Ronchi Andrea, La Mantia Elvira, Gigantino Vincenzo, Perdonà Sisto, De Sio Marco, Facchini Gaetano, Franco Renato, De Chiara Annarosaria
Pathology Unit, Università della Campania "L. Vanvitelli", Via Luciano Armanni, 80138, Naples, Italy.
Pathology Unit, Istituto Nazionale Tumori I. R. C. C. S. "Fondazione Pascale", Naples, Italy.
Diagn Pathol. 2017 Jul 7;12(1):50. doi: 10.1186/s13000-017-0640-5.
Solitary fibrous tumor is an uncommon soft tissue neoplasm with intermediate biological behavior, which rarely metastasizes. Malignant solitary fibrous tumor, although not clearly defined, is rarely described in the prostate. The present case is characterized by some peculiarities if compared with previously reported cases of prostatic malignant solitary fibrous tumor. Firstly, it does not show a homogeneous morphology: part of the neoplasm (about 50%) showed the features of a conventional solitary fibrous tumor, while the remaining part showed the features of a malignant solitary fibrous tumor. In addition, the case is the first malignant solitary fibrous tumor reaching a huge diameter of 20 cm and replacing all prostatic parenchyma. Interestingly, normal prostatic parenchyma was observed on left-lobe trans-rectal needle-core biopsies, but was totally absent in surgical specimen. Since radical prostatectomy was carried out about 4 months after the biopsies, such discordant data may suggest exceedingly rapid growth of the neoplasm.
We report a case of a 62-year-old male, presented at medical observation for urinary retention, constipation and an enlarged prostate gland. A trans-rectal prostatic biopsy showed a low-grade spindle cell neoplasm. Histopathological examination of the prostatectomy specimen showed patternless architecture with hypocellular and hypercellular areas and hemangiopericytoma-like vessels. In some fields the neoplasm was characterized by a high mitotic index and evident cellular atypia. Immunohistochemically, neoplastic cells were positive for CD34, bcl2, CD99, STAT6 and partially for PgR. The neoplasm was diagnosed as a malignant solitary fibrous tumor.
The differential diagnosis of spindle cells tumors arising in the prostrate is broad and includes lesions of epithelial and mesenchymal origin, primary prostatic lesions such as stromal tumors of uncertain malignant potential and stromal sarcoma, as well as anatomically ubiquitous soft tissue neoplasms. Solitary fibrous tumors should be considered in cases of prostatic tumors with a spindled morphology, but malignancy in such tumors is extremely rare in the prostate. A review of literature showed only four additional cases. Because of the unpredictable biological behavior and the possibility of recurrence, a long-term clinical and instrumental follow-up is recommended.
孤立性纤维瘤是一种罕见的软组织肿瘤,具有中间型生物学行为,很少发生转移。恶性孤立性纤维瘤虽然尚无明确定义,但在前列腺中很少见。与先前报道的前列腺恶性孤立性纤维瘤病例相比,本病例具有一些独特之处。首先,它没有呈现出均匀的形态:肿瘤的一部分(约50%)表现出传统孤立性纤维瘤的特征,而其余部分表现出恶性孤立性纤维瘤的特征。此外,该病例是首例直径达20厘米的巨大恶性孤立性纤维瘤,取代了整个前列腺实质。有趣的是,经直肠左叶针芯活检发现有正常前列腺实质,但手术标本中却完全没有。由于活检后约4个月进行了前列腺根治术,这种不一致的数据可能提示肿瘤生长极为迅速。
我们报告一例62岁男性,因尿潴留、便秘和前列腺肿大前来就医。经直肠前列腺活检显示为低级别梭形细胞瘤。前列腺切除标本的组织病理学检查显示结构无规律,有细胞稀少区和细胞丰富区以及类似血管外皮细胞瘤的血管。在一些区域,肿瘤的特征是有高有丝分裂指数和明显的细胞异型性。免疫组化显示,肿瘤细胞CD34、bcl2、CD99、STAT6阳性,部分PgR阳性。该肿瘤被诊断为恶性孤立性纤维瘤。
前列腺中出现的梭形细胞肿瘤的鉴别诊断范围广泛,包括上皮和间叶来源的病变、原发性前列腺病变,如恶性潜能不确定的间质肿瘤和间质肉瘤,以及解剖学上普遍存在的软组织肿瘤。前列腺肿瘤呈梭形形态时应考虑孤立性纤维瘤,但此类肿瘤在前列腺中恶性极为罕见。文献回顾仅发现另外4例。由于其生物学行为不可预测且有复发的可能,建议进行长期的临床和影像学随访。