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Endodermal sinus tumor and mucinous cystadenofibroma of the ovary. Occurrence in an 82-year-old woman.

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.

Adenofibroma↗

Primary endodermal sinus tumor of the endometrium. A clinicopathologic, immunocytochemical, and ultrastructural study.

We report a case of primary endodermal sinus tumor (EST) of the endometrium in a 42-year-old female. Although numerous extragonadal EST have been reported, primary EST of the endometrium is exceedingly rare. To our knowledge this is the fourth documented case of this nature. The tumor had the typical microscopic features of EST, with papillary, tubular, reticular, and solid growth patterns; occasional Schiller-Duval bodies and many intracellular and extracellular periodic-acid Schiff positive hyaline globules were seen. The neoplastic cells stained positively for alpha-fetoprotein (AFP), alpha-1-antitrypsin (A1AT), cytokeratin, and placental alkaline phosphatase. The globules were positive for AFP, A1AT, albumin, transferrin, and fibronectin. The tumor cells were negative for type IV collagen and the beta subunit of human chorionic gonadotropin (B hcG). Electron microscopic examination showed intracellular and extracellular basement membrane-like material, intracytoplasmic lumina with microvilli, and glycogen. The patient was treated with total abdominal hysterectomy and bilateral salpingo-oophorectomy, followed by four cycles of adjunct chemotherapy (vinblastine, bleomycin, and cisplatinum) repeated every 3 weeks. The serum AFP level was elevated significantly before the surgery and the tumor response was monitored by serial determination of serum AFP level. There was no evidence of recurrence 24 months after surgery.

Adult↗

Successful treatment of a primary endodermal sinus tumor of the liver.

A 27-year-old woman had a large hepatic tumor and a markedly increased serum alpha-fetoprotein (AFP) level. A diagnosis of endodermal sinus tumor was made after a needle biopsy was performed on the liver. Clinical and radiologic examinations did not show an alternative primary site. Treatment with cisplatin, etoposide, and bleomycin was started, but, after three cycles, was changed to cisplatin, vincristine, methotrexate, bleomycin, dactinomycin, cyclophosphamide, and etoposide because the serum AFP level was decreasing too slowly. After additional chemotherapy was given, the patient was well but had an increased AFP level and a large residual mass in the liver. A right hemihepatectomy was performed, but no viable tumor was present. The patient is alive and disease-free 5 years later. Thus, AFP levels may be misleading in the presence of large necrotic tumors. The authors stress the need to make a diagnosis of these rare tumors early because aggressive treatment with combination chemotherapy may result in cure.

Adult↗

Fine-needle aspiration biopsy diagnosis of endodermal sinus tumor: histologic and ultrastructural correlations.

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.

Adult↗

Expression analyses of zebrafish transferrin, ifabp, and elastaseB mRNAs as differentiation markers for the three major endodermal organs: liver, intestine, and exocrine pancreas.

In the present work, three zebrafish cDNA clones encoding transferrin, intestinal fatty acid binding protein (IFABP), and elastaseB were cloned and their expression patterns in early zebrafish development were characterized as differentiation markers for the three major endoderm organs: liver, intestine, and exocrine pancreas. transferrin and ifabp mRNAs exhibit a biphasic expression pattern during early development. transferrin mRNAs were first expressed at approximately 7 hours postfertilization (hpf) in the yolk syncytial layer (YSL) and later in the liver rudiment (from approximately 48 hpf) and in the esophagus transiently (72-96 hpf). Ifabp mRNAs were initially expressed in the YSL at the ventral side during late epiboly (8-9 hpf), spread throughout the YSL of later stage embryos, and appeared in the intestine rudiment at approximately 36 hpf. In contrast to the transferrin and ifabp mRNAs, elastaseB mRNAs were not expressed in the yolk sac or YSL, and these transcripts were detected exclusively in the exocrine pancreas after approximately 56 hpf.

Amino Acid Sequence↗

The endodermal sinus tumor: special features.

