Malignant hemangiopericytoma of the liver: Report of a case

A 30 year old female was admitted with right upper abdominal pain and fever. Ultrasonography and computed tomography revealed a large cystic mass in the right lobe of the liver, and aspiration bacteriology was negative. A right hepatic lobectomy was performed for a suspected cystadenocarcinoma, however, the tumor was histologically diagnosed as a hemangiopericytoma with prominent venous invasion. The patient died within a short time of multiple pulmonary metastases. Primary hepatic hemangiopericytoma is extremely rare, and according to our research, this is only the 4th case reported in the literature.     

Papillary glioneuronal tumor: case report and review of literature

Papillary glioneuronal tumor (PGNT) is a recently described central nervous system neoplasm that mostly occurs in the supratentorial system, adjacent to the lateral ventricles.In 2007, WHO classified PGNT as grade I neuronal-glial tumor because of the characteristic papillary architecture and bipartite (astrocytic and neuronal/neurocytic) cell population. As a newly established entity of mixed glioneuronal tumor family, PGNT attracted extensive attention recently.In our report we discuss the clinical, neuroradiological and surgical features. The final result is compared with literature data.     

Dumbbell neurogenic tumor of the mediastinum: A report of three cases undergoing single-staged complete romoval without thoracotomy

Of a total thirteen patients who underwent surgery for a neurogenic tumor in the posterior mediastinum 4 (30.8 per cent) presented with dumbbell type development of the tumor. Along with a comparatively greater incidence in the number of cases of dumbbell neurogenic tumors in the posterior mediastinum, resection has also recently become more popular, necessitating the establishment of a standard operative approach for this type of tumor. We successfully removed dumbbell neurogenic tumor from the posterior mediastinum in our 3 most recent casesvia a dorsal approach by virtue of a laminectomy and resection of a small portion of the neighbouring rib root without opening the parietal pleura at all. These three aptients were a 14 year old female, a 54 year old male and a 68 year old female, respectively, and the largest diameter in cm and level of origin of the tumors were 5.5 at Th 1 in case 1, 3.0 at Th 2 in case 2 and 3.7 at Th 11 in case 3. The operative approach described herein was easy to perform, felt secure and was less invasive and better tolerated by the patients than the thoractomy approach. Avoiding a thoractomy in such cases has many advantages to enumerate, but does not seem to have been clearly aimed at by others to date. We therefore propose our technique as a standard approach for dumbbell neurogenic tumors in the posterior mediastinum.     

Myofibroblastic tumor causing severe neonatal distress. Successful surgical resection after embolization

This report describes a case of a term male 3.1 kg, normal delivery, 38 weeks of gestation with a record of hydramnios by prenatal sonography. He had fetal acute suffering and respiratory distress. The first radiographic study showed a mass filling the whole left thorax cage causing erosion of the inferior edge of the third rib. The mediastinum was displaced to the right. Computed tomography scan confirmed a homogeneous tumor that filled the left thorax and displaced the mediastinum to the right without invasion. Surgical biopsy informed of a highly vascularized mesenchymal tumor. The tumor was embolized with Ivalon microparticles obtaining a nearly avascular mass. Complete surgical excision was made, including the whole mass and costal segments. Microscopically, it was an inflammatory myofibroblastic tumor. It was composed mainly of spindle-shaped cells without malignant features. On immunohistochemistry, the tumor showed positive staining for vimentin, whereas antidesmin antibodies and S-100 protein were negative. The aim of this article is to present an extremely uncommon case of neonatal distress caused by an intrathoracic, extrapulmonary myofibroblastic tumor. Complete surgical resection was possible after embolization.     

Gastrointestinal stromal tumor of the lesser omentum: report of a case

We describe herein an extremely unusual case of a gastrointestinal stromal tumor (GIST) of the lesser omentum. A 45-year-old man was admitted to our hospital with an intra-abdominal mass that was subsequently misdiagnosed as a submucosal tumor of the stomach. The tumor arose from the lesser omentum and was removed without difficulty. Histologically, the tumor was composed of spindle–shaped cells with an interlacing bundle pattern, and immunohistochemical examination showed that it was positive for myeloid stem cell antigen (CD34), but negative for HHF35 and S-100 protein. These findings were consistent with a GIST lacking myogenic features and neural attributes. The patient had an uneventful postoperative course, and was free of recurrence when last seen 11 months after his operation. Received: April 3, 2000 / Accepted: March 6, 2001     

Metachronous testicular tumor developing eight years after retroperitoneal extragonadal germ cell tumor

