Fibrous tissue neoplasms

  • 文章类型: Case Reports
    浅表CD34阳性纤维母细胞瘤(SCPFT)是一种最近描述的疾病实体,其特征是明显的核多态性,低有丝分裂计数,和弥漫性CD34阳性。这是一种罕见的,独特的,低度成纤维细胞肿瘤。迄今为止,在英语文献中仅报告了44例。在这里,我们报告了两例SCPFT,其中一名48岁男性和一名22岁男性左右大腿浅表肿瘤,分别。在这两种情况下都进行了切除术。组织学上,两种肿瘤均显示梭形至上皮样细胞以束状或片状模式排列。大多数细胞显示颗粒状或嗜酸性玻璃质,标记的核多态性,和低有丝分裂率。在免疫组织化学染色上,肿瘤细胞CD34弥漫性阳性,S100蛋白阴性,平滑肌肌动蛋白,和Desmin。广泛切除后,16个月和11个月的临床随访均未出现复发或转移,分别。据我们所知,这是韩国报告的前两例SCPFT病例。我们相信这些病例报告将有助于对SCPFT的临床病理理解,并帮助临床医生将该肿瘤与其他浅表软组织肿瘤区分开来。
    Superficial CD34-positive fibroblastic tumor (SCPFT) is a recently described disease entity characterized by marked nuclear pleomorphism, low mitotic count, and diffuse CD34 positivity. It is a rare, distinctive, low-grade fibroblastic neoplasm. To date, only 44 cases have been reported in the English-language literature. Herein, we report two cases of SCPFT involving a 48-year-old male and a 22-year-old male with superficial tumors on the right and left thighs, respectively. Excision was performed in both cases. Histologically, both tumors showed spindle-to-epithelioid cells arranged in fascicular or sheet-like patterns. Most cells displayed granular or eosinophilic glassy cytoplasm, marked nuclear pleomorphism, and a low mitotic rate. On immunohistochemical staining, tumor cells were diffusely positive for CD34 and negative for S100 protein, smooth muscle actin, and desmin. After wide excision, neither patient experienced recurrence or metastasis after 16 months and 11 months of clinical follow-up, respectively. To the best of our knowledge, these are the first two cases of SCPFT reported in Korea. We believe these case reports would contribute to the clinicopathological understanding of SCPFT and assist clinicians in differentiating this tumor from other superficial soft tissue neoplasms.
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  • 文章类型: Journal Article
    这项研究的目的是更好地表征头颈部孤立性纤维瘤(SFT)并评估手术治疗。这项回顾性研究包括患有头颈部SFT的患者。临床,放射学,收集有关所进行治疗的组织学信息和数据。计算局部和远处转移的风险,对于轨道SFT,使用了特定的分类。总的来说,包括34例患者。大多数SFT在口腔中发现(n=10),其次是颈部区域(n=8)。平均复发时间为67.4个月。所有患者均接受一期手术切除。在5例局部复发和远处转移风险较低的患者中观察到复发。选择的治疗方法是完全切除。复发似乎与手术切缘阳性高度相关。切除病灶时应增加安全裕度,应进行长期随访。
    The aim of this study was to better characterize head and neck solitary fibrous tumors (SFTs) and to evaluate surgical treatment. This retrospective study included patients who presented with head and neck SFTs. Clinical, radiological, and histological information and data regarding the treatments performed were collected. The risk of locoregional and distant metastases was calculated, and for orbital SFTs a specific classification was used. Overall, 34 patients were included. The majority of the SFTs were found in the oral cavity (n = 10), followed by the neck region (n = 8). The mean time to recurrence was 67.4 months. All patients underwent primary surgical resection. Recurrence was observed in five patients with a low risk of locoregional recurrence and distant metastasis. The treatment of choice is complete resection. Recurrence seems to be highly correlated with positive surgical margins. The safety margin should be increased when removing the lesion, and long-term follow-up should be performed.
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  • 文章类型: Case Reports
    We report on a 47-year-old woman with unilateral fibrous dysplasia and three intramuscular masses. Medical imaging revealed possible intramuscular myxomas, so that the suspected diagnosis was Mazabraud syndrome. After biopsy, the suspected diagnosis was verified by histology and molecular pathology. Due to endocrine abnormalities in the patient\'s medical history, McCune-Albright syndrome has was also verified.
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    文章类型: Case Reports
    BACKGROUND: The rare cutaneous solitary fibrous tumor was initially described in the thoracic cavity in relation to the pleura and subsequently been associated with other serous membranes. It has been described in other extraserosal locations including the skin. Knowledge of its existence along with fairly typical histological features and the immunohistochemical expression pattern with intense positivity for CD34 allow the increasing diagnosis of this condition, which suggests that these cases were not previously diagnosed as such.
    METHODS: We report the case of a 43 year-old male with a painless nodule in the first left finger pad clinically suggestive of pyogenic granuloma or nodular melanoma, which was diagnosed by excisional biopsy and immunohistochemical study as a solitary fibrous tumor.
    CONCLUSIONS: Only 11 cases of cutaneous solitary fibrous tumor have been published in the following locations: head, cheek, thigh, chest, back and nose. Our work describes the first case of cutaneous solitary fibrous tumor in the hand. The solitary fibrous tumor derived from mesenchymal cells expresses CD34 and hence its presentation in any location. In our case it was in the hand. It explains the problems encountered in the clinical differential diagnosis with other tumors as nodular melanoma, pyogenic granuloma, giant cell tumor of tendon sheath, fibroma, benign peripheral nerve sheath tumors, etc. As we consider the histology, differential diagnosis should be made with other tumors that also express CD34.
    CONCLUSIONS: Solitary fibrous tumors derived from mesenchymal cells express CD34 and hence its presentation in any location. In our case it was in the finger pad.
    Antecedentes: el tumor fibroso solitario es un tumor poco común. Anteriormente se suponía que afectaba sólo la cavidad torácica, en especial la pleura; posteriormente, se relacionó con otras membranas serosas y se observó en diversas localizaciones extraserosas, entre ellas la piel. El conocimiento de este tumor, junto con el aspecto histológico característico y el patrón de expresión inmunohistoquímica con intensa positividad para CD34 permiten que cada vez se diagnostique con mayor frecuencia. Caso clínico: se comunica el caso de un varón de 43 años de edad con un nódulo indoloro en el pulpejo del primer dedo izquierdo, que sugería clínicamente un melanoma nodular o granuloma piógeno. Mediante biopsia excisional y estudio inmunohistoquímico se diagnosticó como tumor fibroso solitario. Discusión: hasta la fecha se han publicado 11 casos de tumores fibrosos solitarios cutáneos, localizados en cabeza, mejilla, muslo, pecho, espalda y vestíbulo nasal. El caso que se comunica constituye la primera lesión de estas características que afecta la mano. El diagnóstico clínico diferencial del tumor fibroso solitario incluye otros tumores como: melanoma nodular, granuloma piógeno, tumor de células gigantes tenosinovial, fibroma y tumor de vaina de nervio periférico benigno. En cuanto a la histología, se planteó el diagnóstico diferencial con otras neoplasias que también expresan CD34. Conclusiones: el tumor fibroso solitario deriva de células mesenquimatosas y expresa CD34, lo que explica su aparición en cualquier localización, como en este caso, que fue en el pulpejo del quinto dedo.
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