恶性纤维组织细胞瘤1例

报告1例恶性纤维组织细胞瘤。患者男,78岁,左胫前色素性结节10余年,近两年结节缓慢增大呈紫黑色斑块,无明显自觉症状。组织病理检查示,肿瘤中成分呈多形性表现,主要由增生的内皮细胞构成,部分细胞似成纤维细胞样交织排列呈游涡状,有些细胞似组织细胞样,可见形状怪异的多核巨细胞。     

A giant malignant fibrous histiocytoma on the scalp

Malignant fibrosis histiocytoma (MFH) is one of the most common soft tissue sarcomas of adult life. The most common sites of origin are the proximal extremities, particularly the thigh and buttock. The tumors are multilobular fleshy masses, often apparently circumscribed on gross examination. We report a case of a MFH of the head in a 57-year-old woman. Our case is clinically unusual in that the tumor rapidly grew up to 15 cm in a very short time.     

Atypical fibroxanthoma/malignant fibrous histiocytoma

ABSTRACT: Atypical fibroxanthoma (AFX) is an unusual spindle cell tumor occurring on actinically damaged skin of the head and neck. Clinically, it is often confused with basal cell carcinoma, squamous cell carcinoma, or even melanoma. Although initially thought to be a diagnosis of exclusion histologically, newer immunostains have helped in the identification of AFX. Mohs micrographic surgery has been utilized for the treatment due to its tissue sparing ability along with lower recurrence rate.     

Angiomatoid (malignant) fibrous histiocytoma as a second tumour in a child with neuroblastoma

Neuroblastoma occurring as a disseminated disease in children has a poor prognosis. Haematogenous metastases usually involve the marrow, bone, liver and skin. A second neoplasm may also develop. We describe a child with retroperitoneal neuroblastoma (stage 3) who developed a nodular mass in the inguinal area which was suspected to be a metastasis. Histopathology disclosed an angiomatoid (malignant) fibrous histiocytoma, and excision was curative. The occurrence of angiomatoid (malignant) fibrous histiocytoma as a second tumour in a patient with neuroblastoma has not previously been reported.     

复发性恶性纤维组织细胞瘤

报告1例切除14年后复发的恶性纤维组织细胞瘤。患者男，62岁。14年前行右胫前恶性纤维组织细胞瘤切除术，术后恢复良好。3个月前右胫前原手术部位又出现一肿块，逐渐增大，3d前肿块表面皮肤破溃，无明显自觉症状。肿块组织病理检查：肿瘤细胞排列呈梭形或星形，可见核分裂相及核瘤巨细胞，肿瘤细胞位于黏液样基质中，间质血管丰富，出血明显。     

Malignant fibrous histiocytoma arising on chronic osteomyelitis

We present a case of a primary malignant fibrous histiocytoma of the skin (MFH) arising on chronic osteomyelitis in a 67-year-old woman. Although this condition seems to be a predisposing factor for the onset of the malignancy, MFH complicating chronic osteomyelitis is generally localized at the level of the bone tissue. In the case we report the neoplasm was primitively localized at the dermal and subcutaneous level and presented as a rapidly growing mass.     

Aneurysmal fibrous histiocytoma: an unusual variant of cutaneous fibrous histiocytoma

Aneurysmal fibrous histiocytoma (AFH) (Santa-Cruz DJ, Kyriakos M. Aneurysmal ('Angiomatoid') fibrous histiocytoma of the skin. Cancer 1981;47:2053-2061) is a distinct but poorly recognized clinicopathological variant of cutaneous fibrous histiocytoma (CFH) that may result from the slow extravasation of blood into the tumour. The resulting lesion can have a very different clinicopathological appearance resulting in diagnostic confusion. We describe a patient with an AFH that presented as a pigmented nodule on the foot and discuss clinical recognition and histological differentiation from other tumours.     

