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Primary tumors of the sacrum are rare. In adults, the most common sacral tumors are metastases. The most common primary sacral tumor is a chordoma. Chordomas along as well as tumors such as chondrosarcomas, osteosarcomas, myxopapillary ependymomas, myelomas, and Ewing sarcomas are considered malignant. In this article the authors focus on benign sacral tumors.
The germinal bilateral tumor represents from 1 to 4% of the testicle tumors, and it usually turns to be one of the most difficult ones when talking about diagnosis and treatment. We are attaching a 30 years old patient case, with a diagnosis consisting in a seminoma with yolk sac tumor area from the left testicle, that was treated with orquiectomy. Eleven months later, the patient presented a second neoplasm in the contralateral testicle, whose histology was a classical seminoma with carcinoma "in situ" peritumoral, being also treated with orquiectomy. Medical literature is revised with emphasis in the involved risk factors and the techniques used to treat these testis tumor groups: carcinoma "in situ" detection appears to be the strongest predicting factor. Testicular contralateral biopsy is proposed in some select cases. Even though radical orquiectomy is still the treatment to choose, local radiotherapy and conservative surgery are also an option in patients with bilateral tumor.
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OBJECTIVE: Our purpose was to evaluate the prognostic factors in yolk sac tumors of the ovary. STUDY DESIGN: We performed a retrospective review of 47 patients with yolk sac tumors of the ovary from 1979 to 1997. RESULTS: Twenty-two patients had pure yolk sac tumors and 25 had germ cell tumors with yolk sac tissue as a component of the disease. The 5-year survival rate in stages I, II, III, and IV was 95%, 75%, 30%, and 25%, respectively. Patients with stage I disease had a more favorable prognosis than those with stage III and IV disease (P <.001). All patients who did not respond to chemotherapy died of this disease within 36 months of the first treatment. Chemotherapy regimens that included cisplatin gave better results than those without cisplatin (P <.05). The difference in prognosis was significant in cases in which the size of residual tumor was <2 cm in diameter (P <.01) and in cases in which ascites was either absent or <100 mL in volume (P <.05). Coexistence of other components of ovarian germ cell tumors in histologic specimens, preoperative serum alpha-fetoprotein level, fertility-sparing surgery, dissection of intrapelvic nodes, and p53 status had no significant correlation with the prognosis in this study. CONCLUSIONS: Staging and tumor-reductive surgery strongly affected the prognosis of this disease. Tumor-reductive surgery is advisable when ascites is minimal. Cisplatin-based chemotherapy after surgery was superior to chemotherapy without cisplatin; however, p53 status seemed to have no impact on chemosensitivity in yolk sac tumors of the ovary.
There is controversy concerning the treatment of stage I yolk sac tumor of the testis, particularly of those with histological factors that indicate a high risk of relapse. Usually orchiectomy alone is sufficient and adjuvant chemotherapy is unnecessary. Retroperitoneal lymphadenectomy is indicated for patients with persistently high alpha-fetoprotein. Once recurred, treatment at that time is thought to be curative. However, postoperative chemotherapy may be necessary for patients with a tumor expressing histological factors that predict possible relapse. In this paper we report on a case of a 2 year old boy whose tumor invaded the testicular veins. The patient suffered from recurrent disease but was successfully treated by chemotherapeutic regimens including cisplatin and retroperitoneal lymphadenectomy. The importance of the histological factors in making a decision on the treatment strategy for stage I testicular yolk sac tumor is discussed.
The carbohydrate structure of glycoproteins is considered to be tissue-specific or cell type-specific, but there have been no reports on the differences of the carbohydrate structure of alpha-fetoproteins (AFPs) produced by histologically identical tumors in different tissues. The lectin affinity electrophoresis of hepatoid adenocarcinomas and yolk sac tumors from different organs suggested that either the tumor heterogeneity or the tissue specificity is possibly involved, the lectin reactivity of the AFP sugar chain structure produced by the tumors in different tissues.
