Intracranial Yolk Sac Tumor Research Articles

A case of intracranial yolk sac tumor with repeated metachronous recurrences

A case of intracranial yolk sac tumor with repeated metachronous recurrences

Yolk sac tumor of the bilateral basal ganglia in a patient with Down syndrome

We report a case of intracranial yolk sac tumor in a 17-year-old girl with Down syndrome who presented with left slowly progressive hemiparesis. Initial magnetic resonance imaging (MRI) showed a small area of ill-defined abnormal signal intensity without a mass in the bilateral basal ganglia. These findings progressed very slowly for 2 years, but then the lesion rapidly progressed to a huge mass on MRI 4 months after the previous scan. The patient subsequently underwent a blood examination and endoscopic biopsy, and was finally diagnosed with yolk sac tumor. Standard platinum-based chemotherapy and radiotherapy were performed as soon as practicable. The frequency of solid cancer in patients with Down syndrome is generally lower than that in individuals without this disorder. Brain tumor is also rare in Down syndrome, but half of these cases are germ cell tumors. Moreover, slowly progressive non-tumor-like lesions in the basal ganglia may also be germ cell tumors. Therefore, MRI signal abnormalities in the basal ganglia in patients with Down syndrome require careful and frequent follow-up scans based on the possibility of a germ cell tumor that may exhibit rapid growth.

Primary intracranial yolk sac tumor in the posterior fossa: Case report of a child with Down syndrome

Ultrasmall superparamagnetic iron oxide (USPIO) particles to enhance MRI have been used to study neuroinflammation in vivo. Our purpose was to observe the USPIO-enhanced MR signal alterations in the primary ischemic lesion and ipsilateral substantia nigra after middle cerebral artery occlusion (MCAO) to verify the subsequent sequelae of neuroinflammation seen in the primary ischemic focus and secondary degeneration region.Sprague–Dawley rats were subjected to transient MCAO. In addition to conventional T2-, T1-weighted imaging, USPIO-enhanced MRI was performed in USPIO-injected stroke rats, while Gd-enhanced imaging was acquired in control stroke rats, on days 3, 6 using a 3-T MR scanner. The MR signal characteristics in the primary ischemic striatum, ipsilateral substantia nigra were noted, compared on histopathological H&E, Prussian blue (PB) staining.After MCAO, USPIO-induced T2 hypointensity changes were observed in the primary ischemic region with BBB impairment at both time points. In the substantia nigra ipsilateral to the primary ischemic lesion, there was no evidence of USPIO accumulation detected by MRI and PB staining, and no BBB leakage reflected by Gd-enhanced imaging on days 3 and 6.USPIO-enhanced MR signals have variable characteristics in both primary and remote sites after focal cerebral ischemia. This suggests that the neuroinflammatory response to brain ischemia in the primary ischemic focus and secondary degeneration region have different temporal patterns and pathophysiological mechanisms.

A clinicopathologic analysis of primary orbital yolk sac tumor

Objective On rare occasions, yolk sac tumor may arise from extragonadal sites. Orbit is an unusual location for the primary development of this tumor. The presence of intracranial yolk sac tumor on biopsy always makes the diagnosis challenge for pathologists. Herein we report a case of intracranial primary yolk sac tumor in left orbital apex. The clinicopathology of this tumor and its differential diagnosis are discussed. Methods The clinical manifestation of a patient with primary yolk sac tumor occurring in left orbital apex was presented retrospectively. Resected orbital mass was routinely paraffin⁃embedded and stained with hematoxylin and eosin. Dako Envision immunohistochemical staining system was used to detect the tumor antigen expressions, including alpha ⁃fetoprotein (AFP), cytokeratin, placental alkaline phosphatase (PLAP), CD30, CD34, CD45, CD99, CD117, synaptophysin (Syn) and chromogranin A (CgA). Results A 2⁃year⁃old boy presented with 3⁃month history of ptosis of eyelid and exophthalmos on left eye. Magnetic resonance imaging (MRI) scan revealed a lesion occupied the left orbital apex with infiltration of surrounding normal structure, including left posterior ethmoid sinus, the wall of left maxillary sinus and sphenoid. However, there was no evidence of tumor infiltrating in brain parenchyma. Craniotomy was performed and the tumor was removed by en bloc resection. Grossly, the biopsy specimen received in small pieces was 1.20 cm × 1.00 cm × 1.00 cm when aggregated. No fibrous capsule, necrosis, haemorrhage and gross calcification were found in the tissue fragments. Histological examination revealed that the tumor was arranged in a reticular pattern. The cells were relatively large with a clear cytoplasm and vesicular nuclei. Scattered mitotic figures were noted. Schiller ⁃ Duval bodies and periodic acid ⁃ Schiff (PAS) positive (eosinophilic bodies) were evident in the tumor tissue. Immunohistochemical staining showed that the tumor cells were diffusely positive for cytokeratin (AE1/AE3) and focal positive for AFP, CD99 and CD117, but negative for PLAP, CD30, S ⁃ 100, CD45 and CD34. There was no evidence of mixture of other germ cell tumor component in this tumor by serial sections. Based on clinical presentation and histological findings, a final histological diagnosis of pure primary orbital yolk sac tumor, WHO grade Ⅳ, was made according to the criteria of WHO classification. The patient has not received chemotherapy and attended follow⁃up for 3 months, without any neurological deficit or signs of recurrence. Conclusion Despite the lower incidence, intracranial yolk sac tumors usually develop in the midline at the pineal or suprasellar regions occurring in children with distinctive histological features and immunohistochemical phenotypes. In general, intracranial yolk sac tumors are known to entail poor prognosis even after multidisciplinary treatment of operation, radiotherapy, and chemotherapy. It is noted that intracranial yolk sac tumor should be differentiated histologically from other types of germ cell tumors and mixed germ cell tumor. DOI：10.3969/j.issn.1672⁃6731.2012.01.019

