Lung carcinoma metastasis presenting as a pineal region tumor

The pineal region is an unusual site for brain metastasis and most metastatic pineal lesions are asymptomatic. A 53 year-old man presented with severe headache, limitation of upward gaze and diplopia. The patient's neurological examination was unremarkable. Magnetic resonance imaging (MRI) scans of the brain demonstrated a 1.5 x 2 cm well demarcated solitary mass in the pineal region with hydrocephalus. Surgery was performed and adenocarcinoma was diagnosed. A systemic investigation revealed adenocarcinoma of the lung as primary lesion. Although rare, metastatic tumor should be considered in the differential diagnosis of pineal region tumors.     

Pineal metastasis as first clinical manifestation of colorectal adenocarcinoma. Case report.

Cerebral metastases from colorectal adenocarcinomas occur in 8% of the cases. Diagnosis is usually made when primary disease and widespread metastases are already known. We report the case of a patient with single metastases in the pineal region as the first clinical manifestation of a colorectal adenocarcinoma. A 48-year-old female with Parinaud's syndrome for 15 days prior her admission was evaluated in our clinic. She had no symptoms or signs of colorectal disease. MRI examination revealed an heterogeneous lesion with peritumoral gadolinium enhancement, located in the pineal region. The tumor was radically resected through an infratentorial/supracerebellar approach. Histology showed metastatic carcinoma and immunohistochemical examination showed gastrointestinal tract adenocarcinoma. Metastases to the pineal region are extremely rare and should be considered in the differential diagnosis of tumors of this region.     

Neuroendocrine carcinoma of the pineal parenchyma. The first reported case

We report a case of a 35-year-old female patient who presented with worsening headaches, vertigo, and vision changes. MRI of the brain showed an enhancing lesion in the pineal region. The patient was taken for resection of the lesion which was classified as neuroendocrine carcinoma of the pineal parenchyma, intermediate grade. Histologically, the neoplasm was cellular, mitotically active, and composed of tightly packed cells with high nuclear cytoplasmic ratio, scant cytoplasm, and ill-defined cell borders. Immunohistochemically the tumor cells were positive for chromogranin, synaptophysin and AE1/AE3, and negative for CK-7, CK-20, and TTF1. Possible metastasis from any other primary sites was ruled out clinically. This represents the first reported case of neuroendocrine carcinoma of the pineal parenchyma.     

Ventriculostomía y biopsia endoscópica de una masa pineal con hidrocefalia y diagnóstico final inesperado

A 72-year-old female with no relevant medical history consulted in the emergency room for a two-week history of headache, vomiting and gait disturbance. Head CT scan revealed obstructive hydrocephalus secondary to a pineal mass with compression of the third ventricle. Magnetic Resonance showed another mass located in the right Lushcka foramen. Endoscopic third-ventriculostomy and biopsy of pineal mass were performed. Pathological analysis was consistent with metastasis of carcinoma. Full-body CT scan showed a lung mass related to primary carcinoma. The patient received systemic treatment for metastatic lung cancer. She died two months after diagnosis.     

Treatment of germ cell tumors in the pineal region

The authors retrospectively analyzed 107 patients with primary intracranial germ cell tumor (GCT), who were treated at the Department of Neurosurgery, Yonsei Medical Center between January 1986 and January 1996. The incidence of GCT was 2.8% in pediatric patients with intracranial tumor. Of the 107 tumors, 60 were located in the pineal region, 30 in the suprasellar region, 16 in basal ganglia or the thalamic region, and 1 in the posterior fossa. The 60 pineal GCT consisted of 39 germinomas (29 pure germinomas, 6 germinomas with STGC, 4 germinomas mixed with teratoma), 5 mature teratomas, and 16 nongerminomatous GCT. Thirty patients underwent surgery: their operations took the form of total resection in 14 cases, subtotal resection in 10, and biopsy in 6. Thirty patients (27 with germinomas, 3 with endodermal sinus tumors) were managed without surgery on the basis of radiological findings and tumor markers. The 5-year survival was 91% for 39 patients with germinomas, 80% for 5 with mature teratomas, and 49% for 16 with nongerminomatous GCT. Univariate analysis of prognostic factors with the Kaplan-Meier survival curve showed that histological tumor type, radiological findings, results of tumor marker studies, and response to trial radiation or chemotherapy were highly correlated with outcome. Chemotherapy was beneficial as the method of trial treatment in pineal GCT and treatment in recurrent tumors. The administration of trial chemotherapy or radiotherapy without tissue biopsy is well justified as a treatment modality in pineal GCT suspected on the basis of radiological findings and tumor marker studies. Aggressive multimodality approaches with surgery, radiotherapy, and chemotherapy are necessary to improve the outcome in these tumors. We propose new protocol for treatment of germ cell tumors in the pineal region, which is based on a minimally invasive approach.     

