Primary Pulmonary Myxoid Sarcoma and Thoracic Angiomatoid Fibrous Histiocytoma: Two Sides of the Same Coin? Primary pulmonary myxoid sarcoma (PPMS) and thoracic angiomatoid fibrous histiocytoma (AFH) are rare neoplasms with EWSR1 fusions and overlapping morphology. Both tumor types often show epithelial membrane antigen expression, but AFH characteristically co-expresses desmin. We encountered
An Unusual Case of an ALK-Negative Epithelioid Fibrous Histiocytoma in the External Auditory Canal. This article represents the first reported case in the external auditory canal of epithelioid fibrous histiocytoma (EFH), a rare benign cutaneous epithelioid neoplasm. Immunostaining revealed uncommon negative staining for anaplastic lymphoma kinase (ALK) expression. This case and literature review
Clinicopathologic and molecular study of superficial CD34-positive fibroblastic tumours mimicking atypical fibrous histiocytoma (dermatofibroma). Superficial CD34-positive fibroblastic tumour (SCD34FT) is an uncommon but distinctive low-grade neoplasm of the skin and subcutis that shows frequent CADM3 expression by immunohistochemistry (IHC). In this study, prompted by an index case resembling 'atypical fibrous histiocytoma (FH)' that was positive for CADM3 IHC, we systematically examined a cohort of tumours previously diagnosed as 'atypical FH' by applying CADM3 and fluorescence in situ hybridization (FISH) for PRDM10 rearrangement, to investigate the overlap between these tumour types. Forty cases of atypical FH were retrieved, including CD34-positive tumours (n = 20) and CD34-negative
Epithelioid Fibrous Histiocytoma Is on a Continuum With Superficial ALK-rearranged Myxoid Spindle Cell Neoplasm: A Clinicopathologic Series of 35 Cases Including Alternate RET and NTRK3 Fusions. Anaplastic lymphoma kinase (ALK) rearrangements drive most examples of epithelioid fibrous histiocytoma (EFH) and have been reported in an emerging family of receptor tyrosine kinase (RTK) fusion-positive the diagnostic term "myxoid spindle cell variant of epithelioid fibrous histiocytoma."
Kinase expression in angiomatoid fibrous histiocytoma: panTRK is commonly expressed in the absence of NTRK rearrangement. Angiomatoid fibrous histiocytoma (AFH) is a soft tissue tumour of intermediate (rarely metastasising) malignant potential, which harbours gene fusions. These tumours can express anaplastic lymphoma kinase (ALK) in the absence of gene rearrangement or copy number alteration
Expanding Our Knowledge of Molecular Pathogenesis in Histiocytoses: Solitary Soft Tissue Histiocytomas in Children With a Novel CLTC::SYK Fusion. The histiocytoses comprise a histopathologically and clinically diverse group of disorders bearing recurrent genomic alterations, commonly involving the BRAF gene and mitogen-activated protein kinase pathway. In the current study, a novel CLTC::SYK ), Touton giant cells were absent. A separate cohort of classic JXG cases subsequently profiled by fluorescence in situ hybridization were negative for the presence of a CLTC::SYK fusion suggesting that CLTC::SYK fusion-positive histiocytoma is genetically and histologically distinct from JXG. We postulate that the CLTC::SYK fusion leads to aberrant activation of the SYK kinase, which is involved
Angiomatoid fibrous histiocytoma in the spinal canal of T3-T4: a case report and literature review. Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumour that occurs in the superficial tissue of extremities of children and young adults. A painless mass in the deep dermis and subcutaneous tissue is the main clinical manifestation. AFH also occurs infrequently in the central nervous
Benign fibrous histiocytoma of the medial rectus muscle. We report the case of a healthy 17-year-old girl who underwent surgery for excision of a painless, rapidly enlarging subconjunctival mass. The mass was found to be tightly adherent to the medial rectus muscle of the left eye, requiring extensive dissection. Histopathology revealed a mass of bland and foamy spindle cells in a storiform pattern that was positive for CD68, PGM1, and factor XIIIA and negative for S-100. The clinical and histopathologic findings are consistent with benign fibrous histiocytoma.
Malignant fibrous histiocytoma of bone: A survival analysis from the National Cancer Database. Malignant fibrous histiocytoma (MFH) of bone, now known as undifferentiated pleomorphic sarcoma of bone, is a rare neoplasm that accounts for less than 2% of all primary malignant bone tumors. The objective of the current study was to evaluate prognosis and survival for MFH of bone. The 2004 to 2016
Primary Pulmonary Myxoid Sarcoma and Myxoid Angiomatoid Fibrous Histiocytoma: A Unifying Continuum With Shared and Distinct Features. Primary pulmonary myxoid sarcoma (PPMS) is a recently reported, exceedingly rare low-grade lung neoplasm characterized by reticular/lace-like growth of spindle to epithelioid cells embedded in an abundant myxoid matrix. Morphologically, it overlaps with a myxoid variant of angiomatoid fibrous histiocytoma (AFH) of the soft tissue. Genetically, they were both reported to harbor EWSR1-CREB1 fusion, while EWSR1-ATF1 has only been reported in AFH thus far. We report a case of primary pulmonary low-grade myxoid spindle cell tumor with morphologic and immunohistochemical features of PPMS but with an EWSR1-ATF1 fusion gene. In addition, we also encountered a case
Primary Renal Malignant Fibrous Histiocytoma in an End-Stage Renal Disease Patient. Besides renal cell carcinoma with increased risk reported in the dialysis population, other unusual types of renal tumors should also be considered. However, to the best of our knowledge, renal sarcomas have never been reported among end-stage renal disease patients undergoing dialysis in the literature . In this study, we present the first case of a primary renal malignant fibrous histiocytoma (MFH, also called undifferentiated pleomorphic sarcoma) in a 41-year-old woman with end-stage renal disease.
