颅内及椎管内卵黄囊瘤1例

正卵黄囊瘤是一种少见的起源于生殖细胞的生殖细胞瘤,常见于儿童及青少年,多发于卵巢、睾丸及骶尾部,主要沿身体中线分布,发生于其他部位少见,颅内及椎管内者极为罕见。我们收治1例颅内及椎管内髓外卵黄囊瘤患者,报道如下。患者女性,28岁。因"腰腿痛1月余,加重1周"于2016年11月28日收入我院。患者既往体健,曾于1个月前因腰痛伴右下肢疼痛,来我院就诊,初步检查后考虑"肌筋膜炎",给予对症治疗,效果较差,伴随双下肢进行性肌     

Intracranial and intraspinal dissemination from a growth hormone-secreting pituitary tumor. Case report

A 48-year-old woman developed multiple intracranial and intraspinal metastases from an invasive growth hormone-secreting pituitary adenoma after surgery and radiation therapy. This is the first reported case to show that the cells in the metastatic tumors and in the cerebrospinal fluid contained growth hormone.     

Surgical treatment of endolymphatic sac tumor with adjunctive stereotactic radiation therapy--case report

A 44-year-old man presented with an endolymphatic sac tumor (ELST) associated with von Hippel-Lindau disease, which required four surgical procedures within 10 years. The earlier two surgeries resulted in only partial removal of the tumor because of vigorous intraoperative bleeding. Stereotactic radiation therapy was performed twice. The intraoperative bleeding was easy to control in the third operation, and ultimately the tumor was totally extirpated in the fourth operation. Histological examination of the tumor specimen harvested in the final surgery showed that the tumor cells had clearly decreased in number, and the interstitial tissue had become fibrous with organization of the tumor vessels, compared with the tumor specimen from the first surgery. Preoperative radiotherapy may be effective to reduce the devastating intraoperative bleeding of ELST.     

An unusual successfully treated case of pulmonary yolk sac tumor

Extragonadal germ cell tumors are relatively rare, particularly malignant yolk sac tumors arising in the lung parenchyma. We report a case of a huge malignant yolk sac tumor in the right middle lobe. The patient was successfully treated with neoadjuvant chemotherapy followed by complete resection of the tumor.     

Primary yolk sac tumor of the cerebellar vermis: case report.

A rare case of yolk sac tumor in the cerebellar vermis is reported. A 2-year-old boy developed headaches, vomiting, and an unsteady gait. Later a tumor was demonstrated in the medial part of the cerebellum by gadolinium-enhanced magnetic resonance imaging (MRI). The tumor was totally removed, and the surgery was followed by chemotherapy. Soon after surgery the elevated alphafetoprotein (AFP) levels in the serum and cerebrospinal fluid were observed to decrease to normal levels. Three months later enhanced MRI showed a lesion in the vermis without any elevation of AFP, and the lesion turned out to be a granuloma. Six months after the second surgery a tumor recurred that could not be totally removed. Cranial radiotherapy was given together with chemotherapy, which resulted in a decrease of AFP to the normal range. The patient is doing well without any elevation in AFP at 1 year 6 months after onset. Related problems in the diagnosis and treatment of yolk sac tumors are discussed.     

Adult testicular pure yolk sac tumor.

Here we describe the clinical, ultrasonographic and histological features of a rare pure adult yolk sac tumor detected in the right testis of a 44-year-old male. Due to the rarity of this neoplasm (less than 10 cases have been reported), there is no unanimous consensus for therapy following inguinal orchiectomy. We believe that nerve-sparing retroperitoneal lymph node dissection could be potentially curative and useful for future interpretations of this tumor's potential evolution.     

