肝脏原发粒层细胞瘤伴海绵状血管瘤1例报道并文献复习

目的探讨肝脏原发粒层细胞瘤(granulosa cell tumor)伴海绵状血管瘤的临床病理学特点。方法对1例肝脏原发粒层细胞瘤伴海绵状血管瘤进行组织学观察和免疫组化染色,观察其超微结构及临床影像学特点,并复习相关文献。结果患者女性,CT及MRI提示肝脏右叶可见一巨大实性肿物,低密度。肉眼见肿物突向肝脏下方,切面呈实性;另外,在肝脏膈面被膜下见一灰红色出血区,呈疏松海绵状。镜下巨大肿物中肿瘤细胞椭圆形或梭形,呈微滤泡状、小梁状排列,部分区域呈实性弥漫状分布,类似于卵巢粒层细胞;部分区域呈束状排列,间质中可见较多胶原纤维瘢痕形成,似孤立性纤维性肿瘤。肿瘤细胞核内可见小核仁,并可见纵形核沟。免疫组化结果示肿瘤细胞CD99、α-inhibin、CK8、CK18、SMA和BCL-2均(+),Ki-67增殖指数<1%。超微结构显示肿瘤细胞可见各种形式的核皱叠形成,部分细胞呈Call-Exner小体样结构排列,偶见桥粒结构,提示该肿瘤与卵巢粒层细胞瘤极其相似。结论粒层细胞瘤原发于肝脏且合并海绵状血管瘤,非常罕见,目前其临床生物学行为难以确定,需对患者进行长期随访。     

Asymptomatic right atrial cavernous hemangioma: a case report and review of the literature

A case of asymptomatic right atrial cavernous hemangioma in a 50-year-old man is reported. With right atriotomy under total cardiopulmonary bypass, the right atrial mass was identified as arising from the noncoronary Sinus of Valsalva. The mass was successfully resected with the Sinus of Valsalva closed primarily. The patient is alive and well 9 months following the operation. The incidental discovery of the mass, clinical evaluation, operative procedure, and pathologic findings are reported. We also briefly discuss the epidemiology, natural history, and the diagnostic and therapeutic approaches to cardiac tumors.     

十二指肠生长抑素瘤伴肝转移1例并文献复习

目的探讨以肝脏转移灶为首发症状的十二指肠生长抑素瘤的临床病理特点及诊断。方法回顾性分析1例十二指肠生长抑素瘤伴肝转移的临床表现、病理组织学、免疫表型特点及误诊的原因,并结合文献进行复习。结果患者行B超检查考虑为“肝囊肿”。2年后经上消化道内镜于十二指肠降段查见一个盘状病变,病理报告为十二指肠黏膜及黏膜下类癌。随后,肝穿证实为十二指肠类癌肝转移。经免疫组化标记,肿瘤细胞表达以somatostatin为主,最后病理诊断：十二指肠生长抑素瘤伴肝转移。结论本例临床表现及病理形态较为特异,需要依据病理组织学及免疫组化检查予以确诊。对于异时肝转移癌仅凭影像学检查难以准确地揭示疾病的内在本质,只具有参考价值。     

Testicular juvenile granulosa cell tumor in a newborn: case report and review of the literature

Juvenile granulosa cell tumor of the testis of neonates and infants is an uncommon lesion frequently associated with abnormal sex chromosome and ambiguous genitalia. This report describes a juvenile granulosa cell tumor arising in the testis of a neonate. Chromosome analysis of the tumor showed a normal male karyotype 46 XY. Histopathology and immunohistochemical studies revealed the occurrence of 2 well-differentiated epithelial-like and smooth muscle-like components in the neoplasm. The morphologic clues leading to the correct diagnosis of juvenile granulosa cell tumor and the possible histogenesis are briefly discussed.     

Primary glomus tumor of the liver: a rare case report and review of the literature

Glomus tumor is a rare neoplasm of the distal extremities. It occurs very rarely in the deep visceral organs such as stomach, lung, pancreas, ovary, and liver. Herein, we report a very rare case of glomus tumor of the liver in a 50-year-old woman presenting with abdominal mass which was diagnosed after surgery. The tumor was large and cystic; however, the morphology was similar to the ordinary soft-tissue glomus tumor. Tumor cells were reactive with CD34 and SMA. The patient's follow up failed to show any evidence of malignant behavior or tumor recurrence. This report is the third primary glomus tumor of the liver in the English literature.     

