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Neurothekeomas are rare, benign soft tissue tumors with fairly distinctive histomorphological features. They arise from the nerve sheath (nerve sheath myxoma/myxoid type) or fibrohistiocytic matrix (cellular type) and commonly involve the face, neck, and upper extremities in young adults. We report a rare, mixed-type neurothekeoma (showing both myxoid and cellular features), affecting the jaw of an 11-year-old girl. Histology was consistent with typical myxoid pattern, while immunohistochemistry suggested a fibrohistiocytic origin (cellular pattern). We excised the mass completely without any evidence of recurrence after 6 months.

Neurothekeomas are rare, benign soft tissue tumors with fairly distinctive histomorphological features. They arise from the nerve sheath (nerve sheath myxoma/myxoid type) or fibrohistiocytic matrix (cellular type) and commonly involve the face, neck, and upper extremities in young adults. We report a rare, mixed-type neurothekeoma (showing both myxoid and cellular features), affecting the jaw of an 11-year-old girl. Histology was consistent with typical myxoid pattern, while immunohistochemistry suggested a fibrohistiocytic origin (cellular pattern). We excised the mass completely without any evidence of recurrence after 6 months. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans (DFSP) is a rare indolent cutaneous tumor which has been considered as a low-grade dermal and subcutaneous fibrohistiocytic neoplasm. DFSP expands slowly but recurs frequently leading to the general assumption that DFSP is a locally aggressive neoplasm. This low-grade/borderline tumor which is generally found on trunk and proximal extremities of adults has limited potential for metastasis. Clinical presentation is usually typical with a red-brown or skin coloured indurated plaque with multiple nodules or protuberances. Histopathology of DFSP is also characteristical which demonstrates storiform pattern of uniform spindle cells infiltrating deep into the subcutaneous fat tissue constituting honeycomb appearence. Here, we report a case of DFSP in a 50-year-old woman who presents with a twenty year history of slowly growing mass on her left femoral area.

Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder that has been found in all continents and racial groups in relation to faulty repair of DNA with sun exposure. Several cutaneous and ocular tumors have been described in relation to XP including fibrous histiocytoma (FH). The diagnosis of conjunctival FH is challenging owing to the rarity of this tumor and the diversity of its classification into benign, locally aggressive and malignant. We are describing a recurrent FH exhibiting a locally aggressive behavior in a child with history of XP. Detailed histopathological features are presented with literature review. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans (DFSP) is a rare indolent cutaneous tumor which has been considered as a low-grade dermal and subcutaneous fibrohistiocytic neoplasm. DFSP expands slowly but recurs frequently leading to the general assumption that DFSP is a locally aggressive neoplasm. This low-grade/borderline tumor which is generally found on trunk and proximal extremities of adults has limited potential for metastasis. Clinical presentation is usually typical with a red-brown or skin coloured indurated plaque with multiple nodules or protuberances. Histopathology of DFSP is also characteristical which demonstrates storiform pattern of uniform spindle cells infiltrating deep into the subcutaneous fat tissue constituting honeycomb appearence. Here, we report a case of DFSP in a 50-year-old woman who presents with a twenty year history of slowly growing mass on her left femoral area. [ABSTRACT FROM AUTHOR]

Background: Inflammatory myofibroblastic tumors (IMTs) of the central nervous system (CNS) are rare entities with diverse histopathological features and varying propensities to recur. Case Description: A 26 year‑old male with an IMT of the CNS of the left tentorium had tumor progression 2 months after partial surgical resection. Histopathological studies confirmed expression of ALK. Macroscopic total resection was performed followed by radiotherapy. A recurrence occurred 20 months after the second surgery that necessitate reoperation. Including the present case, we identified 30 cases of IMT of the CNS corresponding to our search criteria in the literature. The extent of resection was reported in 26 of these cases. Gross total resection was done in 75% of ALK‑positive and in 61% of ALK‑negative cases. Recurrence rate after gross total resection for ALK‑positive and ALK‑negative cases was 33% and 9%, respectively. Every recurrence in ALK‑positive patients occurred within 2 years after surgery. Conclusion: IMT of the CNS are a heterogeneous group of tumors and the treatment of choice is complete surgical resection. Because of the high recurrence rate reported for IMT of the CNS expressing ALK, a closed follow‑up is recommended. When faced with an early recurrence, a surgical resection followed by radiotherapy may be advised. [ABSTRACT FROM AUTHOR]