Abstract:   A 30 year-old man was admitted to our hospital with a chief complaint of left flank pain. Computed tomography revealed a retroperitoneal tumor. Levels of lactic dehydrogenase, human β-chorionic gonadotropin, α-fetoprotein, DUPAN-2 and carbohydrate antigen 19–9 were elevated. No abnormal findings were present according to palpation and ultrasonography of the testes. The patient was diagnosed as having a retroperitoneal extragonadal germ cell tumor (EGCT) considering the elevated markers. Resection of the tumor, two cycles of neoadjuvant and one cycle of adjuvant chemotherapy (cisplatin and etoposide) were performed. The surgical specimen showed total necrotic tissue. Eight years later, the patient noticed an enlargement of his left testicle. The tumor felt elastic and firm on his left testis. Neither distant metastasis nor lymph node metastasis was present according to computed tomography. Left high orchiectomy was performed and histology revealed seminoma. Twenty-three cases have been reported previously about metachronous testicular tumor developing after retroperitoneal EGCT. We report the 24th case.     

Primitive neuroectodermal tumor of the adrenal gland

We report a rare case of primitive neuroectodermal tumor arising from adrenal gland in adulthood, diagnosed preoperatively as having non-functional adrenocortical adenoma. Laparoscopic adrenalectomy was performed. Immunohistological examination revealed the definite diagnosis as primitive neuroectodermal tumor of the adrenal gland. Although primitive neuroectodermal tumor is a highly malignant neoplasm, there is no evidence of local recurrence and distant metastasis 16 months after surgery.     

Pancreatic neuroendocrine tumor with splenic vein tumor thrombus: A case report

INTRODUCTION

Pancreatic neuroendocrine tumors (PNET) are rare, often indolent malignancies. PNET are classified as functional or nonfunctional based on the secretion of hormones without a negative feedback loop; the latter account for up to 60% of PNET. Although PNET are associated with a better prognosis compared to pancreatic adenocarcinomas, they are often diagnosed in advanced stages, making them a significant source of morbidity for patients. Here we present a rare case of venous tumor thrombus arising from a nonfunctional PNET.

PRESENTATION OF CASE

A 44-year-old woman was referred for evaluation and treatment of a possible tail of pancreas PNET discovered during work-up for a 9 year history of intermittent subcostal pain. Previous endoscopic ultrasound with fine needle aspiration revealed a 3.5 cm × 3 cm mass, with cytological diagnosis of neuroendocrine tumor. Patient was scheduled for laparoscopic distal pancreatectomy. During surgery the mass was found to encase the splenic vein leading the surgeon to perform an en bloc distal pancreatectomy and splenectomy. Pathologic analysis revealed a 1.8 cm × 5 cm tumor thrombus lodged in the splenic vein.

DISCUSSION

Nonfunctional PNET usually present in advanced stages and can be associated with venous tumor thrombi. Preoperative imaging may not accurately predict the presence of venous tumor thrombi.

CONCLUSION

En bloc resection of primary tumor, involved organs and thrombus is the recommended treatment option and often results in long term survival. New multi-modality strategies are needed for detection of venous involvement in nonfunctional PNET to better assist with preoperative planning and counseling.     

阴茎原始神经外胚叶瘤1例并文献复习

目的:探讨原发于泌尿生殖系统的原始神经外胚叶瘤的临床表现、病理特点、治疗方法及预后。方法:分析本院2007年3月收治的1例原发于阴茎的原始神经外胚叶瘤患者的病例资料并复习相关文献。结果:该患者经过病理确诊为阴茎原始神经外胚叶瘤,已采取手术根治,拟进一步化疗。结论:病理及免疫组化有助于对原始神经外胚叶瘤的确诊。对于早期患者手术是首选的治疗方法,晚期患者可以辅助性地行联合化疗,目前原发于阴茎的原始神经外胚叶瘤非常罕见,对预后尚没有评估依据。     

Fetal and neonatal rhabdoid tumor

Purpose

Few studies have focused on the behavior of rhabdoid tumor (RT) in the fetus and neonate. The purpose of this review is to show that perinatal RTs are associated with unusual findings and a poor prognosis.

Methods

The author conducted a 40-year systematic review of the literature. Clinical presentation, pathology, management, and outcome of 72 fetuses and neonates with RTs are discussed.

Results

Seventy-two fetuses and neonates presented with RTs detected prenatally (n = 12) and during the neonatal period (n = 60). The review consisted of 3 main groups: extrarenal noncentral nervous system (CNS) RT, renal RT, and CNS RT. There were some group differences in survival: extrarenal non-CNS RT (3/33 or 9.1%), renal RT (2/27 or 7.4%), and CNS RT (2/12 or 16.7%). Metastatic RT was present at diagnosis in more than half the patients (41/72 or 57%) who had a survival of 2.3%. The overall survival was 9.7%. For statistical results, there was no significant difference in survival among the 3 groups by type of tumor (P = .692). χ² analysis for survival with and without metastases was not valid due to small sample size.

