乳腺上皮样型管周间质肉瘤病理特征及与叶状肿瘤比较观察

目的 探讨乳腺上皮样型管周间质肉瘤的临床病理特点及与叶状肿瘤的关系。方法 采用HE、特殊染色、免疫组化染色(CK，EMA，S-100蛋白，SMA，Vim，Des，MG，CD34，CD99，CD117，PR，HMB45)对1例乳腺上皮样型管周间质肉瘤与5例叶状肿瘤(良性、交界性各1例，恶性3例)做比较性观察。结果 乳腺管周间质肉瘤(上皮样型)有独特的镜下图像：①显著的多角形(上皮样)细胞绕导管或小管的上皮肌上皮层呈间质性增生，无叶状结构；②组织学模式有袖套状、花冠状、菊形团状、结节状、融合结节状和片状浸润；③瘤细胞形态有：多角形(大、小)、柱状和梭形。多角形细胞呈上皮样形态，异型明显，核分裂象多见(10～30个／10HPF，个别区域达6个／1HPF)，病理性核分裂象易见，在浸润灶内见肿瘤性坏死；④瘤细胞Vim弥漫阳性、EMA灶性阳性、CD99和CD117灶性弱阳性、CD34少数阳性，CK、SMA、S-100蛋白、Des、MG、PR、HMB45均阴性。5例叶状肿瘤均具备叶状结构、间质过度增生、细胞密集(异质性分布)、核分裂象2～10个／10HPF等诊断要素。在3例恶性叶状肿瘤中，2例有极少的上皮样袖套状病灶，2例有梭形细胞袖套状病灶。结论 乳腺上皮样型管周间质肉瘤是一种极罕见的恶性纤维上皮肿瘤亚型，它可能是恶性叶状肿瘤的最早期病变，也可能是一种独特的类型。     

Low-grade pulmonary myxoid sarcoma of uncertain histogenesis

The present report describes an unusual case of low-grade pulmonary sarcoma with extensive myxoid change in a 60-year-old man. During 30 months, the tumor enlarged gradually and thereafter rapidly to 9 cm. Preoperative biopsy and cytology gave negative results. The resected mass, located at the periphery of the right upper lobe, was well circumscribed and showed gelatinous without necrosis or hemorrhage. Histologically, the tumor was composed of slightly atypical, spindle-shaped or stellate cells, which were loosely distributed within a prominent myxoid stroma. Epithelial differentiation was not seen. Lacunar structures were occasionally evident, but no cartilaginous matrix was seen. Mitotic figures were infrequent. Immunohistochemistry failed to clarify the nature of the neoplastic cells except vimentin positivity. Histochemically, the myxoid ground substance was composed of hyaluronic acid and acid mucopolysaccharide. Electron microscopy revealed no specific differentiation other than aggregates of filaments, which were seen in a number of neoplastic cells. Flow cytometric analysis of the neoplastic cells revealed a diploid pattern. These findings indicated that the neoplasm was a low-grade myxoid sarcoma; however, a definite diagnosis could not be made. The tumor might have been a variant of extraskeletal myxoid chondrosarcoma, especially considering the histochemical results.     

Myxoid endometrial stromal sarcoma of the uterus

A rare case of a myxoid type of endometrial stromal sarcoma of the uterus in a 41-year-old woman is reported. A tumor was found in the myometrium and was well circumscribed, measuring 9 x 7 x 7 cm in size. The tumor was mainly composed of a hypocellular area with tumor cells separated by prominent myxoid stroma. The tumor cells were spindle-shaped and resembled endometrial stromal cells. Numerous small thin-walled vessels were seen throughout the tumor. Immunohistochemically, the tumor cells were diffusely stained for estrogen and progesterone receptors and CD10, and focally and weakly for HHF35, alpha-smooth muscle actin and desmin, but not stained for h-caldesmon. These results indicated that the tumor originated from endometrial stromal cells. The tumor had an increased mitotic activity (MIB-1 labeling index: 1-10%), and focally showed nuclear pleomorphism. Thus, this tumor had a malignant potential and was diagnosed as a myxoid type of low-grade endometrial stromal sarcoma. The patient is currently well with no evidence of local recurrence or metastasis 21 months after the operation. This case indicates a wide morphological spectrum of endometrial stromal tumor. A myxoid endometrial stromal sarcoma should be considered in the different diagnosis of the intramural myxoid tumors in the uterus.     

