血管肌纤维母细胞瘤2例报道及文献复习

目的 探讨血管肌纤维母细胞瘤(AMF)的临床病理特征及诊断和鉴别诊断。方法 对2例AMF进行组织学观察和免疫组化S—P法标记，抗体为vimentin、desmin、SMA、S—100蛋白、CD34、ER、PR、CK等。结果 1例为30岁女性，表现为外阴囊肿；1例为51岁男性，表现为腹股沟区精索肿块。眼观：肿瘤境界均清楚；镜检：肿瘤均由相互交错分布的细胞密集区和细胞稀疏区组成，其间血管丰富，多为毛细血管至中等大薄壁血管。瘤细胞成巢或束状围绕血管周围排列。可见红细胞漏出。其中1例尚可见成群分布的脂肪细胞存在。免疫表型：瘤细胞vimentin(2／2)、ER(2／2)、PR(2／2)、SMA(1／2)、CD34(2／2)阳性，SMA(1／2)、S—100蛋白(1／2)灶性阳性，desmin、CK阴性。结论 AMF是一种少见的好发于外阴生殖道的间质肿瘤，可能来源于血管周具有多向分化潜能的干细胞，可向肌纤维母细胞分化。在组织形态上AMF应与侵袭性血管黏液瘤、富细胞性血管纤维瘤、浅表性血管黏液瘤、纤维上皮性间质息肉、梭形细胞脂肪瘤相鉴别。     

Spindle cell lipoma of the breast: A case report and literature review

Fine-needle aspiration cytology (FNA) was performed on a breast lump in a 66-year-old female, clinically thought to be a carcinoma. The cytological findings were reported as suspicious of malignancy. Subsequent histological examination showed a tumour macroscopically and microscopically identical with six other cases previously described as benign spindle cell tumours of the breast. This report highlights yet another diagnostic pitfall in FNA cytology of the breast. The presence of floret cells in this case has not been previously reported but supports the concept that this lesion is the same as a spindle cell lipoma, from which it is virtually indistinguishable on light microscopy. The lesion affects both mate and female breast, is predominantly single but may be multiple, and follows a benign course. Local excision is curative.     

Mixed endometrial stromal and smooth muscle tumor: Report of a case with focal anaplasia and early postoperative lung metastasis

A rare case of a mixed endometrial stromal and smooth muscle tumor arising in the uterus of a 74‐year‐old woman is reported. The patient underwent hysterectomy for an enlarging uterine mass, and a large intramural tumor, showing marked central hyaline necrosis with calcification, was found. The tumor consisted of an admixture of a low‐grade endometrial stromal sarcoma (ESS) and a fascicular proliferation of spindle cells suggesting smooth muscle differentiation, and a characteristic ‘star‐burst’ appearance was found. In the ESS region, there were a few small foci of anaplasia where large polygonal cells with atypical nuclei and abundant eosinophilic cytoplasm proliferated, and the proliferative activity was locally increased in these foci. A small metastatic nodule appeared in the lung nine months after the hysterectomy, and the resected metastatic lesion showed features of anaplastic spindle cell sarcoma which was immunoreactive for CD10 but not for smooth muscle markers. Mixed endometrial stromal and smooth muscle tumors should be regarded as malignant neoplasms with the potential for hematogenous metastasis, particularly when they contain foci of cellular anaplasia.     

Low-grade periductal stromal sarcoma of the breast with myxoid features: Immunohistochemistry

A 52-year-old woman was admitted with a painful right breast tumor measuring more than 20 cm in largest diameter, which ulcerated the overlying skin. The lesion had appeared 4 years previously but the patient hesitated to seek medical care due to 'fear of cancer'. Microscopically, the tumor was composed of spindle cells that formed cuffs around multiple open tubules and ducts set in an abundant, myxoid stroma. The spindle cells had significant atypia with nuclear pleomorphism, occasional cytoplasmic vacuolation and moderate mitotic activity. The ducts and lobules surrounded by the proliferating tumor cells had minimal distortion, with a pericanalicular growth pattern devoid of the phyllodes pattern. The tumor had a multinodular growth pattern with coalesced and individual tumor nodules, the latter being found mostly at the periphery of the lesion. On immunohistochemistry the tumor cells were positive for smooth muscle actin, CD34, and vimentin, and focally positive for CD10. A diagnosis of low-grade periductal stromal sarcoma (PDSS) with myxoid features was established. PDSS is a distinct low-grade breast sarcoma, the appropriate diagnosis of which requires extensive tumor sampling and additional broad immunohistochemistry. PDSS should not be confused with other spindle cell breast tumors because they require different treatment.     

