Extraneural metastasis of pineal germinoma through a ventriculoperitoneal shunt, following histological change

Primary germinoma of the central nervous system carries a good prognosis because of their radiosensitivity. Recurrence is rare and extraneural metastases are even more unusual. We report a unique case of a primary intracranial germinoma exhibiting complete response to radiotherapy, but recurring as an intra-abdominal yolk sac tumor. The presence of a VP shunt is thought to have facilitated metastatic spread of the intracranial neoplasm. An 21-year-old (corrected) male was admitted with headache and consciousness disturbance. Computed tomography (CT) revealed an enhanced tumor of the pineal region and hydrocephalus. A ventriculo-peritoneal shunt was emplaced immediately. No serum tumor markers such as alpha-fetoprotein or human chorionic gonadotropin were detectable. A test dose of radiotherapy (whole brain 20Gy) was given. The tumor size was remarkably decreased. Clinical diagnosis was germinoma. Additional whole brain radiation (total 45Gy) and whole spine radiation (20Gy) were given. The tumor and the hydrocephalus regressed completely and the patient returned to school. Three years later, he experienced a feeling of abdominal fullness. CT and echotomography of the abdomen showed a large peritoneal and intra-hepatic tumor. But MRI revealed no recurrent tumor of the pineal lesion or of the other areas in the central nervous system. Radiological and clinical findings showed no tumors in the testis, the retroperitoneal cavity, or the thymus. Laboratory investigation demonstrated elevated serum AFP (26,550 ng/ml). AFP level regressed after combined chemotherapy. However, the patient died due to pneumonia and multiple organ failure. Only needle necropsy was performed. The microscopic appearance of the peritoneal tumor was confirmed to be an endodermal sinus tumor. It was suspected to be a metastasis of the pineal tumor through the V-P shunt system.     

Large abdominal pseudocyst as a complication of ventriculoperitoneal shunt--diagnosis by ultrasonography and whole body CT scan (author's transl)]

We report a case in which a large abdominal pseudocyst developed following a ventriculo-peritoneal shunt (V-P shunt) for hydrocephalus due to pineal germinoma. A 24-year-old man had undergone a V-P shunt and radiotheraphy for unbiopsied posterior third ventricle tumor. Four years after operation he was readmitted because of double vision, conjugate upward gaze palsy and a large abdominal mass. Preoperative diagnosis of abdominal pseudocyst was made by abdominal computed tomography and ultrasonotomography. The shunt tube was clearly deomonstrated in the cavity by computed tomography. It is suggested that, in a patient suspicious of having abdominal pseudocyst who developed abdominal symptoms and signs following V-P shunt, abdominal computed tomography and/or ultrasonotomography provide a definitive diagnosis of this entity.     

Malignant pineal region tumors. A clinico-pathological study.

Eight patients with primary malignant pineal tumors have been seen at this institution over the past 6 years; six of them underwent definitive surgical exploration. Complete gross microsurgical excision of well encapsulated tumors was possible in four of these patients. In two cases of pineal germinomas, a biopsy and a subtotal resection were carried out because of the known radiosensitivity of this tumor. These six surgical patients all received postoperative craniospinal radiation and continue to do well up to 6 years postoperatively. Two nonoperative patients were initially treated at other institutions by ventriculoperitoneal shunt and radiation and were the only ones to develop metastatic disease. One patient had metastasis of her pineoblastoma to her unirradiated spinal canal and the other patient had metastasis of his germinoma to the peritoneum. The former patient was quadriplegic on admission, although her pineal tumor was no longer visible on computerized tomography (CT), and she died of pneumonia. The latter patient's tumor secreted the beta chain of human chorionic gonadotropin (HCG). This patient's massive metastatic tumor burden completely regressed as determined by body CT scan and HCG levels after four courses of chemotherapy with bleomycin, vinblastine, and cis-platinum. In 20 patients with lesions of the pineal region, craniotomy was associated with only one death (a patient with metastatic adenocarcinoma). Thus, microsurgery for pineal tumors provides either a reasonably safe potential for complete tumor extirpation and possible cure, or a tissue diagnosis which is necessary for appropriate therapeutic planning for radiotherapy and/or chemotherapy. The traditional therapeutic approach of empiric radiotherapy without a tissue diagnosis for pineal lesions may no longer be warranted.     