Endometrial stromal sarcoma of the uterus with rhabdoid features

A case of endometrial stromal sarcoma of the uterus with rhabdoid features, occurring in a 57 year old woman is reported. Electron microscopy and immunohistochemistry revealed that the rhabdoid cells contained intermediate filaments which were positive for vimentin, cytokeratin, alpha-smooth muscle actin, and muscle specific actin, but not for myoglobin and desmin. This indicated that the tumor in this case differed somewhat from the three rhabdoid tumors and an endometrial stromal sarcoma with rhabdoid differentiation previously reported and that, therefore, these tumors were heterogeneous.     

低度恶性子宫内膜间质肉瘤临床病理分析

目的 探讨低度恶性子宫内膜问质肉瘤(LESS)的临床病理学特征、诊断和鉴别诊断。方法 分析17例LESS的临床病理特点,通过网织纤维染色、免疫组化染色和电镜观察来研究其病理学特征。结果 LESS临床上主要表现为阴道不规则流血。HE染色见肿瘤组织成巢团样浸润,肿瘤细胞圆形、卵圆形或梭形。肿瘤内有大量的小血管。网织纤维染色见网状纤维丰富,围绕瘤细胞生长。肿瘤细胞14例CD10阳性,12例ER阳性,13例PR阳性,3例actin阳性,C1934、CDll7、Melan—A肿瘤细胞均阴性。电镜观察见肿瘤细胞胞质内的中间丝呈杂乱无序的排列。结论 LESS易误诊,确诊主要依靠组织病理学和免疫组织化学;病理形态上看似良好的低度恶性子宫内膜间质肉瘤,预后不一定好。     

Prostatic stromal sarcoma: case report and review of the literature

We report a case of a 65-year-old man with rare prostatic stromal sarcoma in which suprapubital radical prostatectomy was performed, but neither chemotherapy nor radiation therapy were administered before or after the operation. The well-circumscribed tumor, measuring 5 cm in diameter, showed a homogeneous white grayish cut surface with a hard consistency. Histopathologically, the tumor consisted mainly of medium-sized rounded cells with a sarcomatous and epithelioid appearance intermingled with collagen fiber. Hyalinized foci were also noted in the tumor. Immunohistochemistry revealed that the tumor cells were diffusely positive for vimentin and focally positive for progesterone receptor and CD34, but not for EMA, cytokeratin or estrogen receptor. No recurrence or distant metastasis of the tumor has occurred in 8 years of follow up. The tumor was diagnosed as prostatic stromal sarcoma (PSS) showing epithelioid differentiation and of a progesterone-dependent nature. Possible favorable nature of the PSS might be expected after complete resection.     

Prostatic stromal sarcoma with rhabdoid features

Rhabdoid tumors have been reported in many different anatomic sites as an aggressive tumor and usually present with a rhabdoid tumor component (a composite tumor) rather than a pure rhabdoid tumor. Rhabdoid tumor in the prostate has been described only once in the prostatic region as a possible epithelial origin. Rhabdoid features in prostatic stromal sarcomas (PSSs) have never been described in the literature. Here, we report a case of a PSS with rhabdoid features. A 31-year-old man presented with a 4-month history of voiding difficulty and anal pain. Computed tomography of the abdomen revealed an ovoid mass in the prostate invading rectum and urinary bladder. A needle biopsy was diagnosed as an unclassified spindle cell sarcoma, and 2 cycles of adriamycin-based neoadjuvant chemotherapy were given, followed by radical prostatectomy. The prostatectomy specimen revealed a high-grade sarcoma with fascicles of highly cellular spindle cells and numerous mitoses with hemorrhage and necrosis. In areas, the tumor also contained sheets of loosely cohesive epithelioid cells with rhabdoid tumor component. Both spindle and rhabdoid tumor cells were positive for vimentin, CD34, and progesterone receptor and negative for desmin and cytokeratin immunostainings. The rhabdoid tumor cells retained INI1 expression. The tumor recurred in the bladder, and the patient died of sepsis. To the best of our knowledge, this is the first case of PSS with rhabdoid features. The tumor showed an aggressive clinical behavior with a short-term survival (7 months after diagnosis).     

Endometrial stromal sarcoma arising in vaginaEndometrial stromal sarcoma arising in vagina

Endometrial stromal sarcoma (ESS) arising in the vagina is an extremely rare extrauterine endometrial stroma sarcoma, with only 4 cases reported in the literature up to date. Here we report a case of neoplasm originating from vagina. A 32-year-old woman complained of intermittent vaginal bleeding especially after intercourse. A mass with a diameter of 1.0 cm was found in the middle and upper segments of the right posterior vaginal wall. Biopsy showed ESS. Total abdominal hysterectomy, unilateral salpingo-oophorectomy (right) and partial vaginectomy were performed. No ESS lesion was found in endometrium. The patient received six courses of platinum-containing combination chemotherapy after surgery and was free of tumor 18 months after the diagnosis of ESS. The diagnosis of ESS relies on pathologic examination. CD10 is the most useful immunohistochemical marker for the diagnosis of this tumor. The mainstay treatment of ESS is surgery. Local excision and ovarian retaining may be considered in premenopausal women.     