The authors evaluated the effect of adjuvant therapy (preoperative chemotherapy combined with radiotherapy) followed by radical tumor removal in the treatment of children with primary intracranial yolk sac tumor, embryonal carcinoma, or mixed germ cell tumors containing yolk sac tumor components. Between 1988 and 1995, five consecutive patients were treated with adjuvant therapy followed by total tumor removal. The diagnosis was based on markedly elevated concentrations of serum alpha-fetoprotein (AFP) and/or beta-human chorionic gonadotropin (beta-HCG) in four children and the results of biopsy sampling in one child. The chemotherapy regimen consisted of cisplatin (20 mg/m2) and etoposide (60 mg/m2) daily for 5 days (one course) given three times at 4-weeks intervals. Radiotherapy consisted of 30 to 40 Gy to the whole brain or an area including all ventricles and a 15- to 20-Gy boost to the tumor site. Spinal radiation of 25 Gy was added in one patient. In all patients the serum level of AFP and beta-HCG gradually decreased during the adjuvant therapy and disappeared completely on its completion. In two of the five patients the tumor disappeared as well. In the other three patients the tumor size was moderately or markedly reduced and the remaining tumor was totally removed; there were no neurological deficits. Chemotherapy was maintained after the initial treatment and was repeated every 2 to 4 months for less than 2 years. All children are alive and well without recurrence at 33 to 118 months (average 88 months) after the start of adjuvant therapy. Our preliminary results indicate that adjuvant therapy consisting of combination chemotherapy with cisplatin and etoposide and concomitant radiotherapy, followed by removal of the tumor, is highly effective in the treatment of pediatric patients with primary intracranial yolk sac tumor, embryonal carcinoma, or mixed germ cell tumors containing yolk sac tumor components.
Two yolk sac tumors that arose in the mesentery of the jejunum and the mesentery of the transverse colon of two male patients, aged 2 and 17 years, are reported. Both patients had abdominal masses. The tumors measured 9 and 11 cm in maximum dimension. One of them grew into the bowel lumen. Microscopically, both neoplasms exhibited several of the typical patterns of yolk sac tumor and stained immunohistochemically for alpha-fetoprotein. Both patients received chemotherapy postoperatively and are alive, but follow-up is short. The subject of extragonadal yolk sac tumors is reviewed, and histogenetic implications of their occasional origin in the mesentery is discussed.
PURPOSE: We evaluated the clinical behavior of clinical stage I pure yolk sac tumor of the testis in adults to determine whether the behavior of this entity is different than that of clinical stage 1 nonseminoma. MATERIALS AND METHODS: We searched the testis cancer database at our institution for adults with clinical stage I pure yolk sac tumor of the testis who underwent retroperitoneal lymph node dissection. We identified 12 such patients and reviewed the database and hospital charts to determine clinical behavior. RESULTS: Disease was pathological stage I in 8 of the 12 patients (66%), including 1 with recurrence after retroperitoneal lymph node dissection. Disease was pathological stage II in 14 patients (33%), including 1 who remains disease-free after electing adjuvant bleomycin, etoposide and cisplatin. Of the 3 patients who elected observation after retroperitoneal lymph node dissection only 1 has had recurrence, while 2 (66%) were cured by retroperitoneal lymph node dissection only. CONCLUSIONS: Contrary to juvenile yolk sac tumor, which has a strong tendency toward hematogenous metastasis, the behavior of clinical stage I adult pure yolk sac tumor is similar to that of all other stage I nonseminomas in adulthood.
Five cases of yolk sac tumor of the anterior mediastinum associated with prominent thymic cystic changes are presented. The patients were five men between the ages of 17 and 22 years (mean, 19.5 yr). Clinically, four patients presented with symptoms of chest pain and shortness of breath, whereas the fifth was asymptomatic: his tumor was discovered during a routine physical examination. None of the patients had a history of previous tumor elsewhere. Grossly, the tumors were described as cystic lesions measuring between 8 and 12 cm in greatest dimension. Histologically, the tumors showed prominent cystic changes reminiscent of those observed in acquired multilocular thymic cysts, i.e., the presence of cystic cavities lined by squamous epithelium with prominent inflammatory changes, lymphoid hyperplasia, cholesterol cleft granulomas, and remnants of thymic tissue in the walls of the cysts. In addition, there was a neoplastic cellular proliferation composed of small cells with scant cytoplasm, round nuclei, and inconspicuous nucleoli adopting a reticular pattern of growth and, in some areas, embedded in abundant myxoid stroma. Numerous intra- and extracellular hyaline globules, as well as Schiller-Duval bodies, could also be identified. Immunohistochemical studies showed positive reaction with alpha-fetoprotein antibodies in the tumor cells. Follow-up information obtained in these patients showed that all had died of tumor 1 to 3 years after initial diagnosis. The present cases document another unusual but distinctive growth pattern of yolk sac tumor in the mediastinum and highlight the importance of proper sampling of cystic lesions in this location.
The authors report a rare case of a female infant who underwent successful treatment of a mediastinal yolk sac tumor. Therapy included four cycles of preoperative PVB (cisplatinum, vinblastine, bleomycin), complete surgical resection of the tumor, and one postoperative cycle of PVB and four cycles of VAC (vincristine, actinomycine, cyclophosphamide) chemotherapy. The successful treatment of this tumor depends on preoperative chemotherapy to reduce the size, followed by complete surgical excision with subsequent postoperative chemotherapy. Recent improvements in the treatment of mediastinal yolk sac tumors are discussed.