松果体区肿瘤致男性性早熟8例临床分析

目的探讨男性松果体区肿瘤患儿性早熟的原因和临床特征。方法对8例有性早熟表现的男性松果体区肿瘤患儿的临床资料进行回顾性分析,并在肿瘤病理和生殖细胞肿瘤标志物方面与11例无性早熟表现的患儿进行比较。结果本组8例性早熟患儿占同时期、同年龄段男性松果体区肿瘤患儿的42%。3例性早熟为首发表现,5例为临床表现之一。性早熟多早于其它症状出现,但常被忽视。性早熟患儿的松果体区肿瘤均为恶性生殖细胞肿瘤,且均能分泌β-人绒毛膜促性腺激素（β-HCG）。结论男性松果体区肿瘤患儿的性早熟是假性性早熟,肿瘤分泌HCG导致血清HCG异常增高是性早熟的主要原因。     

Parinaud syndrome due to solitary pineal metastasis

Parinaud syndrome due to metastatic lesion of the pineal gland has been reported rarely in the literature. We present a case of Parinaud syndrome with solitary pineal metastasis of an oat cell carcinoma.     

原发性松果体区胚胎癌的诊断与治疗(附二例报告及文献复习)

目的 原发性松果体区胚胎癌非常罕见,本文报道2例,并结合文献探讨其诊断、治疗和预后.方法 2例经病理证实的原发性松果体区胚胎癌,术前例1患者血清甲胎蛋白(AFP)6 810μg/L,人绒毛膜促性腺激素(β-HCG)正常,例2患者血清AFP正常,β-HCG 5 260 mIU/ml.采用枕部经小脑幕入路显微手术切除肿瘤,术后联合放化疗.结果 2例患者肿瘤均达到镜下全切,术后肿瘤免疫组化示例1:CK、AFP阳性;例2:CK、β-HCG阳性.经放化疗后,血清AFP/β-HCG基本正常.术后1年随访2例患者仍然存活.结论 血清AFP、β-HCG对胚胎癌的诊断及预后判断有重要作用,免疫组化指标CK、AFP、β-HCG对病理诊断有鉴别意义,该病采用手术联合放化疗的综合疗法,可获得较好疗效.

Abstract：

Objective Primary embryonal carcinoma in pineal region is extremely rare Here we report two such cases and combined literatures review to discuss its diagnosis, treatment and prognosis. Method Two primary embryonal carcinoma in pineal region verified histologically were presented. In one cases, the preoperative serum alpha - fetoprotein (AFP) level reached significantly high level, 6 810μg/L, but showed negative staining forβ-human chorionic gonadotropin (β- HCG) . In the other one, accompanied by elevation of β-HCG level, 5 260 mIU/ml, the serum AFP was negative. The tumors were microsurgically removed by transeccipito-tentorial approach. Endoscopic third ventriculostomy was used after tumor removal because of recurrent hydrocephalus. Both patients received adjunctive treatments included radiation therapy (whole central nervous system 30 Gy and tumor bed 50 Gy) and 4 course of chemotherapy. Results In both patients, the tumors were totally removed. Postoperative tumor immunohistochemistry indicators were present in case 1 with positive reaction for cytokeratin(CK) and AFP, in case 2 with positive CK and β - HCG. After adjunctive treatments, the serum AFP and β - HCG of two patiants were decreased to normal ranges. After post - operative follow - up of one year,two patients still survived and the serum markers were almost normal. Conclusions Measurement of serum β-HCG and AFP is extraordinarily significant for the diagnosis and post - treatment monitoring of pineal region embryonal carcinoma. Pathological differential diagnosis requires immunohistochemical indicators including cytokeratin (CK),AFP andβ- HCG. With radically surgical removal and combined adjunctive therapy including radiation and chemotherapy, good consequence could be obtained.     