Lung adenocarcinoma metastasizing to fibrous histiocytoma: A case report. Tumor-to-tumor metastasis is an uncommon phenomenon, and the tumor metastatic to mesenchymal tumor is extremely rare. To our knowledge, this is the first case of lung adenocarcinoma metastasizing to fibrous histiocytoma. A 58-year-old Chinese woman was admitted to our hospital with a complaint of progressive enlargement pleomorphic nuclei were dispersed in an otherwise typical cutaneous fibrous histiocytoma. These atypical cells arranged in poorly glandular structures or irregular epithelioid nest and were demonstrated as metastatic lung adenocarcinoma by immunohistochemical staining. We reviewed the biopsy section and found these 2 sites of adenocarcinoma shared similar histologic morphological features. Therefore
38-year-old woman with nasal congestion: A rare case of septum-originated benign fibrous histiocytoma. Benign fibrous histiocytoma (BFH) is a mesenchymal tumor composed of fibroblasts and histiocytes, which normally occurs in the bone tissue and/or the skin of the limbs. BFH of the nasal septum is rare worldwide. A 38-year-old female patient attended our clinic with congestion in her right nasal
A rare case of primary malignant fibrous histiocytoma: a sarcoma of the kidney. Primary malignant fibrous histiocytoma of the kidney (MFH) is an extremely rare tumor which is indistinguishable from its' mimics in particular renal cell carcinoma (RCC) by clinical features and preoperative imaging evaluation. Due to its high predilection to local recurrence and distant metastases, early diagnosis has great value. Herein, we describe a 62 year-old man with a 3-month history of abdominal discomfort and a palpable right loin mass whom computerized tomography (CT) showed a large heterogeneous solid mass in the right kidney. With the suspicious to RCC; the patient underwent radical nephrectomy. However, histopathological report revealed pleomorphic-storiform malignant fibrous histiocytoma
A huge benign fibrous histiocytoma arising from the renal capsule: report of a case. Neoplasms originating in the renal capsule are very rare. Benign fibrous histiocytoma(BFH) most commonly occurs in the dermis and subcutis, few cases of this tumor appear in the renal capsule. In particular, BFH larger than 20 cm are scarce. Here we report a rare huge one measuring 23 × 13 × 7 cm. We report a 64
Deep benign fibrous histiocytoma occurring in the kidney: Case report. Deep benign fibrous histiocytoma (BFH) is relatively rare in contrast to superficial BFH involving the skin. Moreover, it was extremely rare for deep BFH occurring in the solid organ. To our knowledge, so far, only one case of deep BFH of the kidney was reported in English literature. Herein, we report another case of deep
Management of giant benign fibrous histiocytoma in the spinal region with pleural involvement: A case report. Benign fibrous histiocytoma with pleural involvement in spinal region is a highly unusual disease with no standard curative managements yet. The objective of this study is to report an extremely rare case of a giant benign fibrous histiocytoma with pleural involvement in spinal region of the T7 vertebral and paraspinal region involving the right thoracic cavity, which presented as a solid tumor. Postoperative pathology confirmed the diagnosis of thoracic benign fibrous histiocytoma. The patient underwent thoracic spinal canal decompression, complete tumor resection, pleural defect repair, and T4 to T10 internal fixation procedure via a posterior approach. The patient's symptom improved
ALK Expression in Angiomatoid Fibrous Histiocytoma: A Potential Diagnostic Pitfall. We recently encountered a case of primary pulmonary angiomatoid fibrous histiocytoma (AFH), which was initially misdiagnosed as inflammatory myofibroblastic tumor (IMT) based in part on anaplastic lymphoma kinase (ALK) expression by immunohistochemistry (IHC). Prompted by this experience, we evaluated ALK
Angiomatoid Fibrous Histiocytoma With Paraneoplastic Platelet Storage Pool Deficiency. Angiomatoid fibrous histiocytoma is a rare soft tissue tumor usually discovered in young individuals. This tumor is often mistaken for a hematoma and typically misdiagnosed. It is commonly found in the extremities and may be associated with a site of recent or previous trauma. Characteristic histology includes
Primary intracranial angiomatoid fibrous histiocytoma: a case report and literature review. Angiomatoid fibrous histiocytoma (AFH) is a tumour primarily occurring in the extremities which can very rarely occur as an isolated intracranial lesion. We report a case of a 22-year-old woman presenting with generalized seizure and visual field deficit due to an occipital mass, which immunohistochemistry