Background: Sox10 is a transcription factor associated with neural crest development. Its expression has been reported in melanocytes and peripheral nerve sheath cells and their associated tumors. Objective: To assess Sox10 sensitivity in benign and malignant melanocytic neoplasms of various histologic subtypes and to discern the specificity of Sox10 in distinguishing between melanocytic neoplasms and fibrohistiocytic and histiocytic mimickers. Methods: Sox10 expression was examined by immunohistochemistry in 145 cases of formalin-fixed paraffin-embedded tissue, including benign and malignant melanocytic lesions of various histologies and stages (n = 83), fibrohistiocytic and histiocytic lesions (n = 33), and peripheral nerve sheath tumors (n = 19), among others (n = 10). Results: Immunoreactivity with Sox10 was observed in 100% (83/83) of benign and malignant melanocytic lesions of various subtypes, as well as in 100% (19/19) of benign and malignant peripheral nerve sheath lesions. Among the fibrohistiocytic proliferations and histiocytoses examined, Sox10 was negative in all cases (0/33). Sox10 expression did not vary by histologic subtype in nevi or melanoma; however, both the percentage of tumor nuclei demonstrating Sox10 expression and the intensity of expression were inversely correlated with malignant potential (nevi, melanoma in situ, invasive and metastatic melanoma) (P < .001, P = .016, respectively). Malignant peripheral nerve sheath tumors also showed decreased mean Sox10 expression and decreased intensity of expression when compared with benign counterparts (P < .001, P = .021, respectively). Limitations: This is a retrospective study with 145 cases included. Conclusions: Sox10 is a highly sensitive marker for melanocytic proliferations and may be useful diagnostically when the differential diagnosis includes fibrohistiocytic and histiocytic proliferations demonstrating S100 expression. [ABSTRACT FROM AUTHOR]

Abstract: We report a case of a 5-year-old female who presented with vaginal bleeding of unexplained etiology. There was no evidence of precocious puberty by history and physical examination. Endocrine laboratory studies were in the normal range for a prepubertal female. On vaginoscopy, a friable, granulomatous mass that bled easily was discovered within the vaginal vault. Pelvic sonography and magnetic resonance imaging of the pelvis was significant for a left adnexal mass. Surgical exploration and histological analysis revealed an unusual fibrohistiocytic proliferation. This unusual case broadens the differential diagnosis for vaginal bleeding in the prepubertal child (). [Copyright &y& Elsevier]

Aims : To describe 13 new cases of a rare soft tissue neoplasm currently known as haemosiderotic fibrohistiocytic lipomatous tumour (HFHLL) and to further its characterization. Methods and results : Patients were eight females and five males, aged 8 months to 74 years. Lesions ranged in size from 10 to 130 mm (median 45). Twelve of 13 lesions were located in the ankle/foot region; one involved the hand. Grossly the lesions were fatty/gelatinous or lipoma-like. They were characterized by varying proportions of mature adipocytes and fibroblastic spindle cells, associated with haemosiderin pigment present predominantly in macrophages within the spindle cell component. Dissecting between adipocytic lobules were septa containing short spindle cells with streaming, swirling or honeycomb growth patterns. Most tumours contained scattered osteoclast-like giant cells. Mitoses were rare and only one case showed mild atypia. Immunohistochemistry in 10 cases showed 7/9 CD34+; all were negative for smooth muscle actin, CD68, S100 and desmin. Follow-up in nine cases ranging from 1 to 130 months (median 12) showed local recurrence in three but no metastasis. Conclusions : So-called HFHLL, better termed haemosiderotic fibrolipomatous tumour, is a distinct lesion characterized by an admixture of fibroblastic spindle cells, mature adipocytes and haemosiderin pigmentation, shows a predilection for the distal extremities and quite often recurs locally. [ABSTRACT FROM AUTHOR]

Background: Atypical fibroxanthoma (AFX) is a fibrohistiocytic tumor with relatively high local recurrence rates but low metastatic potential. Wide local excision (WLE) and Mohs micrographic surgery (MMS) are common treatments, although no consensus exists regarding optimal therapy.Objective: To systematically review evidence of AFX recurrence and metastatic rates after different surgical modalities.Methods: A comprehensive search was performed for articles published from 1946 or database inception to March 20, 2017. Studies selected included those that had 5 or more patients with atypical fibroxanthoma treated surgically. Two reviewers independently abstracted the data. Risk of bias was assessed with the Newcastle-Ottawa scale. Main outcomes and measures included recurrence and metastasis.Results: In total, 23 studies were selected (907 patients and 914 tumors); 175 patients were treated with MMS (recurrence rate 2.0%, 95% confidence interval [CI] 0%-4.1%; metastatic rate 1.9%, 95% CI 0.1%-3.8%), and 732 were treated with WLE (recurrence rate 8.7%, 95% CI 5%-12.3%; metastasis rate 1%, 95% CI 0.2%-1.9%). Among immunocompromised patients, no recurrence or metastases developed in the MMS subgroup, although 4 of 10 recurred and 1 of 10 metastasized in the WLE subgroup.Limitations: Low quality of the studies published.Conclusion: MMS for atypical fibroxanthoma is associated with a lower recurrence rate than WLE. [ABSTRACT FROM AUTHOR]

Fibrohistiocytic tumors are a diverse group of reactive and neoplastic lesions including xanthoma, fibrous histiocytoma and its variants, solitary xanthogranuloma, dermatofibrosarcoma protuberans, and atypical fibroxanthoma. This article reviews some of the more commonly encountered fibrohistiocytic tumors with an emphasis on clinical presentation, macroscopic and histologic characteristics, molecular/cytogenetic findings where applicable, and differential diagnoses., (Copyright © 2017 Elsevier Inc. All rights reserved.)