Unicystic granulosa cell tumor: a case report

Granulosa Cell Tumors (GCT) constitutes 1.5% to 3.5% of all primary ovarian neoplasms. They may be solid, cystic or both. Unilocular cystic GCT are very rare. We report here a case of 32 years female with unicystic GCT in which the distinction from follicular cyst was difficult and was made by carefully examining the lining of the cyst,which showed occasional multiple layers of granulosa cells with a few Call-Exner bodies.     

Malignant advanced granulosa cell tumor of the adult testis: case report and review of the literature

Testicular granulosa cell tumors (GCTs) are very rare neoplasms. Although adult GCTs are thought to have a relatively indolent course, several reports have demonstrated the malignant potential of these lesions. In case of distant metastases, the overall survival is very short. To date, there is no well-established treatment for these tumors owing to poor results and very rapid progression. A 55-year-old male patient was diagnosed with a testicular GCT with distant lung metastases. He underwent surgical treatment with orchiectomy and adjuvant polychemotherapy (cisplantine, etoposide, and bleomycine) as well as metastasectomy of the right lung. We report the first case of a successfully treated testicular GCT with bipulmonary metastases at initial diagnosis. Thirty-nine months after treatment, the patient is alive with no evidence of disease. We subsequently reviewed all reported cases of an adult GCT in the published literature (25 published cases). This review will summarize all reported cases and discuss treatment options. The current case suggests that a combination of varying treatment modalities could be a promising and reasonable way to manage malignant advanced GCT of the adult testis.     

Primary liposarcoma of the liver: a case report and review of literature

Liposarcoma is a rare mesenchymal malignant tumor, which usually originates in the retroperitoneum and the extremities. Seven cases of primary liposarcoma of the liver have been previously reported. We present the eighth case, which occurred in an adult female patient. Primary liposarcoma of the liver, although extremely rare, must be considered in the differential diagnosis of a hepatic mass that develops in a noncirrhotic liver, especially in patients who are potential candidates for orthotopic liver transplantation. Liposarcoma is an absolute contraindication for liver transplantation.     

Primary glomangioma of the liver: a case report and review of the literature

Glomangiomas are a subset of glomus tumors that have a rich vascular network. Although a majority of the glomus tumors occur in the skin of the hand, they have also been reported in the deep soft tissue, bone, lungs, and gastrointestinal tract, especially the stomach. To our knowledge, only one such case has previously been reported primarily occurring in the liver. We report a case of a glomangioma primarily arising in the liver of a 57-year-old man who presented with right flank pain of several months' duration. A 3.0-cm hepatic mass was excised and consisted of numerous, small-to-medium branched vessels with the stroma containing small, round, regular cells with sharply outlined round-to-oval nuclei. Immunostains showed the tumor cells to be diffusely positive for vimentin and smooth muscle actin and to be focally positive for calponin. Collagen IV stained the pericellular matrix. The immunostain for CD34 highlighted the vascular network as well as outlined the tumor cells in many areas. Coexpression of actin and CD34 in glomus tumors, although unusual, has recently been reported in the literature. Despite its bland histology, the large tumor size and deep visceral location were suggestive of aggressive behavior; thus, a close clinical follow-up was recommended. The patient had an unremarkable postoperative course and has no evidence of metastatic disease 12 months after the procedure. An accurate diagnosis and an understanding the biology of this rare disease, especially in an unusual location, are crucial to its management.     

Spontaneous rupture of hepatic hemangioma: a case report and literature review

Hepatic hemangioma, a common benign tumor of the liver, has caused great threats to the public health. Most patients have an excellent prognosis because of the benign nature of hemangioma. On some occasions, spontaneous rupture of hepatic hemangioma is rarely observed in patients. Therefore, a majority of scholars propose that surgery should be restricted to specific situations. In this case, we presented a patient with spontaneous rupture of hepatic hemangioma in our hospital. After a literature review, we summarized the management and outcome of the patient, which revealed the survival rates of the patients are satisfactory.     