A case is reported of yolk sac tumor occurring in the left ovary and complicated by pregnancy. The 22-year-old patient presented at 28 weeks gestation with virilization and elevated serum levels of testosterone and alpha-fetoprotein. The tumor showed the typical features of yolk sac tumor with a mixture of islands of Leydig cells. The accumulations of Leydig cells were well demarcated from the cellular components of the yolk sac tumor and were distributed throughout the tumor, although with predominant localization at the periphery. By immunohistochemistry the Leydig cells were intensely positive for vimentin and negative for cytokeratins, allowing clear distinction from the cell components of the yolk sac tumor, which were positive for cytokeratins and negative for vimentin. Testosterone was also identified in the cytoplasm of the Leydig cells. After tumor resection the testosterone and alpha-fetoprotein levels declined simultaneously; this, together with the immunohistochemical demonstration of testosterone, indicates that the Leydig cells were responsible for the endocrine manifestations. Furthermore, antibodies against inhibin alpha-subunit and calretinin could be used to detect the Leydig cells. The present case, a combination of yolk sac tumor and Leydig cells acting as a functioning stroma and causing virilization during pregnancy, is very rare.
An ovarian mucinous cystadenocarcinoma admixed with a component of yolk sac tumor was encountered in a 71-year-old woman, the first such tumor to be reported. The preoperative serum level of alpha-fetoprotein was elevated (55.6 ng/mL). A right ovarian cystic tumor, 8 cm in maximal dimension, was removed and found to have a solid component. Microscopically, the cystic lesion was a mucinous cystadenocarcinoma, whereas the solid portion was typical yolk sac tumor with microcystic, reticular, and labyrinthine patterns, Schiller-Duval bodies, scattered giant cells with bizarre nuclei, and intracellular and extracellular hyaline globules. There was no evidence of other germ cell tumor components. Yolk sac tumor showed positive reactions for alpha-fetoprotein, carcinoembryonic antigen, and alpha-1-antitrypsin but was negative for CA125, CA19-9, and human chorionic gonadotrophin. The patient died of recurrent tumor 6 months postoperatively without response to combination chemotherapy.
A 3-year-old female neutered Staffordshire Bull Terrier presented with a mixed germ cell tumor involving the base of the iris and the ciliary body of the right eye. The tumor mass was composed primarily of packeted vacuolated, polygonal (hepatoid) cells and small round cells; epithelial cells lining tubuloacinar structures were a less prominent component. The hepatoid and round cells stained positively for alpha-fetoprotein and cytokeratin. The epithelial cells stained positively for cytokeratin only, and some contained cytoplasmic mucin droplets. The polygonal cells were interpreted as a hepatoid variant of yolk sac tumor, and the epithelial cells were considered a teratomatous component. Trabeculae of bone were observed within the mass and may have been metaplastic or a teratomatous element. Extragonadal germ cell tumors are rare in dogs and have previously been reported only in the suprasellar region. This is the first report of this tumor type in the eye of a nonhuman species.
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Three cases of primary mediastinal yolk sac tumors with prominent spindle cell features are presented. The patients were three men 24-34 years of age (mean 29). Clinically, two patients presented with symptoms of chest pain and cough; no clinical information was provided for the third patient. Grossly, the tumors were described as large mediastinal masses, with a hemorrhagic and necrotic cut surface. Histologically, the tumors were characterized by a predominantly atypical spindle cell proliferation admixed with areas that showed focally the characteristic reticular growth pattern of yolk sac tumors, with the presence of Schiller-Duval bodies and intra- and extracellular hyaline globules. Immunohistochemical studies performed in one case showed positive staining for keratin and alpha-fetoprotein in both the spindle cell and reticular components of the tumor. Follow-up information was obtained in two patients; they both died of tumor with metastases to the lungs 1 year after initial diagnosis. The present cases expand the spectrum of histopathologic growth patterns that may be observed in yolk sac tumors of the mediastinum and stress the issue of careful sampling and evaluation of mediastinal neoplasms for arriving at the correct diagnosis.
An ovarian mixed germ cell tumor in a 34-year-old woman contained a predominant component of polyembryoma as well as foci of choriocarcinoma, yolk sac tumor, and immature teratoma. No previous cases of identical composition have been found in the literature.
To our knowledge, this case of an abdominal yolk sac tumor with extensive cardiac involvement is the first to be reported in a pediatric patient. The report describes the echocardiographic findings of the tumor and the role of echocardiography in providing important information for patient management decisions.