Secretion of interleukin-6 and vascular endothelial growth factor by spindle cell sarcoma complicating Castleman's disease (so-called 'vascular neoplasia')

So-called 'vascular neoplasia' (VN) is a rare tumour of unknown origin that complicates hyaline vascular type Castleman's disease (CD). This paper reports a case of VN complicating CD of hyaline vascular type, in which neoplastic cells were shown to secrete interleukin-6 (IL-6) and vascular endothelial growth factor (VEGF). In this case, VN first occurred in the retroperitoneum of a 60-year-old male. The lesion showed typical morphology, with three distinct areas: (1) a lymph node-like area with regressively transformed lymph follicles showing hyaline vascular changes and with a hypervascular interfollicular region filled with slit-like vascular channels; (2) an area composed of spindle cell sarcoma; and (3) an area showing angiolipomatous hamartoma. A proportion of the cells in the spindle cell area showed severe pleomorphism. Subcutaneous recurrence after 8 months was composed purely of pleomorphic spindle cells. A karyotypic analysis of the recurrent tumour showed 47, XXY with some instability. Supernatant from primary culture contained high levels of IL-6 and VEGF, suggesting high secretion of these cytokines from neoplastic cells. Immunohistochemically, p53 overexpression was identified only in the pleomorphic spindle cells of the primary lesion and metastatic tumour. No features suggestive of vascular origin were shown on immunohistochemical or electron microscopic analysis of the neoplastic cells. Human herpesvirus type 8 was not detected by immunohistochemistry or PCR analysis. High levels of IL-6 and/or VEGF have been reported to play a role in CD. This is the first case report that clarifies the site of such cytokine production, showing the possibility of CD as a paraneoplastic phenomenon.     

Plexiform spindle cell naevus: a distinctive variant of plexiform melanocytic naevus

Twelve cases of a unique plexiform melanocytic naevus that we have termed plexiform spindle cell naevus are reported. The lesions affected young individuals (mean age 22.5 years) of both sexes and were most frequently located on the shoulders and back. The lesions clinically were slightly raised and blue or darkly pigmented, suggesting blue naevus. Histologically these tumours had a symmetrical wedge-shaped configuration, as seen in typical Spitz naevus, with the apex directed toward the deep reticular dermis or subcutis. The pigmented spindle cells were disposed in fascicles in association with neurovascular bundles and adnexal structures, imparting a plexiform architecture to the lesion. The predominant cell type consisted of spindle cells containing a granular melanin and elongated nuclei. Low-grade cellular atypia was commonly noted. Varying numbers of epithelioid cells were observed in most of the cases. In two cases studied, the naevus cells showed S-100 protein and HMB-45 immunoreactivity. The differential diagnosis of plexiform spindle cell naevus includes malignant melanoma, and spindle and epithelioid cell (Spitz) naevus, blue naevus and combined naevus. Plexiform spindle cell naevus is a distinctive type of pigmented spindle naevus distinguished from the above entities by its striking plexiform architecture, predominance of melanin-containing spindle cells and lack of significant cellular atypia.     

Unusual subcutaneous mixed tumour exhibiting adipose, fibroblastic, and epithelial components.

A subcutaneous mass in the left supraclavicular fossa of a 55-year-old woman proved on histological examination to be composed of islands of squamous cells embedded in bands of spindle cells and associated with mature adipose tissue. Electron microscopy showed that the spindle cells were fibroblastic in nature and not squamous cells showing spindle differentiation. There was also a minor lymphangiomatous component and sparse infiltrates of lymphoid cells. The lesion bore certain similarities to thymolipoma except that the presence of spindle cells and the site were atypical. While it may be speculated that the tumour was thymolipoma occurring in an ectopic cervical thymus the lesion is provisionally regarded as an unusual mixed tumour featuring mesenchymal and epithelial components.     

