humans ; Male ; Mesentery ; Outpatients ; Sarcoma

Dermatofibroma is a fibrohistiocytic tumor of the skin that commonly occurs as a solitary lesion on the lower extremities of young women. Multiple eruptive dermatofibromas (MEDFs), defined as the presence of 5 to 8 dermatofibromas appearing within a period of 4 months, are rare, and the etiology of MEDFs remains unknown. However, MEDFs are frequently thought to be associated with altered immunity, such as autoimmune diseases and immunosuppression. It is unclear why MEDFs occur in association with these conditions, but this relationship may suggest that MEDFs are the result of an abortive immunoreactive process. We herein describe a middle-aged woman who developed multiple dermatofibroma. She had myasthenia gravis for 8 years, and developed MEDFs after undergoing oral corticosteroid treatment (245 mg/week) for 3 months. Therefore, our case suggests that the use of immunosuppressants is a more dominant factor for the development of MEDFs than underlying autoimmune diseases.
Autoimmune Diseases ; Female ; Histiocytoma, Benign Fibrous ; Humans ; Immunosuppression ; Immunosuppressive Agents ; Lower Extremity ; Myasthenia Gravis ; Skin