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Successful treatment of recurrent malignant germ cell tumors: report of two cases.

We report two patients with recurrent malignant germ cell tumors who were successfully treated with radiation therapy and cisplatin combination chemotherapy. Patient 1, who had a suprasellar mixed yolk sac tumor, had been free of tumor for 3.5 years after cisplatin/vinblastine/bleomycin combination chemotherapy alone but showed a recurrent tumor in the pineal region. In Patient 2, who had a pineal alpha-fetoprotein-producing tumor, recurrent tumors appeared in the lateral ventricle 3 years after the tumor was controlled by radiation and cisplatin/vinblastine/bleomycin therapy. They were treated successfully again by radiation therapy, followed by cisplatin and etoposide combination therapy, and they have been free of tumor for 5.5 and 7 years after recurrence, respectively. The total dose of cisplatin in both patients exceeded 1,000 mg/m2, which would have contributed to the control of the recurrent tumors. Anterior pituitary dysfunction observed in Patient 1 did not improve, even after tumor disappearance by chemotherapy, suggesting severe damage to the neurohypophysis by direct invasion of the tumor.

Adolescent↗

Testicular yolk sac tumors in children.

BACKGROUND: Testicular tumors in children are uncommon, comprising about 1% of pediatric malignancies. Yolk sac tumor is the most common malignant testicular tumor in children. Because yolk sac tumor in children is rarely seen, its treatment has been controversial. We reviewed the records of 15 children with testicular yolk sac tumor treated at our hospital in order to evaluate optimal management and treatment outcome. METHODS: From February, 1981, to August, 1996, 15 children with testicular yolk sac tumor were treated. Mean patient age at diagnosis was 15.8 months (range, 7-22 months). Fourteen patients presented with stage I disease and one presented with stage III disease. Mean follow-up was 88 months (range, 2-156 months). RESULTS: All 15 patients received radical inguinal orchiectomy as initial treatment. Serum alpha-fetoprotein (AFP) concentrations were measured in 14 stage I patients preoperatively and were elevated in all of them. During follow-up, the one stage III patient died of the disease. Of the remaining 14 patients, two (14.3%) had recurrence with elevated AFP at three months and 10 months postorchiectomy, respectively. These patients were managed with cisplatin-based combination chemotherapy. To date, they are both alive with no further recurrence, and AFP concentrations returned to normal after chemotherapy. Overall, of the 15 patients with testicular yolk sac tumor, 14 (93.3%) survived without disease. CONCLUSIONS: Our results suggest that testicular yolk sac tumor in children is a tumor with a favorable prognosis. Serum AFP concentration is extremely useful in diagnosis and monitoring of treatment response. Radical inguinal orchiectomy alone seems adequate for patients with stage I disease if serum AFP concentrations return to normal postoperatively. Cisplatin-based combination chemotherapy should be administered in patients with tumor recurrence or metastasis.

Endodermal Sinus Tumor↗

Fine needle aspiration cytology of primary mediastinal germ cell tumors.

Fine needle aspiration cytology was performed on six patients with malignant mediastinal germ cell tumor: 1 pure seminoma, 1 pure embryonal carcinoma, 1 pure yolk sac tumor and 3 mixed germ cell tumors containing teratoma. Their cytologic features were compared with each other and with the cytologic features of thymoma, which arises commonly in the anterior mediastinum. A definitive cytologic diagnosis could be made only in the cases of seminoma because of its characteristic cytologic features. Cytochemical staining for germ cell alkaline phosphatase was helpful in diagnosing seminoma in the cytologic examination, while the presence of hyaline globule or alpha-fetoprotein immunostaining as the cytologic diagnostic feature of yolk sac tumor were not necessarily found in fine needle aspiration cytology. All epithelial, lymphocytic and mixed type thymomas were easily differentiated from the four types of germ cell tumor examined.

Adolescent↗

[Problems in diagnosis and therapy planning in a patient with retroperitoneal yolk sac tumor. Case report and review of the literature].

The case of a 26 years old patient with a primary paraaortic extragonadal yolk sac tumor is presented. After operative reduction of the tumor polychemotherapy with PEB was performed. Following chemotherapy testicular biopsy did not show histological changes typical for testicular intraepithelial neoplasia (TIN). Three years after the initial manifestation of the yolk sac tumor, a malignant tumor of the testis with components of undifferentiated teratoma and yolk sac tumor was observed. In this paper the problems of development of testicular malignancy after polychemotherapy and the validity of a small exploratory excision of testicular tissue in retroperitoneal germ cell tumors and following chemotherapy are discussed.