Conclusions

The review shows that extrarenal RT was more common than either renal RT or CNS RT groups that is different than that observed in older individuals. Concomitant brain tumors were found in almost a third of fetuses and neonates. The CNS involvement occurred more often in patients with renal RT than in those with extrarenal RT. Metastatic disease at diagnosis was noted in more than half of the patients. Higher stage and presence of a CNS tumor were significant determinants in survival.     

Plexiform fibrohistiocytic tumor of the hand: a case with late recurrence

A case of a rare and recently described neoplasm of the superficial soft tissue that occurs mainly in children and young adults is described. The lesion demonstrates morphological features reminiscent of both fibrous histiocytoma and fibromatosis. Microscopically, this tumor is characterized as a multinodular or plexiform proliferation of histiocyte- and fibroblast-like cells associated with multinuclear giant cells. Because of its unpredictable biological behavior, high recurrence rate, and potential for regional lymphatic metastasis, the diagnosis of plexiform fibrohistiocytic tumor raises important treatment considerations.     

Malignant germ cell tumor of the mediastinum: A multimodality therapeutic approach

Twenty-five patients with a malignant germ cell tumor of the mediastinum were treated at the National Cancer Center Hospital, Tokyo. Three patients had pure seminomas while 22 had a nonseminomatous histology. The treatment modalities consisted of surgery alone in 7 patients, surgery with chemotherapy and/or radiation in 14, and chemotherapy and/or radiation without surgery in 4. Cisplatin-based chemotherapy was administered in nine patients, one of whom successfully underwent high-dose chemotherapy with autologous bone marrow transplantation after resection of the tumor. A complete response was achieved in only five patients. The overall survival rate at 5 years was 33.5% and the median survival time was 51 weeks. Eight patients (32%), all of whom underwent surgery either with or without chemotherapy and/or radiation, were free of disease with a mean follow-up period of 439 weeks (72–1,120 weeks). The median survival times for 14 patients undergoing combined modality treatment, 7 patients undergoing surgery alone, and 4 patients undergoing chemotherapy and/or radiation were 83, 16, and 18 weeks, respectively.     

Sertoli cell tumor (gonadal stromal tumor) in an infant.

An 8-mo-old boy had an orchiectomy for a Sertoli cell tumor. Malignancy was suspected histologically. He underwent a retroperitoneal lymphadenectomy and no nodes contained metastases. The histologic criteria for malignancy in Sertoli cell tumors are controversial. Individual case reports of patients with these tumors are encouraged to help establish their natural history and optimum management.     

膀胱孤立性纤维瘤1例报告并文献复习

目的:探讨膀胱孤立性纤维瘤(SFT)的临床病理特征、诊断及治疗方法。方法:回顾性分析1例膀胱SFT患者的的临床资料,临床及影像学诊断为膀胱肿瘤,在腰麻下行经尿道膀胱肿瘤电切术,术后出院。1个月后再次来我院复查,在全麻下行经腹腹腔镜下膀胱肿瘤部分切除术。结果:病理检查提示为梭形细胞肿瘤,免疫组织化学检查倾向于SFT。患者腹腔镜术后痊愈出院。结论:膀胱SFT是一种罕见肿瘤,其诊断主要依靠病理学及免疫组织化学检查,手术是其主要治疗方法。     

ԭ���Ը�Ĥ���������������

目的探讨原发性腹膜后内胚窦瘤的治疗和预后方法回顾性分析1991～2002年收治的11例碍发性腹膜后内胚窦瘤．结果该组病例占同期收治恶性原发性腹膜后肿瘤的8.8％(1l／125)和内胚窦瘤的20.3％(1l／54)。术前B超、CT或MR1检查发现腹膜后肿瘤。术前血清甲胎蛋白升高5例全组手术切除全部或大部分肿瘤。术后用PVH(顺铂、长春新碱、博菜霉素)或BEP方案(博菜霉素、足叶乙甙、顺铂)化疗随访8个月至8年，生存10例，死亡1例。结论以顺铂为基础的联合化疗改善了内胚窦瘤的疗效，测定血清AFP确助内胚窦瘤的诊治，应选择恰当的手术方式并辅以联合化疗来救治病人     

肝脏炎性肌纤维母细胞瘤

目的探讨肝脏炎性肌纤维母细胞瘤的临床病理特征、诊治和预后。方法总结了一例肝脏炎性肌纤维母细胞瘤病人的临床资料,并复习了国内外的文献,分析和归纳其临床表现、诊断和治疗。结果肝脏炎性肌纤维母细胞瘤的临床病理特征和预后如下:①常见于儿童和青少年;②临床症状常表现为贫血、发热、上腹部隐胀痛、体重下降等;③物理检查可见肝脏包块;④病理特征:该瘤由分化的肌纤维母细胞性梭形细胞组成,常伴大量浆细胞和(或)淋巴细胞浸润;⑤外科切除或肝移植后,预后佳,无复发。结论肝脏炎性肌纤维母细胞瘤有其特征性的临床表现,经手术切除后可治愈。     

