博文《漫谈“成纤维细胞”》和《助纣为虐的纤维母细胞——肿瘤相关纤维母细胞》对纤维母细胞的形态和功能进行了叙述，本文对纤维母细胞/肌纤维母细胞来源肿瘤进行归纳总结。曾经对“巨噬细胞/组织细胞”的功能及其来源的肿瘤进行了总结（见博文《七十二变”的巨噬细胞》、《巨噬细胞的胡作非为》、《“巨噬细胞”续集——漫谈“巨噬细胞”与“淋巴瘤”》、《“巨噬细胞”大结局——淋巴瘤相关巨噬细胞》）。按照博文的风格，本文以《“纤维母细胞”大结局——纤维母细胞的胡作非为》为题。

在刘彤华主编的第三版《诊断病理学》一书中，鼻咽血管纤维瘤归为良性肿瘤，它具有局部侵袭性，因此在2016年的《Diagnostic Pathology: Soft Tissue Tumors》一书中将其归入中间性肿瘤（局部侵袭性），WHO软组织肿瘤分类书上未收入此疾病。软组织血管纤维瘤目前认为是纤维母细胞起源的肿瘤，2013年软组织肿瘤分类中未将该肿瘤收录，2016年的《Diagnostic Pathology: Soft Tissue Tumors》一书有介绍。2013年WHO软组织肿瘤分类中，纤维母细胞/肌纤维母细胞来源的中间性肿瘤增加了巨细胞纤维母细胞瘤和隆突性皮肤纤维肉瘤疾病。

纤维母细胞/肌纤维母细胞来源肿瘤主要有如下疾病：

良性肿瘤

良性肿瘤有结节性筋膜炎、颅骨筋膜炎、血管内筋膜炎、增生性筋膜炎、增生性肌炎、缺血性筋膜炎、嗜酸性筋膜炎、局灶性肌炎、器官相关性假肉瘤性肌纤维母细胞性增生、瘢痕疙瘩、腱鞘纤维瘤、皮肤多形性纤维瘤、弹力纤维瘤、钙化性纤维性肿瘤、钙化性腱膜纤维瘤、项纤维瘤、Gardner纤维瘤、婴儿纤维性错构瘤、胶原纤维瘤、乳腺型肌纤维母细胞瘤、血管肌纤维母细胞瘤、细胞性血管纤维瘤、软组织血管纤维瘤、巨细胞血管纤维瘤、栅栏状肌纤维母细胞瘤。

中间性肿瘤（局部侵袭性）

具有局部侵袭性的中间性肿瘤有纤维瘤病（包括颈纤维瘤病、婴幼儿指（趾）纤维瘤病、幼年性透明变性纤维瘤病、脂肪纤维瘤病、阴茎纤维瘤病、手掌及足底纤维瘤病、腹壁纤维瘤病、腹内纤维瘤病、放射后纤维瘤病、瘢痕性纤维瘤病）、巨细胞纤维母细胞瘤和鼻咽血管纤维瘤。

中间性肿瘤（偶见转移型）

偶见转移型的中间性肿瘤有孤立性纤维性肿瘤、炎性肌纤维母细胞肿瘤、低度恶性肌纤维母细胞肉瘤、黏液样炎性纤维母细胞肉瘤、隆突性皮肤纤维肉瘤、婴儿型纤维肉瘤。

恶性肿瘤

恶性肿瘤有成人纤维肉瘤、黏液纤维肉瘤、低度恶性纤维黏液样肉瘤、硬化性上皮样纤维肉瘤。

巨细胞纤维母细胞瘤（Giant cell fibroblastoma，GCFB）

                  ------摘自《Diagnostic Pathology: Soft Tissue Tumors》

Definitions

• Locally aggressive fibroblastic neoplasm, predominantly of childhood and adolescence, 

  that classically features multinucleated giant cells lining pseudovascular spaces

○ Histologically and genetically related to dermatofibrosarcoma protuberans (DFSP)

Epidemiology

• Incidence

○ Rare

• Age

○ Most arise in children (median: 6 years)