Adult↗

Combined yolk sac tumor and adenocarcinoma in a gastric stump: molecular evidence of clonality.

BACKGROUND: Extragonadal yolk sac tumors of the gastrointestinal tract are extremely rare neoplasms. Their greater rarity compared with other extragonadal yolk sac tumors suggests that different pathogenetic mechanisms could be involved according to the site of origin. This report describes a case of a combined yolk sac tumor and adenocarcinoma that arose in a gastric stump in a man age 61 years 43 years after he underwent distal gastric resection and gastrojejunostomy (Billroth II operation) for a benign duodenal ulcer. The coexistence of an adenocarcinomatous component with the yolk sac component suggests that the two histologic patterns may represent distinct phenotypes arising from a common mucosal epithelial cell. METHODS: Immunohistochemical and molecular techniques were used to define the mutation pattern of p53 in both components of the tumor. RESULTS: Single-strand conformation polymorphism and sequencing analyses demonstrated the same pattern of p53 mutation in the adenocarcinomatous and yolk sac tumor components. CONCLUSIONS: This finding suggests that the two tumors could have been derived from the same cellular clone and supports the hypothesis that the two components represented a heterogeneous differentiation of the same tumor.

Adenocarcinoma↗

Cardiac tamponade caused by intrapericardial yolk sac tumor in a boy.

Yolk sac tumor rarely involves the pericardium. A 17-year-old boy had a mediastinal mass leading to total obstruction of the superior vena cava with cardiac tamponade and pleural effusion. Further imaging studies and biopsy showed that the origin was a yolk sac tumor. After surgery and adjuvant chemotherapy, no recurrence was noted during a follow-up of 20 months. This report describes the magnetic resonance imaging characteristics of pericardial yolk sac tumor.

Adolescent↗

Testicular neoplasm presenting as a major pulmonary embolism.

OBJECTIVE: To report an unusual and life-threatening presentation of a nonseminomatous germ cell tumor of the testis. METHODS: A 35-year-old male presented with pulmonary embolism secondary to a neoplastic thrombus from a nonseminomatous germ cell tumor. Prompt management involved surgical thrombectomy with cardiopulmonary bypass and deep hypothermic circulatory arrest, followed by orchiectomy and adjuvant chemotherapy. RESULTS: Three years later, the patient remains disease free according to physical examination, radiographic workup and serum markers. CONCLUSION: The excellent long-term outcome of this patient supports an aggressive multidisciplinary management of nonseminomatous germ cell tumors in young adults.

Adult↗

[Video image analysis of AgNORs distribution in the testicular tumor].

For the purpose of exploring the relationship between AgNORs and the biological behaviour of the testicular tumor, the value of enumeration and image analysis of AgNORs has been investigated in 10 normal testicular tissues and 49 cases of testicular tumors. The major findings were: AgNORs value for number, area, mean area, large partical number (LPN) and roundness in normal, benign and malignant tumors had significant difference (P < 0.01). The former four parameters turned larger when the tumor became more malignant, and they were significantly higher in the good prognosis group than in the bad one. LPN was higher in stage III than in stage I and II (P < 0.05), no difference was found between stage I and II. Other parameters had no significant difference in each stage. These results indicate that AgNORs is helpfull in differentiating the benign and malignant testicular tumors, in determining the degree of malignancy and offering an additional prognostic indicator in testicular tumors. Partical numbers, area and LPN were more helpfull. It had no important value in differenciating each stage.

Endodermal Sinus Tumor↗

[Burned-out testicular tumor: a case report].

A 29-year-old man was referred to our hospital with loss of appetite and a left lower abdominal mass. A small nodule was palpable in his left testis and ultrasonographic examination demonstrated that the nodule was low echoic. Computed tomography showed a large mass in his left retroperitoneal space. We thought the mass was a metastatic lesion from a testicular tumor. Left orchiectomy was done and microscopic examination revealed no viable tumor cells. Only fibrous tissue, small calcified areas, and hyaline bodies were found. As tumor markers were normalized after 3 courses of chemotherapy with bleomycin, etoposide, and cisplatine, the retroperitoneal mass was removed with the left kidney. It consisted of embryonal carcinoma, mature teratoma, and yolk sac tumor. One course of adjuvant chemotherapy was done and the patient has since been free from recurrence. We suppose that the tumor was a so-called 'burned-out' testicular tumor.

Adult↗

Cytologic diagnosis of yolk sac tumor. A report of seven cases.