Metastatic germ cell testicular tumor of the eye and brain: case report

The aim of this report is to contribute to the clinical understanding of this rare combined pathology. Intraocular metastatic tumors are rarely encountered pathologies. Mostly encountered primary sites are breast, prostate, kidney, lung and skin. Testis as a primary site for orbital metastases is rather rare. Metastasis to orbit usually occurs to periorbital structures, but to vitreus is relatively rare. Here we describe a case of intraocular metastatic tumor to the vitreus originating from testicular embryonal cell carcinoma. Patient with intraocular tumor had also intracranial metastasis. He was operated on for intracranial lesion, after an uneventfull postoperative period he was discharged. 2 months later when he died, postmortem examination of the intraocular lesion reported as metastatic embryonal cell carcinoma. As a conclusion testicular embryonal cell carcinoma, eventhough rare, must be considered to metastasize to the eye.     

Multiple germinomas due to early intracranial metastasis.

A case with multiple intracranial germinomas involving basal ganglia and corpus callosum is presented. Clinical and sequential neuroradiological studies demonstrated the entire course of multiple tumour development. These findings suggested that the multiple tumours were intracranial metastatic deposits from primary germinoma in the pineal body. A common primary pineal or suprasellar lesion should be carefully ruled out in the cases of germ cell tumours occurring in unusual regions.     

Bifocal germinomas in the pineal region and hypothalamo-neurohypophyseal axis: Primary or metastasis?

Whether bifocal germinomas (BFGs) synchronously presenting within the pineal region and the hypothalamo-neurohypophyseal axis (HNA) are primary germinomas of dual-origin remains to be elucidated. We analyzed MRI images and clinical features of 95 neurohypophyseal germinomas and 21 BFG patients and developed a tentative definition of the BFGs. We found dual-primary BFGs (true BFGs) do exist. The fundamental difference between primary and metastatic HNA germinomas was the direction of tumor growth. For a true BFG, the primary HNA tumor grew from the neurohypophysis toward the hypothalamus and almost invaded the whole pituitary stalk. For a false BFG (primary pineal germinoma with HNA metastasis), the metastatic HNA tumor first appeared at the third ventricular floor (TVF), grew toward the neurohypophysis, but commonly did not invade the inferior pituitary stalk. Compared to false BFGs, true BFGs commonly had diabetes insipidus as the first symptom, dysfunction of the anterior pituitary, no high-intensity MRI signal at the posterior pituitary, a larger extension of the HNA tumor, and fewer numbers of remote lesions from cerebrospinal fluid seeding. Accordingly, 12.8% (12/96) of our germinoma patients had true BFGs, and of these, 58.3% (7/12) were free of remote metastases and warranted treatment with limited radiotherapy. True BFGs with remote metastases and all false BFGs should be treated with craniospinal irradiation. We provided evidence for the diagnosis of true BFGs that is useful for radiotherapy strategy, suggesting that the existence of metastasis to other locations is not a diagnostic criterion for a true BFG.     

松果体区肿瘤的显微手术及内镜辅助作用

目的 探讨显微手术治疗松果体区肿瘤的手术入路和技术以及内镜的辅助作用.方法 采用显微手术技术通过幕下小脑上入路切除10例松果体区肿瘤,其中内镜辅助手术5例;病理结果：生殖细胞瘤5例、混合性生殖细胞肿瘤3例（生殖细胞瘤合并恶性畸胎瘤1例,合并胚胎癌2例）、脑膜瘤和成熟畸胎瘤各1例;1例术后脑积水行第三脑室底造瘘术,1例术前行第三脑室底造瘘.结果 所有病例全切肿瘤.术后早期结果全部良好,随访1个月～7年良好8例,死亡2例.结论 松果体区肿瘤手术难度较大,但选择适合的手术入路和熟练的显微手术技术加上内镜的辅助可以取得满意的疗效.     