Extraneural metastasis of malignant glioma through a ventriculo-peritoneal shunt: growth in peritoneal cavity as ascitic form

This is the first report of extraneural metastasis of malignant glioma through V-P shunt tube and growth in peritoneal cavity as ascitic form. The patient was a 43-year-old man who was admitted to our hospital with occipital headache. CT scan showed enhanced cystic tumor mass at left temporal lobe. Craniotomy and partial excision of the tumor was done and the histology of tumor tissue showed a malignant astrocytoma. Following this treatment, the patient received the adjuvant therapies of radiation, chemotherapy and immunotherapy with interferon, and also recraniotomy three times. In the mean time, a ventriculo-peritoneal shunt was set up for internal hydrocephalus. One month later, abdominal bulging appeared and yellowish ascites could be obtained with peritoneal tap. In the ascite, tumor cells with glial fibrillary acidic protein were observed at the concentration of 5-10 x 10(4) cells/ml. The patient died three months after extraneural metastasis to the abdominal cavity as ascitic form. At autopsy, solid metastatic mass lesion was not found in extraneural region include abdomen.     

Pineal metastasis--a case report and a review of literature

This is a report of a rare case of pineal metastasis from gastric adenocarcinoma. A 75-year-old male with a previous medical history of subtotal gastrectomy for cancer of the cardia was diagnosed as having hydrocephalus 6 months later. After undergoing a V-P shunt operation, he was transferred to Kyoto First Red Cross Hospital. The patient had neurological abnormalities: bilateral paralysis of upward eye movement, and bilateral sluggish reflex to light. Ten days later the patient suddenly fell into a coma due to intratumoral hemorrhage. The patient soon regained consciousness and received Cobalt-therapy. However he died two months later of pulmonary malfunction due to pulmonary metastasis. An autopsy revealed a pineal tumor metastasized from gastric adenocarcinoma. The tumor involved the body of the pineal gland, and was expanded having a sharply defined margin which extended to the posterior portion of the third ventricle and the bilateral basal ganglia.     

Extraneural metastasis of choriocarcinomatous element in pineal germ-cell tumor. Case report

A case of pineal germ-cell tumor producing human chorionic gonadotropin (HCG) and alpha-fetoprotein (AFP) is reported in a 23-year-old man. Extraneural metastasis developed during a course of combined chemotherapy after radiation therapy. Postmortem examination revealed that the metastatic pulmonary tumor was a choriocarcinoma, producing only HCG.     

Pineal metastatic tumor from lung cancer initially caused by neurological abnormalities of pineal body tumor

A rare case is reported of pineal metastasis from lung cancer initially caused by neurological abnormalities of pineal tumor. A 70-year-old female suffering from headache and deterioration of consciousness for 1 week was admitted. She also had a tumor on both sides of her neck. On admission, neurological examination revealed disturbance of upward gaze, and CT scans showed hydrocephalus and pineal tumor. The tumor was seen as a slightly high density mass on non-contrast CT, and was homogeneously enhanced after administration of contrast material. Right V-P shunt and excision of the left neck tumor were performed at the same time. Pathological diagnosis of neck tumor was undifferentiated carcinoma metastasized to cervical lymph nodes. Extensive study was made, by bronchial fiberscope and biopsy, in order to find the origin of the malignancy and disclosed a small cell lung cancer of left lower lobe. The patient took radiation therapy for both the whole brain (60 Gy) and for the bilateral cervical regions (45 Gy). Two courses of chemotherapy using CDDP, ADR, VCR and CY were administered. Both the neck and the pineal tumors were markedly reduced in size at the termination of radiation therapy. However, she was readmitted 3 months later because of dyspnea. Chest X-P revealed enlargement of the left-lung tumor. She died on April 22, 1987. General autopsy disclosed invasive enlargement of left lung cancer, however, no remote metastasis was found. Examination of pineal region showed only necrotic pineal tissue, and no tumor cell was seen in either macroscopic or microscopic study.(ABSTRACT TRUNCATED AT 250 WORDS)     

Intracranial and intraspinal dissemination from pineal yolk sac tumor treated by PVB therapy--case report.