Intracranial yolk sac tumor in an adult patient: MRI, diffusion-weighted imaging and 1H MR spectroscopy features

Yolk sac tumors represent only 5%-7% of intracranial germ cell tumors, which comprise about 1% of all primary brain tumors in adults. Literature data about nonspecific imaging characteristics of these tumors are scant. We presented magnetic resonance imaging findings with diffusion-weighted imaging and proton magnetic resonance spectroscopy of this rare type of tumor in an adult patient. A 55-year-old man with progressive left side weakness, headache, dizziness and ataxia, underwent preoperative magnetic resonance imaging, diffusion-weighted imaging and proton magnetic resonance spectroscopy. After surgical resection and histological analysis, the final diagnosis of yolk sac tumor was established. Retrospective imaging analysis were performed in order to determine imaging and biochemical parameters that could be useful in the diagnostic evaluation of this tumor type. Though the imaging features of yolk sac tumor are not specific, morphoanatomical and metabolic imaging could offer the information that provides new insights into this tumor that may facilitate further therapeutic decision process and potentially provides better information regarding the disease prognosis.

Intracranial yolk sac tumor in a patient with Down syndrome

The authors report a rare case of intracranial yolk sac tumor in a 13-year-old boy with Down syndrome who presented with left hemiparesis. Admission MR imaging revealed a tumor in the right basal ganglia. Serum α-fetoprotein was markedly elevated. Yolk sac tumor was diagnosed radiologically and serologically. The standard therapy for intracranial yolk sac tumor is platinum-based chemotherapy with concomitant radiotherapy. However, the authors used reduced-dose chemotherapy and asynchronized radiotherapy because of the well-known low tolerance of patients with Down syndrome to chemotherapy. This treatment was successful with no complications. Blood cancers are frequently associated with Down syndrome, whereas solid tumors occur less frequently in these patients, and the risk of chemoradiotherapy is unclear. The results indicate that dose-reduction therapy can be effective for treatment of a brain tumor in a patient with Down syndrome.

Unusual anatomic location of a primary intracranial yolk sac tumor

A nongerminomatous germ cell tumor occurring in the brain parenchyma is extremely rare. A 2-year-old boy presented with symptoms of abnormal movements in the hand and mouth. MRI scanning revealed a lesion occupying the left temporoparietal region. Craniotomy was performed and the tumor was removed by en bloc resection. Histological examination revealed that the tumor was arranged in a reticular pattern, and Schiller-Duval bodies were evident at the center of the tumor. Immunohistochemical study showed that the tumor cells were positive for alpha-fetoprotein and vimentin, but negative for glial fibrillary acidic protein. The histological diagnosis was pure yolk sac tumor).

Unusual Anatomic Location of a Primary Intracranial Yolk Sac Tumor

Unusual Anatomic Location of a Primary Intracranial Yolk Sac Tumor

Primary Yolk Sac Tumor of Bilateral Basal Ganglia

A primary intracranial yolk sac tumor (YST) is a type of germ cell tumor (GCT) and usually involves the pineal or suprasellar regions, as do other GCTs. Primary YST in the basal ganglia is not common, and bilateral basal ganglia involvement is even rarer. Early diagnosis is often difficult because of minimal or subtle findings without space-occupying lesions shown on neuroimaging during the early course of the disease. We report a case of primary intracranial YST encountered in the basal ganglia bilaterally and describe the clinical presentation, diagnostic problem, imaging characteristics, histopathologic features, and prognosis of the tumor. To the best of our knowledge, this is only the third reported case of primary YST confined to the basal ganglia in the literature.