Eight cases of endermal sinus tumor, two extragonadal and six gonadal, seen at the American University of Beirut are reviewed. Three of them present special clinical and pathologic features that further substantiate the extraembryonic origin of this teratoid tumor and its association with dysgenetic gonads. We consider the two histologic types, endodermal sinus pattern and polyvesicular vitelline architecture, to be part of the same tumor. The prognosis in this series was unfavorable.

Adolescent↗

Endodermal sinus tumor of the ovary and alpha fetoprotein: a case report.

Yolk sac tumors of the ovary are associated with elevations in serum levels of alpha fetoprotein (AFP). Thus, an antigenic marker is provided for evaluating therapeutic results in selected patients. A patient with a pure endodermal sinus tumor of the ovary is presented who underwent serial determinations of AFP. The effectiveness and limitations of serial AFP levels as demonstrated by this case are discussed in addition to the surgical and chemotherapeutic management of patients with yolk sac tumor of the ovary.

Adult↗

Aggressive chemotherapy in endodermal sinus tumor.

Twenty-eight patients with pure or predominantly endodermal sinus tumor were studied for their clinical behavior, relation with serum alpha-fetoprotein (AFP), and response to intensive postoperative combination chemotherapy. Eight percent of the patients were younger than 20 years of age. One patient was a pseudohermaphrodite. Serum AFP was estimated in 17 patients and was elevated in all. Seven patients were treated with adjuvant chemotherapy following surgery with a cisplatin-containing combination. Five patients (71%) are alive and continue to remain free of disease, with a median survival of 28 months after treatment and an overall median survival of 34 months. Two patients treated with adjuvant chemotherapy experienced early recurrence of the disease, which was detected with a rise in serum AFP before the clinical manifestation of recurrence.

Adolescent↗

Primary endodermal sinus tumor of the prostate: report of a case.

A 29-year-old man was admitted to the Department of Urology of the University of Padova with a diagnosis of prostatic adenocarcinoma. Cystourethrography showed a prostatic urethral obstruction, and CT scan demonstrated a low pelvic solid mass involving the prostate and the seminal vescicle. A radical prostatectomy was performed, and the prostate was found to be widely infiltrated by a neoplasia formed by tubules, papillary structures, and solid cords of cuboidal or flat elements, each having indistinct borders and scant cytoplasm, in a myxoid or fibrous stroma. "Schiller-Duval-like," PAS-diastase-resistant and alfa-fetoprotein hyaline bodies were present. An endodermal sinus (yolk-sac) tumor was diagnosed. There was infiltration of the periurethral prostatic ducts, prostatic urethra, and seminal vesicles and single lymph node metastasis. Testicular echotomography was negative. In spite of aggressive surgery and chemotherapy, the patient died 10 months after diagnosis. This is the third case in the literature after those presented by Benson and Michel.

Adenocarcinoma↗

Primary yolk sac (endodermal sinus) tumor of the prostate: case report and review of the literature.

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.

Adult↗

Extragonadal endodermal sinus tumors in children: a review of 24 cases.

Endodermal sinus tumors (EST) are highly malignant lesions with a tendency to recur locally and with a high incidence of metastatic disease at the time of diagnosis. The clinical and radiographic spectra of 24 patients with extragonadal EST are reported. There were 17 females and seven males and the mean age of presentation was 21 months. The primary lesion occurred in the sacrococcygeal region in 16 patients; in the vagina in two patients; in the anterior mediastinum in two patients; and in the bladder, liver, nasopharynx and posterior cranial fossa in one patient each. Computed tomography (CT) is the most accurate study in delineating the extent of primary lesions particularly in the pelvis and head and neck and also for demonstrating metastatic disease in the lungs. Significant improvement in survival rates is obtained with a combination of surgery, chemotherapy and radiotherapy.

Child, Preschool↗

Extragonadal endodermal sinus (yolk sac) tumor. Angiographic findings and literature review.