BACKGROUND: Yolk sac tumor (YST) is a rare neoplasm that affects children and adolescents. CASES: Fine needle aspiration biopsies from seven patients with YSTs were reviewed. Eosinophilic hyaline cytoplasmic bodies and irregular deposits of basement membrane-like material were easily recognizable in aspiration smears. Some of the tumor cells contained cytoplasmic vacuoles. These features correlated well with histologic appearances. CONCLUSION: These findings are significant in the fine needle aspiration biopsy diagnosis of YST. A preliminary cytologic diagnosis of this tumor is valuable as a guide in planning further diagnostic studies and therapy.

Biopsy, Needle↗

[Combination chemotherapy with POMB/ACE (cisplatin, vincristine, methotrexate, bleomycin, actinomycin D, cyclophosphamide, etoposide) in advanced non-seminomatous testicular tumor].

Four cases with non-seminomatous testicular tumor in stage III completed chemotherapy with POMB/ACE. Of these 4 cases, metastasis to retroperitoneal lymph nodes was found in all of them. In addition, metastasis to the lung was noted in 3, and to the left supraclavicular lymph nodes in one. After orchiectomy, 5 courses of POMB/ACE therapy were given to each of the 4 cases. Tumor marker returned to normal value in all of the cases after 3-4 courses of treatment, with disappearance of metastasis to the lung and supraclavicular lymph nodes. However, the response rate in metastasis to retroperitoneal lymph nodes was CR in one case, and PR in 3. Therefore, retroperitoneal lymph nodes were excised in all 3 cases. Histologically, 2 of the 3 were found to have necrotic tissues. The remaining one patient had teratoma. An additional 1-3 courses of POMB/ACE therapy were given to these 3 cases. These 4 cases are alive without recurrence 6 years and 4 months, 5 years and 8 months, 4 years and 9 months, and 2 years 9 months, respectively, after orchiectomy. Thus POMB/ACE therapy is considered to be a useful method in the treatment of advanced testicular tumor.

Adult↗

Cytodiagnosis of yolk sac tumor.

To analyze cytomorphologic features of yolk sac tumors of childhood. Four cases of pediatric yolk sac tumor (YST), diagnosed by fine needle aspiration cytology were reviewed (1998-2002). Age of patients ranged from 1(1/2) to 5 years. Three cases presented clinically with an intra-abdominal mass while one case presented with a testicular mass. Fine needle aspirates had been obtained directly as well as under radiologic (USG/CT) guidance. Smears were stained with H & E and Papanicolaou stain. In all cases values of serum alpha-fetoprotein and hCG were available preoperatively. Histopathologic diagnosis was correlated with cytologic findings in all the cases. Cytologic examination showed richly cellular smears with a combination of morphological patterns. Characteristically, tumor cells were arranged in papillary groups, tight cell clusters and formed acinar structures. Cells showed enlarged, moderately pleomorphic, hyperchromatic nuclei and moderate amount of cytoplasm, some of which displayed cytoplasmic vacuolation, displacing the nuclei eccentrically. Preoperatively, serum alpha-fetoprotein level was raised in all cases. Histopathology confirmed the cytologic diagnosis. Yolk sac tumor is common among the germ cell tumors of pediatric age group which presents a spectrum of cytomorphologic features having important differences with other germ cell neoplasm, e.g. embryonal carcinoma. Clinicoradiologic features and tumor markers are additionally helpful for an accurate cytologic diagnosis.

Abdominal Neoplasms↗

Intracranial germ cell tumors: detection of p53 gene mutations by single-strand conformation polymorphism analysis.

Using polymerase chain reaction-single-strand conformation polymorphism (PCR-SSCP) analysis, p53 gene mutation was examined in 12 intracranial germ cell tumors (5 yolk sac carcinomas and 7 germinomas), many of which were derived from young patients in the first to the second decade. A total of 10 mutations were detected in 4 of the 12 cases and, in 3 of them, the mutations were multiple or tandem. Among the 10 mutations, 7 were missense, 1 was splicing and 2 were silent. The 7 missense mutations were located at previously proposed hot spot codons or in their vicinity or, when outside the hot spots, at a codon encoding an amino acid conserved in most vertebrates. These findings suggested that all 7 missense mutations may actually give rise to functional alteration of the p53 protein. The splicing mutation was considered to be a germ-line mutation, though its biological effect was equivocal, since the neoplastic tissue contained an additional mutation. The pattern of the mutations was predominancy of G:C-A:T transition with frequent involvement of the CpG site. These mutations were more frequently detected in yolk sac carcinomas (60%; 3/5 cases) than in germinomas (14%; 1/7 cases), suggesting that the contribution of the p53 mutation to carcinogenesis differed with the histological type of the intracranial germ cell tumor.

Adolescent↗