血管瘤样纤维组织细胞瘤

报告1例血管瘤样纤维组织细胞瘤.患者女,24岁.因左胫前出现无痛性紫红色皮下结节4年就诊.皮损组织病理检查:真皮下层可见肿瘤细胞团块,肿瘤主要由成纤维细胞样细胞和组织细胞样细胞组成,在肿瘤团块中可见数处不规则出血性裂隙和囊腔,但管周无内皮细胞.免疫组化染色示肿瘤细胞波形蛋白(vimentin)、平滑肌肌动蛋白(SMA)、上皮膜蛋白(EMA)和CD68阳性,而结蛋白(desmin)、S-100蛋白、CD31、CD34和Ⅷ因子相关抗原均阴性.结合临床表现、组织病理改变和免疫组化染色,诊断为血管瘤样纤维组织细胞瘤.     

动脉瘤样纤维组织细胞瘤一例

患者,女,71岁。左胫前黑褐色结节5年。结合临床症状和组织病理检查,诊断为动脉瘤样纤维组织细胞瘤。给予手术完整切除治疗,随访至今未复发。     

Benign fibrous histiocytoma of the eyelid with an unusual clinical presentation

Benign fibrous histiocytoma is a common soft tissue tumor that can be deep or superficially located. Although the deep type of fibrous histiocytoma has a predilection for the orbit, the eyelids are an unusual location for the cutaneous type. A 42-year-old woman had bilateral yellowish nodular masses of the eyelids for two years. Pathological examination after excision revealed benign fibrous histiocytoma. Our case is an unusual clinical presentation of cutaneous fibrous histiocytoma as well as a rare location such as the eyelids.     

Malignant fibrous histiocytoma of the scalp. Multidisciplinary treatment

BACKGROUND: Malignant fibrous histiocytomas (MFH) are uncommon in the skin, and even less frequent on the scalp. On the scalp they are often very difficult to excise and it is even more difficult to close the resulting wound. OBJECTIVE: To review all malignant fibrous histiocytomas diagnosed and treated in our Department during the past 6 years, and to describe the multidisciplinary procedure employed to treat one special case of aggressive malignant fibrous histiocytoma on the scalp that recurred twice. RESULT: Malignant fibrous histiocytomas represent 0.01% of malignant cutaneous tumors in our area. The immediate results after a multidisciplinary treatment performed on a recurrent malignant fibrous histiocytoma located on the scalp were excellent, but recurrence was observed 6 months later. Two years later we have also treated another case of MFH on the scalp. The same surgical technique was performed, but the patient received high-dose-methotrexate-based neoadjuvant chemotherapy (HD-MTX). One year later, this patient is still alive and no signs of recurrence have been detected. CONCLUSION: When malignant fibrous histiocytoma occurs on the scalp it must be treated immediately by means of an excision with a large peripheral edge of 2 cm from the visual or CAT limits of the lesion, since the first treatment must be definitive.     

Aneurysmal fibrous histiocytoma showing transition from factor XIIIa-positive to -negative

A 39-year-old woman visited us because of a subcutaneous induration on her forehead of 7 months' duration. The tumor was tender, firm, and colored blue-black. Microscopically, the tumor was composed of fibroblastic and histiocytic cells showing no atypicality. In some part intra- or extracellular haemosiderin was diffusely seen. There were some blood-filled spaces of varying size without endothelial lining. From these findings, this case was diagnosed as aneurysmal fibrous histiocytoma (AFH), a variant form of dermatofibroma. Immunohistochemical analysis showed that factor XIIIa-positive histiocytic cells were distributed in the area without haemosiderin, but such cells were absent in the area with its deposition. Furthermore, the cells near the blood-filled space showed transition from factor XIIIa-positive to -negative, suggesting that the stromal instability induced by the attenuation of factor XIII may lead the formation of the blood-filled spaces in AFH.     

Metastatic aneurysmal fibrous histiocytoma in a 20-year-old woman: A rare case report with review of the literature and discussion of its genomic features