Peripheral dentinogenic ghost cell tumor: report of a case and literature review

Peripheral dentinogenic ghost cell tumor is a rare tumor with only 24 cases previously described in the English literature. The majority of cases have been reported to occur in the anterior part of the jaws. A case occurring in posterior (molar region) of the mandible in a 75-year-old edentulous woman is reported. The patient presented with a nodular swelling in the left mandible that showed erosion (saucerization) of the underlying bone radiographically. On microscopy, the tumor showed mainly solid epithelial islands resembling ameloblastoma in fibrous connective tissue. The islands were associated with ghost cells and dysplastic dentin. This report includes the clinical, radiographic, and microscopic features of the patient, in addition to the review of the English literature on the tumor.     

Primary giant cell tumor of soft tissues similar to bone giant cell tumor: A case report and literature review

In this report we describe a primary giant cell tumor (GCT) of soft tissues located in the left dorsal wrist of a 52-year-old man. Plain radiographs did not reveal any lesion in his carpal or hand bones. Although the tumor was clinically considered a ganglion initially, the microscopic features were identical to those found in classic GCT of bone. Light microscopy showed a lesion composed of a homogeneously mixed proliferation of spindle and polygonal mononucleated stromal cells and evenly distributed multinucleated, osteoclast-like giant cells. Whereas most bone tumors have an extraosseous counterpart, only 13 cases of GCT in soft tissues had been published until 1998. Moreover, 64 new cases have been reported in three series. Nevertheless, most major reviews and textbooks do not consider this tumor as a specific entity and regard it as a low grade variant of malignant GCT of soft tissue. We describe the clinical, histologic, and immunohistochemical features of this rare benign neoplasm emphasizing the differential diagnosis with its malignant soft tissue counterpart, an ominous tumor.     

Intraosseous spindle cell hemangioma of the calcaneus: a case report and review of the literature

Spindle cell hemangioma, a rare benign tumor characterized by cavernous blood vessels and spindled areas, typically arises in the subcutis of the distal extremities, particularly the hand. The case of intraosseous spindle cell hemangioma is extremely rare, and only 1 case arises in the frontal bone has been reported previously. We describe herein a case of intraosseous spindle cell hemangioma occurring in the left calcaneus in a 65-year-old woman. The patient was successfully treated by the operation. The present case is instructive especially in the differential diagnosis of primary bone tumor structured by spindle cells, for which the possibility of spindle cell hemangioma should be considered.     

Sertoli-Leydig cell tumor with heterologous element: a case report and a review of the literature

The patient was a 19-year-old female who presented with a chief complaint of progressive pelvic pain. Preoperative ultrasound of the right ovary revealed an ovarian torsion as the cause of the patient’s progressive pain. Laparoscopy confirmed the torsion and revealed a right ovary measuring 10 cm in greatest diameter. Intraoperative incision into the ovary revealed a simple ovarian cystic mass measuring 3.0 x 1.5 x 0.8 cm. A solid component within the cyst was identified. Histological sections of the cystic mass demonstrated mononuclear and hyperchromatic Sertoli cells with a trabecular growth pattern. Clusters of medium-sized epithelioid cells with abundant eosinophilic cytoplasm consistent with Leydig cells were also identified between the trabeculae of Sertoli cells. In addition, focal areas of intestinal type mucinous epithelium were identified embedded within the trabeculae of Sertoli cells. Immunohistochemical studies revealed that the Sertoli cells were positive for calretinin (bright) while the Leydig cells were positive for calretinin (dim), inhibin, CAM5.2 and AE1&3. CEA showed positivity mainly of the intraluminal contents of the mucinous type intestinal epithelium. The patient had an uneventful post-operative course and was disease-free for 3 years.     

