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- Adult Fibrosarcoma (FS): A Reevaluation of 154 Putative Cases
Adult Fibrosarcoma (FS): A Reevaluation of 154 Putative Cases Diagnosed at a Single Institution over a 48 Year Period
A Bahrami, AL Folpe. Mayo Clinic, Rochester, MN.
Background: Adult FS, defined by the WHO as a “malignant tumor, composed of fibroblasts… and, in classical cases, a herringbone architecture” was once considered the most common adult sarcoma. Currently FS is regarded as a diagnosis of exclusion, representing only 1-3% of adult sarcomas. This trend is likely due to: 1) widespread use of immunohistochemistry (IHC) and molecular diagnostic techniques, 2) recognition of distinct FS subtypes, and 3) distinction of FS from undifferentiated pleomorphic sarcoma (UDPS). However, no recent series has critically re-evaluated putative FS, to estimate their true incidence.
Design: 178 cases diagnosed as adult FS in soft tissue locations were retrieved from
our institutional archives for the period 1960-2008. 24 cases with insufficient material were excluded. Based on the morphology of the final 154 cases, IHC was performed using some combination of: wide-spectrum CK, EMA, high molecular weight CK,
S100, Melan A, HMB-45, CD34, CD31, TLE1, smooth muscle actin, desmin, Myo-D1,
myogenin, c-kit, INI1, calretinin, WT1 and TTF1. Revised diagnoses were based on
clinical, morphological, and IHC findings.
Results: The original group of putative FS occurred in 81 M and 73 F (median 53
years, range 2-99 years), and involved the legs (50 cases), head/neck (30 cases), thorax/abdomen (22 cases), arms (21 cases), mediastinum (8 cases), abdomen/ retroperitoneum/pelvis (13 cases), and lung (10 cases). Only 22 cases met WHO criteria for FS. These occurred in 13 M and 9 F (median 49 years, range 6-74 years), and involved the legs (11 cases), head/neck (6 cases), thorax/abdomen (2 cases) mediastinum (2 cases), and arm(1 case). Non-FS (132 cases) were reclassified as: UDPS (38 cases), synovial sarcoma(21 cases), SFT (14 cases), myxo-FS (10 cases), MPNST (6 cases), DFSP, desmoplastic melanoma (4 cases each), LG fibromyxoid sarcoma, sarcomatoid CA, desmoid, RMS, myofibroblastic sarcoma, spindle cell liposarcoma (3 cases each), sclerosing epithelioid FS, fibroma-like epithelioid sarcoma, leiomyosarcoma, cellular fibrous histiocytoma (2 cases each), and other (9 cases).
Conclusions: Using modern diagnostic criteria and ancillary IHC we have been able
to reclassify 87% of putative FS. Exclusive of UDPS, the distinction of which from FS
is subjective, 61% of putative FS were reclassified, most commonly as monophasic
synovial sarcoma and solitary fibrous tumor. We conclude that true FS is exceedingly
rare, accounting for <0.1% of ∼18,000 adult soft tissue sarcomas seen at our institution during this time period, and should be diagnosed with great caution
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本帖最后由 于 2010-04-05 16:36:00 编辑
Adult Fibrosarcoma (FS): A Reevaluation of 154 Putative Cases
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Adult Fibrosarcoma (FS): A Reevaluation of 154 Putative Cases Diagnosed at a Single Institution over a 48 Year Period
成人型纤维肉瘤（RS）：一个研究结构48年期间154例有争议病例的重新评价
A Bahrami, AL Folpe. Mayo Clinic,
Background: Adult FS, defined by the WHO as a “malignant tumor, composed of fibroblasts… and, in classical cases, a herringbone architecture” was once considered the most common adult sarcoma. Currently FS is regarded as a diagnosis of exclusion, representing only 1-3% of adult sarcomas. This trend is likely due to: 1) widespread use of immunohistochemistry (IHC) and molecular diagnostic techniques, 2) recognition of distinct FS subtypes, and 3) distinction of FS from undifferentiated pleomorphic sarcoma (UDPS). However, no recent series has critically re-evaluated putative FS, to estimate their true incidence.