A mediastinal germ cell tumor of yolk sac type--case report

OBJECTIVE: We report an extremely rare case of germ-cell tumor localized at the level of the anterior mediastinum. Clinical presentation: A 36-year-old man who presented with left subclavial vein thrombosis was admitted to our hospital for specific cure. Computed tomographic scan of the chest showed a large anterior mediastinal mass. Surgical intervention revealed an infiltrative mediastinal tumor involving the left subclavial vein, which was biopsied for morphological examination. Histologically, the tumoral mass proved to be a carcinoma, with papillary and tubular growth patterns. Immunohistochemical stains for alpha-fetoprotein were positive in the tumor cells while stains for carcinoembryonic antigen and placental like alkaline phosphatase were negative. The serum level of alpha-fetoprotein of this patient was elevated, as well. This supported the diagnosis of Yolk sac tumor, a rare primary tumor within the mediastinum. Postsurgery, the patient received a combination chemotherapy consisting of cisplatin, vepesid and bleomycin every 3 weeks for a total of 4 cycles. During the treatment, the alpha-fetoprotein level, was decreasing. Conclusion: Primary mediastinal Yolk sac neoplasm is a rare tumor. The diagnosis should be made not only by morphological studies but also the patient's age and the elevation of serum alpha-fetoprotein. In spite of modern chemotherapy, the prognosis of mediastinal yolk sac tumor remains poor. The single most important prognostic indicator is whether the tumor mass can be completely excised before or after chemotherapy.     

Gonadal and extragonadal yolk sac tumor

Yolk-sac tumours are germ-cell tumours with gonadal and extragonadal location. Histologically they consist of primitive cellular forms configurated as a loose network. These tumours are of extreme malignancy and should be treated as radical as possible. Surgery should be combined with anticancer chemotherapy. However, with adequate treatment only children suffering from testicular tumours and not older than 2 years of life have a fair chance to survive. 3 cases are dealt with in detail.     

A case of mediastinal yolk sac tumor successfully treated with chemotherapy and surgery

A 28-year-old man was admitted due to increasing respiratory symptoms. X-ray examination of the chest showed a tumor mass in the anterior mediastinum with possible invasion into the chest wall and upper lobe of right lung. No tumor was found in the testis. Serum alpha-fetoprotein (AFP) concentration was 6400 ng/ml. Serum levels of CEA and HCG were within normal limits. Percutaneous biopsy of the tumor strongly suggested yolk sac tumor with an evidence of AFP by an immunoenzyme labelling technique. The serum AFP rapidly decreased after two courses of combination chemotherapy. En bloc resection of the tumor was successfully performed and third chemotherapy was added. Mediastinal yolk sac tumors should be treated with combination chemotherapy and surgical resection.     

Intracranial tumor developing from the vitelline sac. Apropos of a case

The authors report a case of a primary intracranial yolk sac tumor which is a quite rare eventuality. The patient, an 18 months old girl was referred for severe intracranial hypertension. Neurological examination revealed a right hemiparesis, a left abducens nerve palsy and a bilateral papilledema. The serum and C.S.F. levels of alpha fetoprotein were at 2100 Ugr/l and 2500 Ugr/l, respectively. The computerized tomography showed a mass with a low density area in the left temporoparietal lobe and the carotid angiogram a highly hyper-vascular tumor. The child underwent a left temporo-parietal craniotomy and the friable vascular tumor was totally removed. A radiotherapy was associated to the surgical treatment. Histologically, glomerular like structures (Shiller Duval body), intra and extra cellular hyaline globules PAS positive were frequently seen. The tumor marker was demonstrated immunohistochemically.     

Microcystic Leydig cell tumors mimicking yolk sac tumor: a report of four cases.