肝脏炎性肌纤维母细胞瘤

目的 探讨肝脏炎性肌纤维母细胞瘤的临床病理特征、诊治和预后。方法总结了一例肝脏炎性肌纤维母细胞瘤病人的临床资料，并复习了国内外的文献，分析和归纳其临床表现、诊断和治疗。结果肝脏炎性肌纤维母细胞瘤的临床病理特征和预后如下：①常见于儿童和青少年；②临床症状常表现为贫血、发热、上腹部隐胀痛、体重下降等；③物理检查可见肝脏包块；①病理特征：该瘤由分化的肌纤维母细胞性俊形细胞组成．常伴大量浆细胞和（或）淋巴细胞浸润；⑤外科切除或肝移植后，预后佳，尤复发。结论肝脏炎性肌纤维母细胞瘤有其特征性的临床表现，经手术切除后可治愈。     

Lymphangioma of the kidney

Lymphangiomas are rare benign tumors that are congenital malformations of the lymphatic system. Most cases present in children as a soft, cystic mass in the neck and the axilla. Primary renal lymphangioma is exceedingly rare, with only 35 cases reported so far. We report a case of primary lymphangioma arising from the kidney. A 59-year-old man was referred for evaluation of a right renal mass found in an abdominal ultrasonography during a health checkup. Abdominal ultrasonography and computed tomography (CT) revealed a 3.2 x 2.9 cm multiloculated cystic mass in the upper pole of the right kidney. We could not deny malignant disease such as cystic renal cell carcinoma with any diagnostic modalities. The patient was brought to surgery. During the surgical procedure, the tumor was suspected to be lymphangioma of the kidney as a result of a frozen- section histopathological evaluation. Therefore enucleation of the tumor was performed. Pathological evaluation of the specimen revealed lymphangioma arising from the kidney. The patient is free of disease after a 3-month follow-up period.     

Prenatal diagnosis of a large, cervical, intraspinal, neurenteric cyst and postnatal outcome

Neurenteric cysts are rare congenital anomalies derived from the notochord and located in front of the spinal column, mostly at the cervical level. They consist of an intraspinal cystic component that is connected to a mediastinal or thoracic cyst. A case of a cervical, intraspinal, neurenteric cyst, diagnosed at 28 weeks of gestation and operated on at 3 and half months after birth, is reported. Herein, we discuss important features of this case including its rare occurrence, intrauterine diagnosis, and early surgical intervention. In conclusion, the nature of prenatal intradural cysts should be fully evaluated and differentiated between neurenteric cyst and other types of cysts with fetal ultrasonography. Therefore, it is essential to be aware of this potential cyst in fetal ultrasonography in utero. Neurenteric cyst should be considered in the differential diagnosis of cervical intradural cystic lesions. In infants, successful surgery provides a cure in patients with cervical neurenteric cysts.     

The solid-pseudopapillary tumor of pancreas: the clinical characteristics and surgical treatment

BACKGROUND AND OBJECTIVE: Since being named and reclassified by WHO in 1996, solid-pseudopapillary tumor (SPT) of pancreas has been recognized as a special entitative disease that is different from pancreatic cancer and should be recognized and treated more accurately in the surgical process. The clinic characteristics and surgical strategy on 25 cases of SPT of pancreas from the authors' center are discussed. METHODS: The clinical pathology and the surgical methods of 25 SPTs were retrospectively studied. The analyses were performed by the statistical software package SAS6.12. RESULTS: No tumor recurrences were found in all patients. There was significant difference between operative types in radical resection and the tumor position of the pancreas (P = 0.0011). The judgment on the tumor's boundary could directly affect the adoptable operative types (P = 0.0099). CONCLUSIONS: As a uniquely entitative disease, SPT is a kind of uncommon neoplasm with low-grade malignancy with a strong rate of occurrence in women. Surgical resection is most favorable in the treatment of SPT, which has excellent prognosis. The course of SPT, the possible malignant cells by the frozen section biopsy, and the tumor's boundary are important for operators to decide an operative scheme. SPT that has infiltrated contiguous vessels, organs, even with local liver metastasis should not be regarded as operative contraindication. The choice of the local tumor resection, the part of pancreas resection or radical resection depends on the judgment of the tumor's boundary, whereas operative types in radical resection depend on the tumor position of the pancreas.