Preliminary observations for a new treatment in children with primary intracranial yolk sac tumor or embryonal carcinoma. Report of five cases.

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.

Aziridinylbenzoquinone (AZQ) in the treatment of recurrent pediatric brain and other malignant solid tumors

To assess the response rates and toxicity of AZQ in children with recurrent brain and other malignant solid tumors, a phase II study was implemented by the Pediatric Oncology Group. Eligible patients received AZQ 18 mg/M2/week i.v. for 4 doses followed by a 2 week rest period. Each dose was given over four hours (1/3 over the initial 20 minutes). After the first year, the dosage was reduced to 13 mg/M2 due to myelotoxicity resulting in treatment delays. No objective responses were observed in 73 evaluable children with various non-central nervous system tumors. Of the 91 patients with brain tumors, there were 4 CR's and 2 PR's in patients with astrocytoma, ependymoma, glioblastoma multiforme, oligodendroglioma, brain stem glioma and intracranial yolk sac tumor (median duration, 10 months; range, 2-20+ months). Three of 4 CR's were achieved with a dosage of 18 mg/M2/week. An additional 13 children with brain tumors experienced stable or improved disease (duration, 2-36 + months; median 7.5 months). The principal toxicity was myelosuppression which was cumulative but there were also 3 allergic reactions to AZQ. We conclude that for selected brain tumors, the rates of objective response and stable disease plus the duration of responses support further assessment of AZQ in combination with other agents. Furthermore, the 18 mg/M2 dosage may provide better responses.

Primary Pineal Yolk Sac Tumor: Ultrastructural and Immunohistochemical Features

In an autopsy of a 16-year-old female with a primary intracranial yolk sac tumor (endodermal sinus tumor), histological evaluation of the surgical specimen obtained from the pineal region showed proliferation of undifferentiated neoplastic embryonal cells without characteristic histological features, such as periodic acid-Schiff-positive hyaline droplets or Schiller-Duval bodies. Ultrastructural study of the tumor cells, however, revealed typical cytological features of yolk sac tumor, including a voluminous, homogeneous, basement membrane-like substance, both within and outside of the cells, and intracytoplasmic vesicles containing amorphous material. Alpha-fetoprotein was detected in serum and cerebrospinal fluid by radioimmunoassay and within tumor tissues by immunohistochemical techniques. After surgery, chemotherapy with cis-diamminedichloroplatinum (II), vinblastine, and bleomycin was employed without significant benefit, and the patient died 5 months after tumor removal. Histological examination of the tumor at autopsy supported the diagnosis of yolk sac tumor. Electron microscopic and immunohistochemical studies of germ cell tumors are discussed.

Ultrastructural study of intracranial yolk sac tumor: with special reference to the oncologic phylogeny of germ cell tumors.

Ultrastructural studies of yolk sac tumors (YST) have generally demonstrated the resemblance of this neoplasm to the primitive human yolk sac. However, these studies have failed to explain the common occurrence of mixed germ cell tumors with yolk sac elements. This report describes the ultrastructural features of a suprasellar YST. This tumor resembled those previously described. Also demonstrated, however, was the presence of primitive germ cells admixed within the neoplasm. A new schema is proposed based on these observations for the interrelationship of germ cell tumors, which may explain the common occurrence of mixed germ cell tumors.

Abdominal Metastases of Primary Intracranial Yolk Sac Tumors through Ventriculoperitoneal Shunts

Three cases of rare primary intracranial yolk sac tumor are reported. Two cases had a pineal location, whereas the third presented as a suprasellar mass. After the placement of ventriculoperitoneal shunts for relief of hydrocephalus, all of the patients developed metastases restricted to the peritoneum, as demonstrated by autopsy in one patient (Case 1) and clinical and radiographic evidence in two patients (Cases 2 and 3). The peritoneal metastases were directly associated with the death of one patient, but were successfully treated with chemotherapy in another patient, who is still alive more than 2.5 years after initial presentation. The value of ascitic fluid cytology and alpha-fetoprotein determination in the diagnosis of this complication was demonstrated in one patient. These cases emphasize the need for awareness of this mode of metastasis and its potentially lethal effect.