A 15-year-old male was admitted because of diplopia and persistent headache. Postcontrast computed tomography (CT) revealed a homogeneously enhanced large mass, 3 x 4 cm in size, in the pineal region and moderate obstructive hydrocephalus. A right ventriculoperitoneal shunt was installed. At that time, the serum alpha-fetoprotein (AFP) level increased to 23,036 ng/ml, but the level of serum beta-subunit of human chorionic gonadotropin was less than 0.2 ng/ml. These data indicated the tumor to be a pure yolk sac tumor. Following cisplatin-vinblastine-bleomycin (PVB) therapy and whole-brain irradiation (50 Gy), the tumor disappeared on CT, although the AFP level did not return to normal. Eight months after the completion of initial therapy, he had lumbago. Spinal magnetic resonance imaging revealed a metastatic mass at the L5-S2 levels, which was subtotally removed and histologically diagnosed as yolk sac tumor. Postoperative local irradiation (30 Gy) was performed. Seven months after the operation, spinal dissemination at the Th7 level occurred and, 1 month later, intracranial dissemination in the left cerebellopontine angle was detected. He died 25 months after the first admission. PVB therapy did not prevent spinal dissemination in this case.     

Liver abscess secondary to ventriculoperitoneal shunt

Liver abscess is a rare complication following the ventriculoperitoneal (V-P) shunt operation. There has been only one case reported in the literature. We present a case of liver abscess developed about 3 months after V-P shunt operation. A 31-year-old female was admitted to our hospital in comatose condition due to second bleeding from an aneurysm of the right internal carotid artery on January 1, 1984. Obliteration of the aneurysm was performed on the following day. She received V-P shunt operation for the marked hydrocephalus on February 4, but she developed low spinal fluid pressure syndrome. She was able to walk by herself after the replacement of shunt valve on March 4. In the middle of April, she suffered from abdominal pain with a pyrexia for about 5 days. On May 13, a new peritoneal tube was placed in another part of the peritoneal cavity because of the recurrence of hydrocephalus. On the following day, she developed severe abdominal and back pains with a high fever. Abdominal CT scans and ultrasonogram were performed on May 22, showing a well-defined, cystic mass lesion in the liver and the peritoneal tube lying just beneath the mass lesion. Approximately 100 ml of white creamy pus was aspirated from the cystic mass by ultrasound-guided percutaneous puncture, and a 8.3 French pigtail nephrostomy catheter was left in place for 9 days until purulent drainage stopped. Microbiologic examination demonstrated staphylococcus epidermidis in the cerebrospinal fluid (CSF) from the shunt tube but was negative in the abscess fluid. The ventricular fluid was drained externally with the V-P shunt tube for a while, but the new ventricular drainage was instituted because of continuous positive cultures in the CSF from the shunt tube. Thereafter, the cultures of the CSF became negative and ventriculoatrial (V-A) shunt operation was performed on July 2. Postoperative course was uneventful. It is considered that the formation of the liver abscess seems to be caused by the focal injury to the liver surface by the insidiously infected peritoneal tube with St. epidermidis, and by the decrease in systemic resistance to infection. Percutaneous aspiration and drainage under the guidance of abdominal computed tomography or ultrasonography are very useful and efficient for the diagnosis and the treatment of liver abscess. When patients show signs of infection to the V-P shunt, we should remove the shunting system and place a new external ventricular drainage, and institute a V-A shunt after confirming negative cultures of the CSF.     

Central nervous cryptococcosis giving rise to ascites after ventriculo-peritoneal shunting--a case report