Demonstration of myxoid change in fine-needle aspiration of synovial sarcoma: A case report

We report a case of synovial sarcoma with extensive myxoid change diagnosed by fine-needle aspiration. The patient is a 46-year-old woman who presented with a right paratibial mass. Aspiration cytology demonstrated a spindle cell neoplasm consistent with a synovial sarcoma but containing a prominent myxoid matrix. The clinical suspicion and cytologic diagnosis of a synovial sarcoma was confirmed by histologic and immunohistochemical findings. The cytologic differential diagnosis of spindle cell neoplasms with extensive myxoid change should be broadened to include synovial sarcoma. Diagn. Cytopathol. 1998;18:188–191. © 1998 Wiley-Liss, Inc.     

Low-grade endometrial stromal sarcoma with an extensive epithelial-like element

A case of low-grade endometrial stromal sarcoma with extensive epithelial-like element (ELE) is reported. This tumor was composed of classical endometrial stromal sarcoma (CESS) showing diffuse proliferation, and ELE occupying approximately 72% of the tumor mass. On immunohistochemistry, ELE was negative for sex-cord differentiation markers, and was positive for myogenic markers used in our investigation, and had a particularly prominent positivity for alpha-smooth muscle actin within the ELE. Therefore, it was considered that ELE showed no true sex cord feature, but smooth muscle differentiation. Moreover, ELE was also positive for CD10, suggesting that it was derived from CESS. It has been reported that there is a distinct clinical behavior between endometrial stromal tumors with abundant ELE and those with limited ELE. In the present case, the Ki-67 labeling index was markedly higher in CESS than in ELE. Therefore, a difference in cell proliferative activity between ELE and CESS might underlie a different clinical prognosis.     

Low-grade fibromyxoid sarcoma versus low-grade myxofibrosarcoma in the extremities and trunk. A comparison of clinicopathological and immunohistochemical features

AIMS: Low-grade fibromyxoid sarcoma (LGFMS) is a distinctive variant of fibrosarcoma and has been reported to have metastatic potential despite its low-grade histological findings. Low-grade myxofibrosarcoma (MFS) is an important differential diagnosis of LGMFS, because it shows different biological behaviour. Of 75 MFSs in the extremities and trunk, we defined 22 grade 1 tumours as low-grade MFS according to the French Federation of Cancer Centres grading system and compared the clinicopathological factors and immunohistochemical expression of cell cycle regulators with those of 11 LGFMSs. METHODS AND RESULTS: The two entities could be distinguished on histological grounds. Low-grade MFS was characterized by the presence of prominent elongated, curvilinear capillaries and pseudolipoblasts, accompanied by an abundant myxoid matrix. It had no extensive solid areas. LGFMS was composed of bland spindle cells arranged in a whorled pattern with alternating myxoid and fibrous stroma. Curvilinear capillaries were not prominent and cytological atypia was absent. No tumour necrosis was observed in any of the 11 LGFMSs, whereas only one case showed tumour necrosis in less than 50% of the tumour in 22 low-grade MFSs. The patients with low-grade MFS were significantly older than those with LGFMS (low-grade MFS average, 60.1 years; LGFMS average, 31.5 years; P < 0.0001) and low-grade MFS occurred more frequently in a superficial location (low-grade MFS 14/20; LGFMS 2/11; P = 0.0077). As for cell cycle regulator expression, the MIB-1 labelling index (LI) (14.76 on average) and cyclin E LI (11.55 on average) in low-grade MFS were significantly higher than those (MIB-1 LI, 4.68 on average; cyclin E LI, 3.38 on average) of LGFMS, while p21 LI (25.53 on average) and p27 LI (42.68 on average) in low-grade MFS were significantly lower than those (p21 LI, 42.74 on average; p27 LI, 57.28 on average) of LGFMS. CONCLUSIONS: We conclude that low-grade MFS and LGFMS are distinctly different clinicopathological entities and the assessment of the immunohistochemical expression of MIB-1, cyclin E, p21 and p27 as well as conventional clinicopathological features may be helpful to distinguish low-grade MFS from LGFMS.     

