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Pathomorphology, clinic and therapy of endodermal sinus tumor in pregnancy are explained and discussed by means of a women of 25 years. The importance of estimation of alpha-fetoprotein with regard to diagnosis and follow up is referred, too. The prognosis in former times very bad could be improved by a combination of radical surgery and polychemotherapy.
Ataxia-telangiectasia, an inherited disorder characterized by progressive cerebellar ataxia and telangiectasias, is often associated with primary immunodeficiency and high incidence of malignancies, mostly of the lymphoreticular type. Endodermal sinus tumor is a rare germ cell tumor of the ovary characterized by an extremely rapid growth and poor prognosis. Both these diseases are associated with an abnormal production of alpha-fetoprotein. Primary tumors of the ovary in patients with ataxia-telangiectasia are extremely rare and the association of an endodermal sinus tumor and ataxia-telangiectasia has never been reported in the literature. This case report serves to focus on the particular problems encountered in the diagnosis and management of two diseases both characterized by the same serum marker.
The literature reflects a poor prognosis for women with endodermal sinus tumor. Two women with this neoplasm, 1 having stage IA and the other stage IIB disease were treated for 1 year postoperatively with a combination of actinomycin-D, 5-fluorouracil, and cyclophosphamide. Each had therapy discontinued after a negative second laparotomy and 15 and 21 months later are without recurrence. It is suggested that this drug regimen is a benficial adjunct to surgery in the treatment of endodermal sinus tumor.
Features of hepatic and enteric tissue have been detected in serial sections of 2 out of 6 endodermal sinus tumors (EST) and embryonal carcinomas of the testis showing endodermal sinus areas. Hepatic nests, which appeared as tubules, cords and giant multinucleated cells, were PAS-positive as well as immunoreactive for alphafetoprotein (AFP) and cytokeratin (CK). Enteric tubules were found within EST areas and were characterized by their lack of cell atypism. The presence of diffuse PAS-, AFP- and CK-reactivity in cytoplasm, together with a brush border or an apical staining for CEA, were quite distinctive. EST showed a potential for both intra- and extraembryonal endodermic development and may be interpreted as a monophyletic teratoma.
The human yolk sac is said to synthesize not only alpha-fetoprotein but also other serum proteins, i.e., albumin, prealbumin, alpha 1-antitrypsin, and transferrin. Two endodermal sinus tumors (yolk sac tumors) were successfully transplanted into athymic nude mice. Concentration of these human serum proteins in the sera of the nude mice and tumor extracts was determined quantitatively by electro-immunodiffusion and radioimmunoassay. alpha-Fetoprotein and albumin were detected in one case, and alpha 1-antitrypsin and alpha-fetoprotein in the other. In the former case, the amount of alpha-fetoprotein synthesized was larger than that of albumin, whereas the amount of alpha-fetoprotein was smaller than that of alpha 1-antitrypsin in the latter. It is tempting to consider that human endodermal sinus tumors simulate the human yolk sac functionally, but the pattern of protein synthesis may differ from case to case.
Most adult germ cell tumors have a consistent cytogenetic abnormality, i(12p), and are aneuploid. Many pediatric germ cell tumors are biologically distinct from their adult counterparts, particularly endodermal sinus tumors (ESTs) of young children. We report cytogenetic and ploidy analysis of nine ESTs involving children under 3 years of age (four extragonadal and five testicular). Structural abnormalities were present in seven tumors and were identifiable in six: 5/6 had a structural abnormality of chromosome 1, usually terminal deletion of 1p; 5/6 showed 6q deletion; 3/6 had structural abnormalities of 3p; 2/6 showed abnormalities of chromosome 2. None showed an i(12p) or abnormality of chromosome 12. Ploidy analysis of the tumors correlated with the cytogenetic analysis; in particular, the tumor that was cytogenetically normal showed no aneuploid peaks. To determine if a marker chromosome was derived from chromosome 12 or if karyotypically normal cases included nondividing tumor cells, interphase fluorescence in situ hybridization using an alpha satellite probe for chromosome 12 was performed. These studies showed no evidence of an i(12p). We conclude that ESTs in young children show cytogenetic differences from their adult counterparts and that loci on 1p, 6q, and 3q need to be further studied.
The case presented is a 17-year-old male patient with a pineal region endodermal sinus tumor manifested by obstructive hydrocephalus and upward gaze limitation. The highly aggressive nature of this rare malignancy was evidenced by spontaneous tumor bleeding. The patient was treated with a cerebrospinal fluid (CSF) shunt and microsurgical tumor excision followed by irradiation and combination chemotherapy consisting of cisplatin, vincristine, methotrexate and bleomycin. The response to therapy was monitored by serial measurements of serum and CSF alpha-fetoprotein level. The patient remains free of any clinical or radiographic evidence of tumor recurrence 38 months after the initial diagnosis. This experience supports the rationale for an aggressive multidisciplinary approach toward this rare and highly malignant neoplasm.
Fine-needle aspiration biopsies from five patients with endodermal sinus tumors (ESTs) were reviewed, and the findings were correlated with histologic and ultrastructural appearances. In the aspiration smears, two types of tumor cells were seen, forming clusters of variable sizes. Type A cells had distinct cell borders, and their cytoplasm contained only occasional vacuoles. Type B cells had ill-defined cell borders and formed syncytial clusters; their cytoplasm was characterized by large numbers of rounded vacuoles. The background contained patches of mucoid material and macrophages with foamy cytoplasm. Eosinophilic hyaline cytoplasmic bodies and irregular deposits of intercellular basement membrane-like material were recognized easily in aspiration smears. These features correlated well with histologic and ultrastructural appearances. The significance of these findings in the fine-needle aspiration biopsy diagnosis of EST and its distinction from other germ-cell and non-germ-cell tumors is discussed.