Renal capsuloma: description of a case with predominantly muscular differentiation

The Authors report a case of Renal Capsuloma with a histologic biphasic pattern constituted of epithelioid and spindle cells. The lesion is observed in a nephrectomy specimen as incidental finding concurrent with a Papillary Transitional Cell Carcinoma of calices and renal pelvis. The lesion presents a distinct muscular immunophenotype (actin+, desmin+), with coexpression of the melanogenesis marker (HMB45+) in a lower cellular population. This combined immunophenotype is a characteristic feature of the Perivascular Epithelioid Cell's (PEC) lesions. Actually Renal Capsuloma is a PEC's lesion (Pecoma) closely related to Angiomyolipoma (AML). Particularly Capsuloma is thought as a variant of AML predominantly composed by muscular spindle cells (monotypic spindle cell-leiomyoma like variant of AML). In the presented case the spindle cell component is associated with an epithelioid cellular population which also shows a distinct muscular differentiation. The Authors have not found in the literature the association between Renal Capsuloma and Papillary Transitional Cell Carcinoma of the renal pelvis.     

Uterine leiomyoma with massive lymphoid infiltration: case report

Uterine leiomyoma with massive lymphoid infiltration is a rare and unusual pathological finding; only 20 cases have been reported in the literature. We report a case of this unusual lesion in a 35-year-old woman who underwent a myomectomy. On gross examination, the tumor was of white color and firm consistency. Histological sections showed interlacing bundles of spindle shaped cells of low density with moderate to severe lymphocytic infiltrate associated to lymphoid follicles and few plasma cells. Immunohistochemically, the diffusely infiltrating lymphoid cells were predominantly of T cell phenotype. The interspersed spindle shaped cells were positive with alpha smooth muscle actin, desmin and h-caldesmon. The cause of this unusual lesion is not clear, but the recognition of its distinct histological features is important to avoid possible confusion with differential diagnosis including malignant lymphoma, inflammatory pseudotumor and pyomyoma.     

青春期前型外阴纤维瘤临床病理学观察

目的 探讨青春期前型外阴纤维瘤(PVF)的临床病理学特征、免疫表型、诊断和鉴别诊断.方法 分析2例PVF的临床表现、病理形态和免疫表型,并复习文献.结果 1例发生于8.5岁幼女,1例发生于54岁成年女性,均以右侧大阴唇复发性肿块就诊.大体上,肿块不明显,切面呈灰白色,纤维组织样.组织学上,病变位于真皮层内,周界不清.细胞密度低,由稀疏的梭形纤维母细胞样细胞和大昔的胶原纤维样间质组成.梭形细胞无异型性,核分裂象罕见.病变向皮下组织延伸,在邻近的脂肪组织之间、血管之间或在神经周围穿插性生长,形成类似错构瘤样的结构.免疫组织化学标记显示,梭形细胞主要表达波形蛋白,部分弱阳性表达CD34,不表达α平滑肌肌动蛋白、肌特异性肌动蛋白、结蛋白、高分子钙调蛋白(h-caldesmon)、CD99、S-100蛋白、bcl-2、β-catenin、ER和PR.结论 PVF是一种好发于青春期前幼女外阴的良性间叶性病变,偶可发生于成年人.PVF可能代表了一种外阴正常间叶组织的过度增生.临床上近1/3的病例如切除不净可发生局部复发,偶可自发性消退.     

Suprasellar spindle cell lipoma

Spindle cell lipoma of the suprasellar region has not been reported in the literature. We report a case of a 4-year-old male with a suprasellar spindle cell lipoma. The patient presented with headaches and visual disturbances. An imaging study revealed a 4 to 5-mm suprasellar mass that was initially thought to be a lipoma or dermoid cyst. Pathologic examination from the partial resection showed a circumscribed lesion consisting of mature adipose tissue with intermixed uniform spindle cells associated with a mucoid matrix, morphologically consistent with a spindle cell lipoma. The spindle cells and vasculature demonstrated focal positivity with CD34 antibody. There was no evidence of lipoblasts, mitoses, necrosis, or osseous tissue.     

Intranodal palisaded myofibroblastoma – a rare case report and literature review

Intranodal palisaded myofibroblastoma (IPM) is a benign mesenchymal neoplasm originating from smooth muscle cells and myofibroblasts. The inguinal region is the most common site of this rare tumor. As there are only about 89 such cases reported in the literature, the precise etiology and pathogenesis have yet to be explained adequately. It is characterized by spindle cells, amianthoid fibers, and by the proliferation of hemosiderin‐containing histiocytes in the lymph node. A nodular lesion was excised from the inguinal region of a 47‐year‐old female patient with the clinical diagnosis of lymphoma and/or metastase. Macroscopic examination of a section of the lesion demonstrated a solid appearance. Microscopic examination revealed spindle‐cell proliferation, amianthoid fibers, hemosiderin pigment, and extravasated erythrocytes. Nuclei of the spindle cells displayed a palisaded appearance. Compressed lymphoid tissue was observed around the lesion. Neoplastic cells were identified by the presence of vimentin, SMA, Cyclin D1, and beta‐catenin. The Ki67 index was less than 1%. Histological examination confirmed the diagnosis of IPM. Although IPM is benign, it is frequently confused with metastatic lesions and lymphomas.     