We report four cases of Leydig cell tumor of the testis with a microcystic pattern that mimicked yolk sac tumor. The patients ranged in age from 27 to 35 years and, except for one tumor that was discovered incidentally, presented with testicular masses. All tumors were intratesticular, and three were well circumscribed by a rim of fibrous tissue, whereas one showed minor, focal extension into the adjacent testis. The tumors typically had a vaguely lobular architecture subdivided by fibrous bands. Three of the cases had a complex microcystic appearance caused by individually vacuolated cells and coalescent cystic spaces; this pattern accounted for the majority of two tumors. Another case had focal collections of Leydig cells with prominent cytoplasmic vacuoles but lacked the coalescent spaces. The microcyst contents ranged from optically clear to eosinophilic or lightly basophilic, with the latter having the staining qualities of acid mucopolysaccharide. Three tumors had uniform, bland nuclei and low mitotic rates (<1 mitotic figure per 10 high power fields), but one had marked, random nuclear pleomorphism and an average mitotic rate of five mitotic figures per 10 high power fields. By immunohistochemistry, all were diffusely positive for vimentin; two of three were positive for inhibin, and one showed focal positivity for cytokeratin (CAM 5.2). All were negative for alpha-fetoprotein and placentalike alkaline phosphatase and, apart from having microcystic and solid areas, lacked other features typical of yolk sac tumor. Clinical follow-up ranged from 2 months to 2 years with no patient having recurrence or metastasis. The distinction of Leydig cell tumor from yolk sac tumor has important clinical implications because patients with the former usually receive only clinical follow-up, but the latter often requires chemotherapy.     

Extragonadal yolk sac tumor in a superior scrotal mass.

We report a case of an extragonadal yolk sac tumor in a superior scrotal mass. Testicular ultrasonography was normal preoperatively and after chemotherapy. Serum alpha-fetoprotein was initially increased to 344.55 ng./ml. (normal 0 to 15) but it returned to normal after surgery and chemotherapy.     

Malignant transformation of intracranial mature teratoma to yolk sac tumor after late relapse. Case report

The recurrence of intracranial mature teratomas as germ cell tumors of different histological types is rarely reported. The authors describe the first case of the malignant transformation of an intracranial mature teratoma into a yolk sac tumor in a 16-year-old boy who presented with a 1-month history of anorexia and somnolence. Seven years prior to this presentation, the boy had undergone surgery for extirpation of a mature pineal teratoma. Computed tomography images obtained at his second presentation revealed a homogeneously enhanced mass within the third ventricle. The tumor was resected and the results of a histological examination were consistent with a yolk sac tumor. After resection, the patient underwent radiation therapy followed by chemotherapy with cisplatin and etoposide but died of tumor progression 15 months after his second hospitalization.     

Cerebral composite atypical teratoid/rhabdoid tumor and yolk sac tumor in the frontal lobe of an infant. Case report

A 1-year-old male infant presented with a rare cerebral composite tumor consisting of atypical teratoid/rhabdoid tumor (AT/RT) with epithelial and mesenchymal components and yolk sac tumor (YST) with Schiller-Duval bodies. He was admitted to our medical center with a 2-month history of right hemiparesis. Computed tomography and magnetic resonance imaging revealed a large, intra-axial solid tumor with a cyst in the left frontal lobe. Total resection of the tumor was performed. Histological examination showed two different main growth patterns: solid sheets of undifferentiated polygonal cells and a few rhabdoid cells with rosette structures and rhabdomyoblastic cells; and reticular or papillary structures with occasional Schiller-Duval bodies in a myxoid matrix. The immunohistochemical and electron microscopy findings indicated composite AT/RT and YST. Initial total resection of the tumor was subsequently followed by local recurrence, hydrocephalus, and spinal metastasis. Despite adjuvant chemotherapy, the patient died 9 months after admission. AT/RT is a recently established entity of the central nervous system. The present case of composite AT/RT and YST in the frontal lobe indicates the poor prognosis of such tumors.     