Aneurysmal fibrous histiocytoma is an uncommon variant of cutaneous fibrous histiocytomas with a local recurrence rate of 19%. We present a case of aneurysmal fibrous histiocytoma in a 20-year-old female with a regional lymph node metastasis and subsequent satellite nodule. The patient initially presented with a 1-month history of two palpable nodules in left lower anterior shoulder and left axilla. Needle core biopsies from both lesions revealed an atypical spindle cell neoplasm with a differential diagnosis of aneurysmal fibrous histiocytoma and angiomatoid fibrous histiocytoma. The axillary dissection confirmed a metastatic deposit in 1 out of 22 lymph nodes. At 6 months a satellite nodule arose between the resection scar and the axilla histopathologically demonstrating a cellular spindle cell nodule at the dermis subcutaneous junction with large, blood-filled pseudovascular spaces lined by histiocytes. The periphery of the lesion showed collagen trapping without a lymphoplasmacytic infiltrate. The lesional cells were diffusely positive for CD10 and focally for CD68 and Illumina RNA fusion panel sequencing was negative. Herein we present this case of metastatic aneurysmal fibrous histiocytoma with review of the literature and discussion of biology, cytogenetic alterations, and differential diagnosis.     

EMA positivity in epithelioid fibrous histiocytoma: a potential diagnostic pitfall

Background: Epithelioid fibrous histiocytoma (EFH) represents a morphologic variant of cutaneous fibrous histiocytoma (FH) but can lack many characteristic features. The presence of epithelioid cytomorphology may mimic other dermal neoplasms. Our anecdotal experience of epithelial membrane antigen (EMA) expression in some examples of EFH has caused diagnostic difficulty. Our aim was to examine the immunohistochemical profile and incidence of EMA expression in EFH. Methods: Forty‐four cases of EFH were retrieved from consultation files. Clinicopathologic and immunohistochemical features were evaluated. Results: Membranous EMA positivity was found in tumor cells in 27/42 cases (64%). Focal positivity for factor XIIIa was found in 10/14 (71%) and D2‐40 in 14/27 (52%). Scattered smooth muscle actin (SMA)‐positive tumor cells were seen in 11/43 (25%). Focal positivity for claudin‐1 was found in 3/42 (7%). CD163 staining highlighted stromal macrophages; however, in five cases it was difficult to exclude focal staining of tumor cells. Tumor cells were consistently negative for pan‐keratin, AE1/AE3, S100, CD31, CD34, CD68, desmin, p63, GFAP and CD45/LCA. Conclusion: Frequent EMA expression in EFH represents an unexpected finding and constitutes a potential diagnostic pitfall. Although of uncertain significance, this finding, when combined with established morphologic differences, raises the possibility that EFH is unrelated to classic FH. Doyle LA, Fletcher CDM. EMA positivity in epithelioid fibrous histiocytoma: a potential diagnostic pitfall.     

Management of unusual cutaneous malignancies: atypical fibroxanthoma, malignant fibrous histiocytoma, sebaceous carcinoma, extramammary Paget disease

Atypical fibroxanthoma, malignant fibrous histiocytoma, sebaceous carcinoma, and extramammary Paget disease are rare cutaneous tumors. Their recognition and diagnosis are critical in decreasing long-term morbidity and mortality. Surgical excision is the treatment of choice for these tumors, and Mohs micrographic surgery has been shown to be as favorable or better than wide local excision in providing long-term clearance rates.     

Atypical fibrous histiocytoma of the skin with CD30 and p80/ALK1 positivity and ALK gene rearrangement

We report the case of a two patients who presented with a solitary, asymptomatic, angiomatoid nodule on the right thigh. Histopathological finding showed a poorly circumscribed lesion, located in the dermis. The morphological aspect strongly suggested the diagnosis of atypical fibrous histiocytoma (AFH), but surprisingly, the neoplastic cells were diffusely CD30+, with a membrane staining devoid of paranuclear dot. The lesions were tested for p80/ALK1 expression. Surprisingly, we found a diffuse cytoplasmic positivity. Interestingly, using break‐apart fluorescent in situ hybridization (FISH), we evidenced an ALK rearrangement in nearly 50% of the neoplastic cells. The expression of CD30 and ALK1 with ALK gene rearrangement raised the possibility of three diagnoses: a primary cutaneous anaplastic large cell lymphoma (ALCL), a cutaneous inflammatory myofibroblastic tumor (IMT), an AFH of the skin associated with ALK gene rearrangement and CD30 positivity. The three hypotheses were discussed and finally, although p80/ALK1 expression and cytogenetic abnormalities in fibrous histiocytoma (FH) are not yet reported to the best of our knowledge, we favored the diagnosis of AFH.