The ascites retention as a complication after ventriculo-peritoneal shunting (V-P shunt) is very rare. In this paper, a case of central nervous cryptococcosis giving rise to ascites after a V-P shunt is presented. A 45-year-old female was referred to Prefectural Gifu Hospital complaining of nausea and disturbance of recent memory. She had no remarkable medical history. CT scan films on admission showed enlarged ventricles except for the fourth ventricle, indicating an obstructed hydrocephalus. Lumbar cerebrospinal fluid (CSF) examinations revealed an increase in cell counts (mostly lymphocytes) and protein content, and positive Pandy and Nonne-Apelt reactions. Based on this information, it was tentatively suggested that she had a certain infection in the central nervous system such as mycosis or tuberculosis etc. Continuous ventricular drainage was performed for about three weeks. During this period, several lumbar and ventricular CSF cultures were negative. Therefore, a V-P shunt operation was performed. However, about seven weeks after the V-P shunt, she developed abdominal distention without any peritoneal signs. Abdominal CT scan films showed an abnormal ascites retention. Laboratory tests revealed positive CRP and increased ESR values. Again, it was suggested that she had not only a central nervous but also a peritoneal infection giving rise to ascites. From samples of ventricular CSF and ascites, cryptococcus neoformans was cultured. The lumbar CSF revealed positive latex agglutination titer for cryptococcal antigen, although the culture was negative. The ascites examination revealed an increase in cell counts (mostly lymphocytes) and protein content, and positive Rivalta and Runneberg reactions.(ABSTRACT TRUNCATED AT 250 WORDS)     

Alpha-fetoprotein-producing transitional cell carcinoma of the urinary bladder: a case report

We report a 76-year-old man with alpha-fetoprotein (AFP)-producing transitional cell carcinoma of the bladder. Although the serum level of AFP was 1,428 ng/ml, and he was anti-hepatitis C virus (anti-HCV) antibody-positive, liver tumors were not detected by either a computed tomography (CT) scan or a hepatic angiography. However, removal of a bladder tumor by transurethral resection and subsequent pathological examination revealed a grade III transitional cell carcinoma (TCC). Furthermore, immunohistochemical detection of AFP was diffuse-positive. After the tumor partially responded to concomitant chemoradiotherapy, the serum AFP levels decreased to 966 ng/ml. However, the tumor eventually progressed with multiple lung metastases, and serum AFP levels increased to 3,906 ng/ml. In conclusion, AFP-producing TCC of urinary bladder is rare, and the nature and pathophysiology remains unclear and warrants further investigation.     

Multiple liver metastases of a suprasellar germ cell tumor treated with combined chemotherapy of cisplatin and etoposide

A 23-year-old man was admitted with progressively disturbed vision and easy fatigability. CT scans demonstrated an enhanced mass in the sellar region. Physical and endocrinological examinations revealed atrophy of both optic nerves, temporal field cuts in both eyes, and panhypopituitarism. Concentrations of human chorionic gonadotropin (HCG) in the serum and cerebrospinal fluid were 12 and 33IU/L, respectively. On November 11, 1987, the tumor was partially removed using the transsphenoidal approach. The histological diagnosis was germinoma with syncytiotrophoblastic giant cells. Following postoperative craniospinal irradiation (whole brain, 30Gy; local, 18Gy; spinal canal 28Gy), CT scans showed no residual tumor and the HCG levels decreased until they were undetectable. Eighteen months later, the patient complained of abdominal pain. His serum HCG level had increased to 2,554 IU/L. CT scans of the abdomen revealed multiple low density areas in the liver. Chest X-ray was negative. A Ga scintigram disclosed only liver metastasis. Administration of a chemotherapy was started on June 26, 1989. Cisplatin and etoposide in doses of 20mg and 40mg respectively were given for 5 consecutive days in one course. Following four courses of the combined chemotherapy, the tumor entirely disappeared on CT scans and the HCG level returned to normal. The patient is now able to work well without evidence of recurrence. Multiple liver metastases of an intracranial germ cell tumor had been fatal in previous reports. This may be the first case with liver metastases in which the victim is still alive. The present case indicates that combined chemotherapy with cisplatin and etoposide is effective for extraneural metastases of an intracranial germ cell tumor.     

Bone metastasis associated with shunt-related peritoneal deposits from a pineal germinoma

Summary The case of a 15-year-old boy with a pineal germinoma is reported. The patient first underwent a ventriculoperitoneal (VP) shunt followed by a stereotactic biopsy, then, because of the rapidly deteriorating neurological status, an emergency craniotomy with subtotal removal of the tumour was performed. Two months after surgery, a left femoral metastasis and extensive peritoneal lesions became evident; they were regarded as due to haematogenous and VP shunt spread of the germinoma. At that time, extremely high serum levels of placental alkaline phosphatase were detected. The patient died 6 months after the inital diagnosis. The occurrence of extraneural metastases as well as of shunt related peritoneal deposits from primary intracranial germinoma is discussed. As far as we know this is the first reported case of a combination of haematogenous as well as VP shunt spread of a pineal germinoma.     