Primary pineal melanocytic tumor. Case report

A primary melanocytic lesion arising from the pineal gland is very rare. The authors report a case of primary pineal melanocytic tumor with dissemination to the right hippocampus in a 50-year-old woman who presented with memory disturbance. Magnetic resonance (MR) imaging revealed a mass that was hyperintense on T1-weighted and hypointense on T2-weighted MR images. The pineal tumor was removed subtotally via the occipital transtentorial approach, and the patient underwent whole-brain irradiation. Results of histological examination revealed that the tumor predominantly consisted of atypical cells with scanty melanin pigment and some necrotic foci. The strongly pigmented areas of the tumor contained well-differentiated cells similar to those of melanocytoma. An ultrastructural study demonstrated evidence of a mature type of melanosome. The patient died 11 months after surgery and radiotherapy (1.7 years after the onset of symptoms). The autopsy findings demonstrated tumor invasion into the parenchyma through the leptomeningeal space and the ventricular wall. The tumor was diagnosed as being malignant, and it was finally concluded that the atypical cells in the tumor were probably responsible. This pineal melanocytic tumor exhibited a wide spectrum of differentiation, ranging from highly malignant melanoma to well-differentiated melanocytoma, which may have contributed to the patient's relatively long survival period. The biological behavior and morphological characteristics of this tumor appear to be similar to those of other pineal parenchymal lesions.     

Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage. Case report

Endolymphatic sac tumors (ELSTs) are locally invasive neoplasms that arise in the posterior petrous bone and are associated with von Hippel-Lindau (VHL) disease. These tumors cause symptoms even when microscopic in size (below the threshold for detectability on imaging studies) and can lead to symptoms such as hearing loss, tinnitus, vertigo, and facial nerve dysfunction. While the mechanisms of audiovestibular dysfunction in patients harboring ELSTs are incompletely understood, they have critical implications for management. The authors present the case of a 33-year-old man with VHL disease and a 10-year history of progressive tinnitus, vertigo, and left-sided hearing loss. Serial T1-weighted magnetic resonance (MR) imaging and computed tomography scans revealed no evidence of tumor, but fluid attenuated inversion recovery (FLAIR) MR imaging sequences obtained after hearing loss demonstrated evidence of left intralabyrinthine hemorrhage. On the basis of progressive disabling audiovestibular dysfunction (tinnitus and vertigo), FLAIR imaging findings, and VHL disease status, the patient underwent surgical exploration of the posterior petrous region, and a small (2-mm) ELST was identified and completely resected. Postoperatively, the patient had improvement of the tinnitus and vertigo. Intralabyrinthine hemorrhage may be an early and the only neuroimaging sign of an ELST in patients with VHL disease and audiovestibular dysfunction. These findings support tumor-associated hemorrhage as a mechanism underlying the audiovestibular dysfunction associated with ELSTs.     

Testicular yolk sac tumor in children. Current view on diagnosis and treatment

5 children with yolk-sac tumor of the testis (= infantile embryonal carcinoma) were admitted to our hospital between 1973 and 1981. Evaluation of serum alpha-fetoprotein concentrations and ultrasonography of the retroperitoneal area proved to be extremely valuable to predict the tumor stage in these children. Therapy consisted of high inguinal orchiectomy, retroperitoneal lymphadenectomy and cytostatic chemotherapy in 3 children using the T2-protocol. All children are now between 1 1/2 and 8 years free of recurrence and metastases. Therapy of this tumor ought to be differentiated according to the individual tumor stage. Most patients with stage-I tumor require only radical orchiectomy for cure.     

Testicular seminoma after the complete remission of extragonadal yolk sac tumor : a case report

Background

Between 2% and 5% of malignant germ-cell tumors in men arise at extragonadal sites. Of extragonadal germ cell tumors, testicular carcinoma in situ (CIS) are present in 31–42% of cases, and CIS are reported to have low sensitivity to chemotherapy in spite of the various morphology and to have a high likelihood of developing into testicular tumors. A testicular biopsy may thus be highly advisable when evaluating an extragonadal germ cell tumor.

Case presentation

A 36-year-old man was diagnosed as having an extragonadal non-seminomatous germ cell tumor, that was treated by cisplatin-based chemotherapy, leading to a complete remission. In the meantime, testicular tumors were not detected by means of ultrasonography. About 4 years later, a right testicular tumor was found, and orchiectomy was carried out. Microscopically, the tumor was composed of seminoma.

Conclusions

We herein report a case of metachronous occurrence of an extragonadal and gonadal germ cell tumor. In the evaluation of an extragonadal germ cell tumor, a histological examination should be included since ultrasonography is not sufficient to detect CIS or minute lesions of the testis.