The angiographic and pathologic features of an endodermal sinus tumor (EST) arising from the retroperitoneum in a 19-month-old boy are presented. Although rare instances of extragonadal origin of EST have been reported, this is the first detailed description of the angiographic abnormalities. The angiographic appearance was that of a primarily retroperitoneal tumor with parasitized feeding arteries from the liver and right kidney, early filling venous radicles, sinusoidal pooling of contrast material, and inferior vena caval obstruction.

Aortography↗

Endodermal sinus tumour associated with benign teratoma of the common bile duct.

We report a 5-year-old boy with endodermal sinus tumour associated with benign cystic teratoma of the common bile duct (CBD). To our knowledge, there has been one case of teratoma of the CBD in the English literature with no morphological or radiological description. Our case presented a lobulated polypoid mass obstructing the distal CBD on sonography and computed tomography, which resembled the botryoid masses of rhabdomyosarcoma.

Child, Preschool↗

Endodermal cyst of the foramen magnum: case report and review of the literature.

The majority of endodermal cysts occur in the cervicothoracic spine, ventral to the cord. Intracranial locations are rare. We report a case involving the foramen magnum in a 14-year-old child, which was an incidental finding following a traumatic head injury. A review of the literature revealed six other cases involving this same location. These lesions are asymptomatic for a long time, and may cause brain stem medullary compression. Treatment is surgical. Effective simple removal can be achieved by a posterior approach.

Adolescent↗

Chemical analysis and immunolocalisation of lignin and suberin in endodermal and hypodermal/rhizodermal cell walls of developing maize (Zea mays L.) primary roots.

The composition of suberin and lignin in endodermal cell walls (ECWs) and in rhizodermal/hypodermal cell walls (RHCWs) of developing primary maize (Zea mays L.) roots was analysed after depolymerisation of enzymatically isolated cell wall material. Absolute suberin amounts related to root length significantly increased from primary ECWs (Casparian strips) to secondary ECWs (suberin lamella). During further maturation of the endodermis, reaching the final tertiary developmental state characterised by the deposition of lignified secondary cell walls (u-shaped cell wall deposits), suberin amounts remained constant. Absolute amounts of lignin related to root length constantly increased throughout the change from primary to tertiary ECWs. The suberin of Casparian strips contained high amounts of carboxylic and 2-hydroxy acids, and differed substantially from the suberin of secondary and tertiary ECWs, which was dominated by high contents of omega-hydroxycarboxylic and 1,omega-dicarboxylic acids. Furthermore, the chain-length distribution of suberin monomers in primary ECWs ranged from C(16) to C(24), whereas in secondary and tertiary ECWs a shift towards higher chain lengths (C(16) to C(28)) was observed. The lignin composition of Casparian strips (primary ECWs) showed a high syringyl content and was similar to lignin in secondary cell walls of the tertiary ECWs, whereas lignin in secondary ECWs contained higher amounts of p-hydroxyphenyl units. The suberin and lignin compositions of RHCWs rarely changed with increasing root age. However, compared to the suberin in ECWs, where C(16) and C(18) were the most prominent chain lengths, the suberin of RHCWs was dominated by the higher chain lengths (C(24) and C(26)). The composition of RHCW lignin was similar to that of secondary-ECW lignin. Using lignin-specific antibodies, lignin epitopes were indeed found to be located in the Casparian strip. Surprisingly, the mature suberin layers of tertiary ECWs contained comparable amounts of lignin-like epitopes.

Cell Wall↗

The Xenopus bagpipe-related homeobox gene zampogna is expressed in the pharyngeal endoderm and the visceral musculature of the midgut.

In Drosophila, the bagpipe homeobox gene is expressed in a subset of dorsal mesodermal cells and in the absence of bagpipe gene function, development of the visceral musculature is disrupted. In Xenopus, one bagpipe-related gene, Xbagpipe (Xbap) has previously been described. Here we report the isolation of a second Xenopus homologue named zampogna (zax). zax is transcribed within the muscular layer of the forming midgut as well as in the embryonic head, where zax transcripts mark both the pharyngeal endoderm and the future infrarostral cartilage.

Amino Acid Sequence↗