A 76-year-old woman underwent surgery for pelvic mass, during which a 13 x 8-cm right ovarian tumor was discovered. On histopathological examination, she was diagnosed with an endodermal sinus tumor with right tubal metastasis. The patient was treated with four cycles of Bleomycin, Etoposide and Cisplatin. She died of disseminated disease four years later.
We encountered an unusual ovarian tumor consisting of a mixture of typical endodermal sinus tumor (EST) and mucinous cystadenofibroma that occurred in the ovary of an 82-year-old female patient. The EST component showed the classic histologic features of this tumor. Serum alpha-fetoprotein (AFP) level was not determined. Tumor stains were negative for AFP but positive for alpha-1-antitrypsin. The malignant germ cell component was intimately associated with the benign mucinous component. Focal production of epithelial mucin and carcinoembryonic antigen (CEA) in the EST component suggested a probable association between the two tumor types. The tumor was confined to one ovary, and the patient is disease-free 2 years after surgical therapy. This neoplasm is unique not only for the malignant germ cell component occurring in an 82-year-old woman, but for the unusual combination of tumor types. The pathogenesis is unknown.
We report a case of a 16-month-old girl with a primary endodermal sinus tumor (EST) of the parotid gland. The girl was admitted to the hospital with a left side cervical tumor with a quick growth (within 3 wk). The tumor was surgically resected. The gross examination showed a 7.0-cm hemorrhagic and fragmentized mass. Diagnosis of an EST was established on the hematoxylin and eosin-stained slides from the resected material. Elevated serum alpha-fetoprotein (AFP) levels were detected and follow-up examinations of the patient did not show ESTs in other locations or other manifestations due to the tumor. The patient received chemotherapy after the surgery and remained alive without evidence of disease at the time of this writing (2 years after the diagnosis). As far as we know, this is the second report in the literature of a parotid gland's EST.
This case report concerns an endodermal sinus tumor (EST) arising in the pineal region of a 16-year-old boy who died 3 months after radiation. He developed extensive abdominal metastases through a ventriculoperitoneal shunt, whereas the primary tumor and a suprasellar metastasis could be controlled by radiotherapy, demonstrated by autopsy. The histologic diagnosis was supported by an elevated level of alpha-fetoprotein in serum and the demonstration of this marker in the tumor tissue by immunoperoxidase method. The poor diagnosis of all previously reported cases with pineal EST requires a combined modality of surgical approach, radiotherapy, and concomitant chemotherapy.
A primary yolk sac (endodermal sinus) tumor of the prostate is described in a 40-year-old male. This is the second documented case of a yolk sac tumor occurring in the prostate gland. Treatment included surgical removal and subsequent combination chemotherapy. Four months following diagnosis, the patient died of complications. At autopsy, no residual tumor was detected. The role of combination chemotherapy as potentially curative therapy in the treatment of extragonadal yolk sac tumors is discussed.
Primary endodermal sinus tumor (EST) of the mediastinum has been regarded as a rare and rapidly fatal germ cell neoplasm. We describe two cases of extensive EST treated with a new high-dose sequential combination chemotherapy regimen (CISCA-VB) followed by radical surgical excision. They are alive at 11 and 20 months, respectively, postoperatively. These cases stand in marked contrast to previously reported series. A new approach to the management of this tumor is proposed.
Forty-one patients with pure endodermal sinus tumor of the ovary who were treated at the M. D. Anderson Hospital from 1944 to 1981 are retrospectively reviewed. The median age of these patients was 19 years. The most common presenting symptom was abdominal pain, occurring in 33 patients (80%). No bilateral ovarian involvement was noted at initial surgery. With the exception of 1 patient treated by surgery alone, only patients treated with postoperative combination chemotherapy survived. Twenty-one of the 41 patients are alive and well. Sixteen of 22 patients treated with vincristine, actinomycin-D, and cyclophosphamide have survived. The roles of second-look laparotomy and alpha-fetoprotein monitoring are discussed. Optimal treatment seems to consist of surgery followed by aggressive combination chemotherapy.
This is a case report of an endodermal sinus tumor occurring in the pineal region of a 14-year-old boy who died 4 weeks after surgery and irradiation. Germ cell tumors of this type demonstrate a selective overgrowth of yolk sac endoderm associated with extraembryonic mesoblast and arise preferentially in the gonads of young children. This tumor in the pineal region is rare and carries a grave prognosis as in other extragonadal sites. A summary of previously reported cases is also presented.
The authors present the clinical history of a 15-year-old young woman, student, from David, Chiriquí, who complained of menstrual irregularities associated with the sensation of a mass in the right iliac fossa. Exploratory laparotomy revealed a right ovarian tumor, which was totally resected. The histopathological examination showed an endodermal sinus tumor, stage one, in the right ovary. The patient received four cycles of cytotoxic i.v. therapy every 3 weeks, after surgery, with vinblastine 6 mg/m2 body surface (B.S.) on days 1 and 2, bleomycin 15 u daily for 5 days, cisplatin 20 mg/m2 B.S. daily for 5 days, plus hydration with normal saline (5 to 6 l/day). Follow up studies with abdominal and pelvic sonograms, chest x-ray, feto protein and physical examination have not shown evidence of tumor activity 36 months after diagnosis.
We report on a case of a 46-year-old woman with a conus-cauda syndrome due to an endodermal sinus tumor of the right ovary with multiple metastases in the spine and pelvic bone. Before removing the tumor surgically, combination chemotherapy was given to treat the metastases, which threatened to compromise the spinal cord.