Gastrointestinal haemorrhage from a jejunal gangliocytic paraganglioma.

A case of jejunal gangliocytic paraganglioma is reported in a 54 year old woman who presented with brisk melaena. The tumour was not encapsulated, involved the mucosa and submucosa, and was composed of epithelial nests, spindle cells, and ganglion cells. These cells were mixed, giving carcinoid-like, paraganglioma-like, and ganglioneuroma-like patterns in different areas of the tumour. The lesion was excised locally and recovery was uneventful. Only four previous cases have been reported at this site.     

Nontuberculous mycobacteria‐associated spindle cell pseudotumor of the nasal cavity: A case report

Mycobacterial spindle cell pseudotumor (MSP) is a rare mass‐forming lesion caused by mycobacterial infection, mostly in immunocompromised patients. Since it is composed of a proliferation of spindle‐shaped fibrohistiocytic cells without forming epithelioid cell granulomas, histological distinction from other spindle cell lesions is often difficult and its pathophysiology is poorly understood. MSP arising in the nasal cavity is extremely rare, and only two cases have been reported previously. Here we report a case of MSP of the nasal cavity in an 83‐year‐old man with no evidence of immunodeficient state. The resected tumor consisted of spindle cells, which contained numerous acid‐fast bacilli in the cytoplasm. By polymerase chain reaction and sequencing using DNA extracted from the paraffin sections, the bacilli were identified as Mycobacterium intracellulare. Immunohistochemistry revealed that the spindle cells were positive for CD68, CD11c and S100 protein, confirming the histiocytic nature of these cells. They were also positive for CD163 and CD204, suggesting that they showed a phenotype similar to alternatively activated (M2) macrophages and the phenotype might contribute to the maintenance of mycobacterial infection despite apparent immunocompetence of the host.     

Proliferative fasciitis of the forearm: Case report with immunohistochemical, ultrastructural and DNA ploidy studies and a review of the literature

A case of prollferative fasclitis arising In the left forearm of a 56-year-old man was examined. The lesion was preceded by blunt trauma, measured 1.5 times 1.3 times 1.0 cm, was poorly circumscribed and appeared white to light gray on the cut surface. Light microscopic examinations revealed that spindle cells and giant cells with one or two nuclei and abundant basophllic cytoplasm ware arranged without any organized patterns In collagenous stroma. Ultrastructurally, well-developed rough endoplasmic reticulum separated by varying amounts of fine to course fibrillar materials was detected in the giant cells. Only vimentin lmmunoreactivity was detected In both spindle and gtant cells. The Ki-67 labeling Index of spindle cells was 35% but that of giant cells was less than 5%, and this reflects the quiescent or slow-growing features of these giant cells in proliferative fasclitis. DNA content of the calls, which was examined by Image cytometry, demonstrated diploidy In both spindle (DNA Index = 1.01) and glant (DNA Index = 1.09) calls.     

Benign and malignant neoplasms of myoepithelial cells: Cytologic findings

We report two myoepithelial cell neoplasms; a salivary gland tumor was malignant and a breast neoplasm was benign. Both were studied histologically, immunohistochemically, cytologically, and ultrastructurally. The malignant myoepithelioma recurred twice and metastasized to one regional lymph node. This tumor was infiltrative with areas of necrosis and hemorrhage. It was composed of malignant-appearing spindle and plasmacytoid cells. Both types of cells were immunoreactive to muscle specific actin, S-100 protein, cytokeratin, vimentin, and neuron-specific enolase. Ultrastructurally, features of myoepithelial cells were seen. Fine-needle aspirate smears showed spindle and plasmacytoid cells, numerous mitoses, and malignant-appearing nuclei. Spindle-cell adenomyoepithelioma of the breast, a small well-circumscribed firm nodule, featured multiple lobules of spindle cells associated with clear-cell glands at the tabular periphery. Histologically and cytologically, the lesion was cellular but appeared benign. The differential diagnosis of myoepithelial neoplasms is discussed.     