○ 75% occur before age 20

○ Occasionally may occur in adults

• Sex

○ 2:1 male predominance

Site

• Arises in dermis &/or subcutaneous tissue

• Most common on trunk

• Infrequent on extremities, head/neck region

Presentation

• Superficial, often protuberant mass

○ May be polypoid

• Slow growing, often painless

• May arise in site of previous DFSP excision

Treatment

• Wide surgical excision with margins

Prognosis

• Local recurrence in up to 50%

○ Often related to marginal or incomplete excision

• No documented reports of metastasis in histologically pure GCFB

○ Hybrid GCFB/DFSP tumors behave more like DFSP

General Features

• Poorly defined, superficial lesion

• Tan-gray to yellow mucoid cut surface

Size

• Mean: 3.5 cm

Histologic Features

• Infiltrative growth

○ Adnexal structures entrapped but not destroyed

○ Often shows "honeycomb" pattern of fat infiltration

• Hypocellular proliferation of small spindled cell with bland, wavy nuclei within myxoid

  to collagenous stroma

○ Areas of increased cellularity can be seen

• Variable number of multinucleated giant cells in stroma

○ These cells also characteristically line irregular, cleft-like pseudovascular spaces

• Mitoses rare; necrosis absent

• Intralesional hemorrhage may be present

• Perivascular chronic inflammatory infiltrate not uncommon

• Minority of cases contain areas of conventional DFSP

(hybrid DFSP/GCFB)

○ May be present in primary tumor or in recurrence

○ Rare pigmented cells, myoid nodules, or fibrosarcomatous change

Immunohistochemistry

• CD34(+) in spindled and multinucleated giant cells

• Negative for S100 protein, SMA, desmin, keratin

Molecular Genetics

• Characteristic t(17;22) leading to COL1A1-PDGFB fusion protein

Differential Diagnosis

• Dermatofibrosarcoma Protuberans (DFSP)

• Angiosarcoma

• Dermatofibroma (Fibrous Histiocytoma)

• Pleomorphic Lipoma

• Myxofibrosarcoma

References 

[1]. Macarenco RS et al: Genomic gains of COL1A1-PDFGB occur in the histologic evolution of giant cell fibroblastoma into dermatofibrosarcoma protuberans. Genes Chromosomes Cancer. 47(3):260-5, 2008

[2]. Jha P et al: Giant cell fibroblastoma: an update and addition of 86 new cases from the Armed Forces Institute of Pathology, in honor of Dr. Franz M.Enzinger. Ann Diagn Pathol. 11(2):81-8, 2007

[3]. Terrier-Lacombe MJ et al: Dermatofibrosarcoma protuberans, giant cell fibroblastoma, and hybrid lesions in children: clinicopathologic comparative analysis of 28 cases with molecular data-a study from the French Federation of Cancer Centers Sarcoma Group. Am J Surg Pathol. 27(1):27-39, 2003

Angiofibroma of Soft Tissue（软组织血管纤维瘤）

          ------摘自《Diagnostic Pathology: Soft Tissue Tumors》

Definitions

• Low-grade fibroblastic neoplasm featuring a prominent and

complex stromal vasculature

Epidemiology

• Age

○ Wide range (median: 49 years)

• Sex

○ More common in females (2:1)

Site

• Superficial or deep soft tissues of extremities (lower limb

most common)

○ Also back, pelvis, chest wall, and abdominal wall

Presentation

• Slow-growing, painless mass

Treatment

• Simple conservative excision

Prognosis

• Benign

• Rarely recurs, even with incomplete excision

General Features

• Well circumscribed

• Firm, rubbery cut surface

Size

• 1-12 cm (mean: 4.3 cm)

Histologic Features

• Generally well circumscribed, ± fibrous capsule

• Cellularity varies widely

• Bland, uniform spindle cells in a variably myxoid to

collagenous stroma

○ Rare degenerative mild nuclear atypia

• Very prominent and complex stromal vasculature

○ Vessels range from small and thin-walled to medium and

large ectatic channels

○ Variable perivascular hyalinization or fibrosis

• Stromal chronic inflammatory infiltrate common

• Mitoses uncommon

Immunohistochemistry

• Focal EMA(+) in 1/2 of cases

• SMA, CD34, and desmin usually negative or at most focally positive

○ CD34 highlights prominent vascular network

• Tumors cells negative for S100 protein, CD31, STAT6

Molecular Genetics

• Recurrent t(5;8) (p15;q13) translocation resulting in fusion

ofAHRR and NCOA2 genes

Differential Diagnosis

• Low-Grade Fibromyxoid Sarcoma

• Myxoid Liposarcoma 

• Solitary Fibrous Tumor

• Lobular Capillary Hemangioma (Pyogenic Granuloma)

• Myxofibrosarcoma (Low Grade)

• Deep Benign Fibrous Histiocytoma

本文允许在互联网范围内转载，使用时请注明作者姓名、作品名称及作品来源。