低度恶性肌纤维母细胞性肉瘤

目的 探讨低度恶性肌纤维母细胞性肉瘤的临床病理学特征、免疫学表型和超做结构特点。方法 对1例发生于左上颌窦的低度恶性肌纤维母细胞性肉瘤进行临床资料复习、光镜观察、免疫组化标记和电镜检测。结果 患者因“左上颌窦囊肿”2次行切除活检，病理诊断分别为“纤维组织增生”和“鳞状细胞癌Ⅰ级”。4个月后因左上颌骨隐痛行左上颌骨部分切除术，术后病理检查未发现肿瘤组织。5个月后左上后磨牙区肿块复发，再行左上颌、颊、颈联合根治术，诊断为“侵袭性纤维瘤病”。9个月后左侧颧部出现包块，术后病理为“左上颌纤维肉瘤，部分伴平滑肌分化”复片显示，第1次活检标本中的“纤维组织增生”实际上是梭形细胞肿瘤组织，而第2次活检中的“鳞状细胞癌1级”实为鳞状上皮假上皮瘤样增生。第3次术后标本中肿瘤组织不明显，而第4次术后标本则由成束的梭形细胞组成，弥漫浸润至邻近的软组织内，类似侵袭性纤维瘤病，但部分区域内可见鱼骨样排列结构，类似低度恶性纤维肉瘤。第5次术后标本中瘤细胞显示轻～中度的异型性，可见核分裂象(2个／10HPF)，并弥漫浸润横纹肌组织，在部分区域内，瘤细胞穿插在肌束之间形成类似增生性肌炎中的棋盘样结构，另一些区域则在形态上类似经典的纤维肉瘤。免疫表型：瘤细胞表达Vim、α—SMA和Ki—67，不表达MSA、Des、h—caldesmon和S—100蛋白。电镜下瘤细胞胞质内可见到平行肌丝。结论 低度恶性肌纤维母细胞性肉瘤是一种少见的软组织肉瘤，组织学形态、免疫表型及超微结构均显示瘤细胞具肌纤维母细胞性分化。     

Low-grade endometrial stromal sarcoma with sex cord-like differentiation metastatic to the thoracic spines

We report a case of monostotic low-grade stromal sarcoma (ESS) with sex cord-like elements metastatic to the thoracic spines, which to the best of our knowledge has not previously been documented. A 48-year-old female who had undergone total abdominal hysterectomy for low-grade endometrial stromal sarcoma 7 years previously presented with insidious onset of severe back pain of 2 months' duration. Magnetic resonance image (MRI) showed involvement of the eleventh and twelfth thoracic vertebral bodies. Decompression at the level of T10-12 was performed. Histologically, the laminae of thoracic vertebrae 11 and 12 were replaced by sheets of ovoid cells with plump nuclei intermixed with anastomosing trabeculae, cords and small nests, reminiscent of a sex-cord stromal tumor pattern. The tumor cells showed diffuse nuclear immunostaining for estrogen receptors (ER) and progesterone receptors (PR), as well as membranous immunostaining for CD10. The immunostaining for smooth muscle actin was focal and sparse. These findings confirmed the diagnosis of metastatic low-grade ESS with sex cord-like differentiation. Low-grade ESS with sex cord-like differentiation is an uncommon tumor which rarely metastasizes to the bone, and use of a panel of ER, PR, CD10, actin, cytokeratin and inhibin immunostains is essential to establish the diagnosis.     

子宫内膜间质肉瘤9例临床病理分析

目的 探讨子宫内膜间质肉瘤(endometrial stromal sarcoma,ESS)的临床病理特征、诊断、鉴别诊断及预后.方法 对9例ESS患者进行临床、病理资料分析、免疫组化检测及随访.结果 患者年龄39～64岁,中位46.3岁.临床主要表现为阴道流血及子宫增大/占位.肿瘤直径2.3～11 cm,平均4.6 cm.光镜下8例呈低度恶性子宫内膜间质肉瘤(low grade endometrial stromal sarcoma,LGESS),均由类似增殖期子宫内膜间质肿瘤细胞构成,细胞密集,异型性不明显,呈不规则舌状或岛状浸润肌层,并伴较多薄壁螺旋小血管;1例为高度恶性子宫内膜间质肉瘤/未分化子宫内膜肉瘤(high grade endometrial stromal sarcoma/undifferentiated endometrial sarcoma,HGESS/UES),肿瘤细胞直接替代子宫肌层,具有明显的细胞异型性,无LGESS常见的螺旋小血管.免疫组化检测显示肿瘤细胞CD10、vimentin均阳性,PR、ER大部分阳性,SMA和desmin及h-Caldesmon为极少数局灶阳性,S-100、CD34均阴性.术后随访7例(平均53个月),只有1例HGESS/UES死亡.结论 ESS是女性生殖道很少见的一种恶性肿瘤,恶性度相差很大.确诊主要依靠其临床病理特点,并辅以免疫组化标记综合分析.诊断时要与子宫内膜间质结节、平滑肌肿瘤、低分化癌等鉴别.     