Duodenal Periampullary Gangliocytic Paraganglioma: Report of Two Cases with Immunohistochemical and Ultrastructural Study

We report two cases of Gangliocytic Paraganglioma (GP) of the ampulla of Vater occurring in a 63-year-old and a 34-year-old individual. The patients were both admitted for a long history of intermittent gastrointestinal bleeding and abdominal discomfort, with no other symptoms. At endoscopy, the GP appeared as a polypoid, ulcerated mass in the ampullar region, measuring 2.5x1.8 and 2 cm, respectively. Microscopically, the tumors showed similar features and were composed of epithelial cells (more than 50%), spindle cells, and ganglion-like cells. The epithelial cells showed clear cytoplasm and formed nests (zellballen or paraganglioma-like groups), and less frequently, cords (carcinoid-like), extending to mucosa and submucosa. Ganglion cells were sparse, constantly associated with the spindle cells. Both epithelial and ganglion cells were synaptophysin, chromogranin A, and anti-neurofilament immunoreactive. The spindle cells were all S-100 positive. Ultrastructural studies revealed dark and light cells, rare elongated cellular processes, secretory granules, and fine fibrils resembling neurofilaments. The histogenesis of GP is still a matter of debate, however its neoplastic nature is supported by the occasionally reported malignant evolution.     

Spindle cell haemangioendothelioma: a clinicopathological and immunohistochemical study indicative of a non-neoplastic lesion

Spindle cell haemangioendothelioma is an uncommon vascular lesion, of which 35 cases have been previously reported. A further 20 cases are reported herein. These lesions affected a wide age range in both sexes, and showed a predilection for the extremities. Twelve patients had multiple lesions and, in these cases, local progression over many years but no true recurrence was typical. None of the 20 patients developed metastases, but one later developed an angiosarcoma. Individual cases were associated with congenital lymphoedema. Klippel-Trenaunay syndrome and early-onset varicose veins. Histologically, in addition to the admixture of cavernous spaces and solid spindle cell/epithelioid cell areas, the presence both of irregularly distributed and perivascular smooth muscle cells and of malformed variably-sized vessels at the periphery of almost every lesion was noted in each case. Reticulin staining, immunohistochemistry and electronmicroscopy revealed the presence of primitive vessel formation and partial endothelial differentiation in the solid spindle cell areas. Combining these data with those from previously published series, it is suggested that spindle cell haemangioendothelioma is a non-neoplastic lesion (rather than a borderline malignancy as it is currently regarded) and that its development correlates with histological and/or clinical evidence of a malformed vasculature at the affected site.     

So-called pseudosarcoma of the esophagus: nodal metastases of the spindle cell element

A polypoid lesion of the esophagus with all the morphological features of a so-called pseudosarcoma produced nodal metastases of the spindle cell element. Ultrastructurally, these spindle cells showed only fibroblastic features. The findings in this case and a review of cases reported as pseudosarcoma of the esophagus lend little support for the contention that they differ from cases categorized as carcinosarcoma of the esophagus.     

A successfully treated inflammatory myofibroblastic tumor of the mandible with long-term follow-up and review of the literature

Inflammatory myofibroblastic tumor (IMT) of the oral cavity is an extremely rare clinical and pathological disease entity. It was originally described in the lung but has recently been reported in various anatomic sites. We report such a case of inflammatory myofibroblastic tumor of the mandible in a 14-year-old girl. The patient presented with an aggressive ulcerative soft tissue mass of 3 months duration in the mandibular molar gingiva. Histologically, the lesion was composed of fibroblastic or myofibroblastic spindle cell proliferations with infiltrative margins in an inflammatory background. Immunohistochemically, the fibroblastic or myofibroblastic spindle cells were positive for vimentin, α-smooth muscle actin, and Ki-67 (MIB-1) but negative for desmin, pan-cytokeratin, S-100 protein, CD34, CD68, CD99, bcl-2, β-catenin, estrogen receptor, progesterone receptor, ALK-1, and p53. These spindle cells were focally and weakly Ki-67- (MIB-1-) positive. The MIB-1 labeling index was 5%. The results of in situ hybridization for Epstein-Barr virus-encoded-RNA were negative. The ratio of IgG4+/IgG+ plasma cells was about 10%. A pathological diagnosis of inflammatory myofibroblastic tumor was made. The postoperative course was uneventful, and the patient has had no recurrence in the 10-year follow-up period. Although no evidence of oral inflammatory myofibroblastic tumor recurrence or malignant transformation has been reported, it has been observed that in inflammatory myofibroblastic tumors of other regions, a prolonged follow-up is necessary after surgical resection. No other case of an IMT patient under 20 years of age has appeared in either the English or the Japanese literature.