背景：成人型FS，WHO定义为“由纤维母细胞组成的恶性肿瘤…，经典型病例呈鱼骨样结构”曾被认为是最常见的发生于成人的肉瘤。目前FS是作为一种排除性诊断，仅占成人肉瘤的1-3%。这种趋势可能是由于1）免疫组化和分子诊断技术的广泛应用，2）独特的FS亚型的确认和3）FS与未分化多形性肉瘤（UDPS）的区分。然而，近来没有系列研究对有争议的FS进行严格的重新评价而评估其真正的发生率。
Design: 178 cases diagnosed as adult FS in soft tissue locations were retrieved from
our institutional archives for the period 1960-2008. 24 cases with insufficient material were excluded. Based on the morphology of the final 154 cases, IHC was performed using some combination of: wide-spectrum CK, EMA, high molecular weight CK, S100, Melan A, HMB-45, CD34, CD31, TLE1, smooth muscle actin, desmin, Myo-D1, myogenin, c-kit, INI1, calretinin, WT1 and TTF1. Revised diagnoses were based on clinical, morphological, and IHC findings.
设计：收集我们研究结构在1960-2008年间诊断的软组织部位的成人FS178例。24例因标本量不足而除去。根据最后的154例病例形态学，我们使用了下面一组免疫标记：光谱CK、EMA、HMW-CK、S100、, Melan A、HMB-45、CD34、CD31、TLE1、SMA、desmin、Myo-D1、myogenin、c-kit、IN1、calretinin、WT1和TTF1。根据临床、形态学和IHC结果对原来的诊断进行了修改。
Results: The original group of putative FS occurred in
结果：原来诊断的FS男性81例，女性73例（发病年龄2-99岁，平均年龄53岁），发病部位分别为腿部（50例）、头/颈部（30例）、胸/腹腔（22例）、胳膊（21例）、纵膈（8例）、腹部/后腹膜/盆腔（13例）和肺（10例）。仅有22例符合WHO FS诊断标准。男性13例，女性9例（发病年龄6-74岁，平均49岁）。发生部位分别为腿部（11例）、头/颈部（6例）、胸/腹腔（2例）、纵膈（2例）和胳膊（1例）。132例非纤维肉瘤被重新分类为未分化多形性肉瘤（28例）、滑膜肉瘤（21例）、SFT（14例）、粘液性纤维肉瘤（10例）、MPNST（6例）、DFSP和促纤维增生性黑色素瘤（各4例），低级别纤维黏液样肉瘤、肉瘤样癌、硬纤维瘤、横纹肌肉瘤、肌纤维母细胞肉瘤、梭形细胞脂肪肉瘤（各3例），硬化性上皮样纤维肉瘤、纤维瘤样上皮样肉瘤、平滑肌肉瘤、富于细胞性纤维组织细胞瘤（各2例）以及其他肿瘤（9例）。
Conclusions: Using modern diagnostic criteria and ancillary IHC we have been able to reclassify 87% of putative FS. Exclusive of UDPS, the distinction of which from FS is subjective, 61% of putative FS were reclassified, most commonly as monophasic synovial sarcoma and solitary fibrous tumor. We conclude that true FS is exceedingly rare, accounting for <0.1% of ∼18,000 adult soft tissue sarcomas seen at our institution during this time period, and should be diagnosed with great caution
结论：使用现代诊断标准和免疫组化辅助技术，我们能对87%有争议饿FS重新分类。除了与未分化多形性肉瘤的区分是主观外，61%有争议的FS被重新分类，其中最常见为单向性滑膜肉瘤和孤立性纤维性肿瘤。我们认为真正的FS非常罕见，在此段时间内我们单位诊断的18,000成人软组织肉瘤的<0.1%。诊断FS应特别谨慎。