Malignant rhabdoid tumor of the liver: case report and literature review

A case of malignant rhabdoid tumor (MRT) occurring as a primary hepatic neoplasm in a 12-month-old Japanese female infant is presented. The patient had a slight fever for 2 weeks and presented with a palpable mass in her left hypochondrial region. After admission, the hepatic artery was embolized due to intra-abdominal hemorrhage arising from the tumor. The patient received chemotherapy with cisplatin, cyclophosphamide and adriacin. Despite treatment, the patient developed dyspnea, pancytopenia and disseminated intravascular coagulation. Rupture of the tumor resulted in death within 3 weeks. A limited abdominal autopsy revealed that the liver weighed 1240 g and was occupied by multiple hemorrhagic and/or necrotic tumor nodules. Histologically, neoplastic cells had an abundant eosinophilic cytoplasm containing paranuclear inclusions, and vesicular nuclei with a centrally located prominent nucleolus. Ultrastructurally, the cytoplasmic inclusions were composed of whorled filaments measuring 10 nm. Immunohistochemically, almost all of the neoplastic cells were positive for vimentin and cytokeratins (CK) 8 and 18, some were positive for CK 7 and 19, while none were positive for CK 1, 10, 13-17 and 20. The tumor cells did not express desmin, myoglobin, and alpha-fetoprotein. We found 18 cases of MRT of the liver published in English language literature and then, adding the present case, we summarized the 19 cases. Hepatic MRT is an uncommon neoplasm. However, it should be considered in the differential diagnosis of an aggressive liver neoplasm in childhood.     

Primary adenomatoid tumor of the testis: report of a case and review of literature

Adenomatoid tumor (AT) is an extremely rare benign tumor in the testis of infants. A case of 14-month-old boy with testicular adenomatoid tumor was reported in this study. On physical examination, a smooth solid nodule sized 8 mm could be palpated with little tenderness on the head of the right testis. It could be clearly revealed by B ultrasonic scanning and computerized tomography. The patient underwent right radical orchiectomy. In postoperative histopathological study, the tumor was characterized by diffuse sheets of epithelioid cell and desmo-stroma structures. There was positive immunohistochemical staining of mesothelioma-associated antigens. The tumor should be differentiated from the tumor of the male genital tract including benign and malignant tumors of both epithelial and stromal origin. And we followed the case and no nodule was found in his scrotum by physical examination and scrotal ultrasonography after 3, 6, 12, 18, 24, 30, 36, 42, 48, 54, 60 months. These findings have important implications that the histogenesis of adenomatoid tumor of the testis is unclear yet. The diagnosis depends on pathologic studies, and should be differentiated from paratesticular malignant mesothelioma and sclerosed lipogranuloma. Radical surgery is the common choice, and as a result of getting a good prognosis.     

Late recurrence of the granulosa cell tumor: a case report

Granulosa cell tumor of the ovary is an uncommon neoplasm characterized by a long natural history, late recurrence capacity and good prognosis. We report the case of a woman with adult-type granulosa cell tumor of the ovary, which developed late recurrence with contralateral ovarian disease, intra-abdominal spread and liver metastases.     

微静脉型血管瘤1例临床病理观察并文献复习

目的：探讨微静脉型血管国形态学特征和免疫组化表型,侧重于病理诊断和鉴别诊断。方法：以１例微静脉血管瘤进行临床病理学分析和免疫组织化学研究。结果：肿瘤出不规则分枝状的增生性薄壁血管组成,增生性的血管在真皮的全层内呈浸润性生长。部分内皮细胞略肿胀,但细胞无异型性,也无分型象。多数血管内皮细胞的外围可见梭形的血管周细胞。血管之间的基质伴有不同程度的胶原样变性。免疫组化标记显示内皮细胞强阳笥表达ＣＤ３１、     

原发性子宫内膜鳞癌1例报道及文献复习

目的探讨子宫内膜鳞癌的临床病理特点。方法对1例原发性子宫内膜鳞癌进行免疫组化观察并结合文献复习其临床病理特点。结果原发性子宫内膜鳞癌镜下为伴角化珠及间桥的高分化鳞癌，浸润子宫壁肌层内2／3，淋巴管见多发性瘤栓。子宫颈和颈管正常。免疫表型：高分子CK( )、低分子CK( )、ER( )、PR( )、HPV胞质阳性、p53( )及P-gp( )。结论原发性子宫内膜鳞癌非常罕见，恶性度高。腺上皮鳞化癌变，可能是其病变过程；HPV的作用还不清楚。该肿瘤术前诊断困难，确诊主要靠术后病理检查。