Stromal sarcoma of the breast with leiomyosarcomatous pattern

We report an unusual case of stromal sarcoma of the breast with leiomyosarcomatous pattern, which recurred locally and was finally treated by radical mastectomy. The tumor was composed of pleomorphic and hyperchromatic spindle-shaped cells arranged in an interdigitating fascicle. The nuclei were of moderate to severe atypia. An average of 10 mitoses per 10 high-power fields was seen. Immunohistochemically, the stromal cells were positive for vimentin and alpha-smooth muscle actin, but negative for S-100 protein, cytokeratin and desmin. The average Ki-67 (MIB1) labeling index in the stromal cells was 34%. Electron microscopic evaluation revealed further evidence of smooth muscle differentiation; stromal cells had frequently indented nuclei, well-developed rough endoplasmic reticulum, thin basal lamina and dense patch-like structures within the cytoplasm. Analysis of previous literature on 17 cases reveals mitotic activity of the tumor seemingly of little prognostic value. This case indicated difficulty in diagnosing leiomyosarcoma. The risk of local recurrence remains even if the surgical margin is free of tumor cells.     

Diagnostic pitfalls associated with fine-needle aspiration biopsy in a patient with the myxoid variant of monophasic fibrous synovial sarcoma

Synovial sarcoma (SS) is one of the most common soft tissue tumors that typically presents in the extremities of young adults, but may occur at any site and affect children during the first decade. Herein we discuss a 12-yr-old male who complained of left foot pain and plantar mass. A fine-needle aspiration biopsy of an 8 cm subcutaneous mass was performed revealing a myxoid spindle cell neoplasm. The cytologic differential diagnosis included a myxoid neurofibroma, neurothekeoma, and a myxoid sarcoma. Subsequent excision of the mass revealed a monophasic fibrous SS with myxoid features. Examination of the tissue by fluorescence in situ hybridization confirmed the presence of characteristic SS SYT gene rearrangement at chromosome 18q11.2. This case underscores that the cytologic distinction of mxyoid spindle cell tumors may be challenging. We report the cytologic features of a myxoid monophasic fibrous SS, and discuss its distinction from other benign and malignant myxoid soft tissue neoplasms.     

原发性子宫外子宫内膜间质肉瘤5例临床病理分析

目的 探讨原发性子宫外子宫内膜间质肉瘤(extrauterine endometrial stromal sarcoma,EESS)的临床病理特征、免疫表型、生物学行为及诊疗进展.方法 收集5例EESS的临床病理资料,并进行免疫组化检测及预后随访.结果 5例患者病理诊断均为低级别EESS,其中1例发生于腹腔、网膜及肠系...     

Endometrial stromal sarcoma: clinicopathological and immunophenotype study of 18 cases

Malignant tumors of the uterine corpus are uncommon. They originate from the endometrial stroma, smooth muscle, blood vessels, or from a mixture of them. The objective of this article was to know the frequency and the clinical, morphologic, and immunophenotype characteristics of the endometrial stromal sarcoma (ESS). We reviewed the cases of ESS observed from 2002 to 2008 at the Pathology Unit of the General Hospital of Mexico. The following data were analyzed: age, clinical stage, degree of differentiation, and immunophenotype. We found 18 cases, and the average age of patients was 48.6 years; 66% were in clinical stages 1 and 2. Fifteen cases (83.3%) were classified as low-grade sarcomas and 3 (16.6%) as high-grade or undifferentiated sarcomas. We determined immunohistochemical markers in 17 cases; receptors to estrogens were positive in 5 (29.4%) and to progesterone in 9 (52.9%). CD10 was expressed in 10 (58.8%) and p53 in 11 cases (64.7%). Two cases were associated to primary tumors of the ovary (papillary cystadenocarcinoma). In conclusion, ESS was present at 0.6% in our institution; and most were low grade. Expression of markers, such as p53, CD10, and hormonal receptors, was positive.     

New developments in endometrial stromal sarcoma

Endometrial stromal tumours have been recently reclassified in the WHO 2014 Classification due to the discovery of new genetic fusions. This has enabled the subdivision of previously described undifferentiated endometrial sarcomas into the molecularly-defined high grade endometrial stromal sarcoma (HG ESS) and undifferentiated uterine sarcoma (UUS). In this review, we discuss the discoveries behind the 2014 Classification and its rationale, and give practical tips for diagnosis of these neoplasms, as well as discussing the differential diagnoses that one may consider.