A case of disproportionately large communicating fourth ventricle (DLCFV) combined with syringomyelia and Chiari malformation

We report a rare case of disproportionately large communicating fourth ventricle (DLCFV) combined with syringomyelia and Chiari malformation. The case was a 27-year-old male who underwent ventriculoperitoneal (V-P) shunt on the right side for hydrocephalus caused by traumatic intracerebral and intraventricular hemorrhage. One month later, he became somnolent with posterior fossa symptoms (nausea, vomiting and nystagmus). CT scan demonstrated enlarged fourth ventricle, which was diagnosed as DLCFV because the ventriculogram revealed patency of the aqueduct. One and half month later a second V-P shunt was made on the left side to increase the shunt flow. He became ambulatory with a cane, although the fourth ventricle remained moderately dilated on CT scan. Two months after the additional V-P shunt, he slipped and hit the occiput and immediately became tetraparetic. The patient was treated conservatively under the diagnosis of central spinal cord injury. The MRI taken 2 months after the accident revealed Chiari malformation (type 1), syringomyelia and a dilated fourth ventricle which was compressing the brainstem. After the fourth ventriculoperitoneal (FV-P) shunt, the tetraparesis transiently improved but then again worsened. On the CT scan the syrinx did non change in size, while the size of the fourth ventricle became normal. After syringoperitoneal (S-P) shunt the patient showed a moderate improvement of tetraparesis. Unfortunately he suffered appendicitis complicated with peritonitis and all the shunts were immediately changed to external drainage. However, the patient developed meningitis and became paraplegic. The motor function of the upper extremities slightly improved by aspiration of fluid via the external drainage system from the syrinx.(ABSTRACT TRUNCATED AT 250 WORDS)     

Detection of AFP and HCG in metastatic testicular cancer after treatment with chemotherapy or radiation therapy

We used an indirect immunoperoxidase technique to detect alpha-fetoprotein (AFP) and human chorionic gonadotropin (HCG) in tissue sections of nine metastatic germ cell tumors excised after treatment with chemotherapy or radiation therapy, and correlated the results with the serum levels of AFP and HCG. In all but 1 case yolk sac tumor (YST) was the only histologic type that reacted for AFP (AFP+) and syncytiotrophoblasts (STB) were the only histologic type that reacted for HCG (HCG+). Among 5 cases with normalization of the serum AFP before surgery, 3 were associated with YST-/AFP-, 1 with YST+/AFP+, and 1 with YST+/AFP- metastases; and among 4 cases with normalization of the serum HCG all were associated with STB-/HCG- metastases. Among 3 cases with persistent elevation of the serum AFP, 1 was associated with YST+/AFP+, 1 with YST+/AFP-, and 1 with YST-/AFP- metastases; and of 2 cases with persistent elevation of the serum HCG, 1 was associated with STB-/HCG- and 1 with STB+/HCG+ metastases. These data suggest that marker normalization in the face of persistent tumor results primarily from eradication of YST and STB, but also from treatment-induced inhibition of AFP and HCG synthesis or secretion.     

Case of hydrocephalus associated with vestibular schwannoma, treated by tumor removal

A 57-year-old male presented with gait disturbance, urinary incontinence, dementia resembling symptom of normal pressure hydrocephalus (NPH) and was admitted to our institusion. A computerized tomographic scan (CT) and magnetic resonance imaging (MRI) revealed hydrocephalus as well as a tumor in the right cerebellopontine angle. We diagnosed communicating-type hydrocephalus associated with the vestibular schwannoma and tumor removal was performed prior to ventriculo-peritoneal (V-P) shunt. After surgery, symptoms of the patient disappeared promptly and cerebrospinal fluid protein elevation was normalized. One month after surgery, CT and MRI revealed reduction of the ventricle size and V-P shunt became unnecessary. Hydrocephalus is occasionally complicated by vestibular schwannoma. The majority results from obstruction of the cerebrospinal fluid (CSF) pathway by large tumors. But, small tumors also cause communicating-type hydrocephalus. Although the selection of the treatment is controversial, tumor removal without a shunt is considered as a good strategy for management of hydrocephalus associated with vestibular schwannoma.     

Immunohistochemical study of placental alkaline phosphatase in primary intracranial germ-cell tumors

Indirect immunoperoxidase staining by the peroxidase-antiperoxidase (PAP) technique was carried out on 23 human primary intracranial germ-cell tumors (17 germinomas, one embryonal carcinoma, one yolk-sac tumor, three teratomas, and one teratoma with embryonal carcinoma) and on six human primary pineal non-germ-cell tumors (one pineocytoma, two pineoblastomas, two astrocytomas, and one glioblastoma multiforme). The technique used specific rabbit antisera against placental alkaline phosphatase (PLAP), alpha-fetoprotein (AFP), and human chorionic gonadotropin (HCG). Thirteen of 17 intracranial germinomas (76.5%) showed positive staining for PLAP mainly on the tumor cell membrane. In six primary intracranial non-seminomatous germ-cell tumors, there was weak positive staining indicating the presence of PLAP in only a few cells of one embryonal carcinoma, and in some glandular epithelial cells of one teratoma; this staining was limited to the cytoplasm. None of the other six primary pineal non-germ-cell tumors showed any positive PLAP reaction. From these results, PLAP was shown to be very useful in histopathology as a diagnostic tumor marker of intracranial germinoma. Positive AFP staining was seen in several yolk-sac tumor cells and a few embryonal carcinoma cells. However, no intracranial germinomas and non-germ-cell tumors of the pineal region showed positive reaction. As for HCG, only one suprasellar germinoma and one pineal embryonal carcinoma among 29 specimens contained a few positive-staining tumor cells.     

Pure yolk sac tumor of the testis with brain metastasis: report of an adult case]

Herein we report an adult case of pure yolk sac tumor with brain metastasis. The patient was a 37-year-old male who presented with indulation of his left scrotum for 10 months. The plain computerized tomographic (CT) scan on entry demonstrated tumor metastasis to his lung and liver and serum alpha-fetoprotein (AFP) level was 786 ng/ml. Five days after admission, he developed hemiplegia secondary to the cerebral metastasis and hemorrhage. After chemotherapy and operation of right-posterior lobectomy, PVB (cisplatinum, vinblastine, bleomycin) chemotherapy produced a complete remission and the elevated serum AFP was normalized. However, the second course of chemotherapy had to be discontinued because of drug-induced hepatitis. He died of massive tumor metastasis to his brain 6 months after craniotomy.     

An immunohistochemical study of intracranial germ cell tumours

Summary Histologically verified intracranial tumours, mainly germ cell tumours of the pineal and suprasellar regions, were studied immunohistochemically using anti-serum of alpha fetoprotein (AFP), human chorionic gonadotropin (HCG), carcinoembryonic antigen (CEA), human placental lactogen (HPL), pregnancy specific -1 glycoprotein (SP-1), glial fibrillary acidic protein (GFAP), S-100 and neuron specific enolase (NSE). In germinomas, HCG positive cells were occasionally demonstrated in cells presenting as syncytiotrophoblastic giant cells (STGC), and GFAP and S-100 positive cells were found in the surrounding gliotic lesions. Teratomas were positive for CEA in their epithelial components. Endodermal sinus tumours were positive for AFP, choriocarcinomas for HCG and SP-1, and embryonal carcinomas for AFP, HCG and SP-1. HCG and SP-1 positive cells were demonstrated in STGC. As for the relationship between serum AFP level and tissue localization, many cases presenting a serum AFP level exceeding 220 ng/ml were positive for AFP in tumour tissue.     

原发性肝癌肝外转移癌六例再切除体会

目的探讨原发性肝癌肝外转移癌再切除的应用和价值。方法回顾性分析1986年至2008年我院27例原发性肝癌再切除中的6例肝外转移癌的临床治疗资料，其中肺部转移癌2例，腹腔转移癌4例。结果6例肝外转移癌切除均无手术死亡及术后并发症发生，术后中位生存期从再次切除算起4．6年。结论原发性肝癌肝外转移癌的高选择性再切除是安全有效的，可明显延长生存期。腹腔、肺部癌结节选择再切除或伽玛刀治疗，腹腔种植癌和浸润癌灶行切除术，可明显延长生存期，是进一步提高疗效的重要途径。