Histopathological patterns of papillary tumour of the pineal region

Papillary tumour of the pineal region (PTPR) is a rare neoplasm that has been formally included in the 2007 WHO classification of central nervous system tumours. The critical diagnosis of this neoplasm is often difficult because of its similarity to other primary or secondary papillary lesions of the pineal region, including parenchymal pineal tumours, papillary ependymoma, papillary meningioma, choroid plexus papilloma and metastatic papillary carcinoma. We present the variability of the histopathological pattern in three cases of PTPR. All cases showed predominant epithelial-like morphology but with various degrees of papillary formation and intensity of cellular pleomorphism. One tumour was highly cystic and exhibited cellular sheets containing vessels covered by several layers of uniform columnar to cuboidal tumour cells. The second tumour showed distinct papillae covered by layers of polymorphous cells with atypical, often hyperchromatic nuclei. Numerous cells displayed foamy, eosinophilic or clear, sometimes vacuolated cytoplasm. The third case consisted of solid cellular areas composed of pseudostratified columnar cells, most often arranged in perivascular pseudorosette formations. The cells lining papillary structures exhibited marked polymorphism with atypical, often plump nuclei. Mitotic figures were rare and areas of necrosis were observed only in one case. Immunohistochemical staining showed diffuse immunoreactivity for neuron-specific enolase, S-100 protein, cyto-keratin and vimentin. Focal reaction for synaptophysin and chromogranin A and epithelial membrane antigen (EMA) were observed. The tumours lacked expression of GFAP. The Ki-67 labelling index was relatively low but its focal increase was noted in two cases. The final diagnosis of PTPR was based on both predominant papillary morphology and immunohistochemical results. PTPR should be considered in diagnosis of pineal tumours but their natural history, therapeutic strategy and prognosis remain controversial.     

Primary Malignant Melanoma in the Pineal Region

A 59-year-old male patient had 5-month history of gait disturbance and memory impairment. His initial brain computed tomography scan showed 3.5×2.8 cm sized mass with high density in the pineal region. The tumor was hypointense on T2 weighted magnetic resonance images and hyperintense on T1 weighted magnetic resonance images with heterogenous enhancement of central portion. The tumor was totally removed via the occipital transtentorial approach. Black mass was observed in the operation field, and after surgery, histopathological examination confirmed the diagnosis of malignant melanoma. Whole spine magnetic resonance images and whole body 18-fluoro-deoxyglucose positron emission tomography could not demonstrate the primary site of this melanoma. Scrupulous physical examination of his skin and mucosa was done and dark pigmented lesion on his left leg was found, but additional studies including magnetic resonance images and skin biopsy showed negative finding. As a result, final diagnosis of primary pineal malignant melanoma was made. He underwent treatment with the whole brain radiotherapy and extended local boost irradiation without chemotherapy. His preoperative symptoms were disappeared, and no other specific neurological deficits were founded. His follow-up image studies showed no recurrence or distant metastasis until 26 weeks after surgery. Primary pineal malignant melanomas are extremely rare intracranial tumors, and only 17 cases have been reported since 1899. The most recent case report showed favorable outcome by subtotal tumor resection followed by whole brain and extended local irradiation without chemotherapy. Our case is another result to prove that total tumor resection with radiotherapy can be the current optimal treatment for primary malignant melanoma in the pineal region.     

Metastatic gastric adenocarcinoma to the pineal body a case report

Summary The case of metastatic gastric adenocarcinoma to the pineal body reported herein is believed to be the first case of this kind reported in the literature. It appears also to be the first unequivocal case in which a metastatic pineal tumor gave rise to clinical signs of a primary pineal tumor.

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Zusammenfassung Es wird ein Fall von Magencarcinommetastase in der Zirbeldrüse beschrieben; es ist anzunehmen, daß dies der erste bisher bekannt gewordene Fall dieser Art ist. Von besonderem Interesse erscheint die Tatsache, daß die Metastase die klinischen Symptome eines Pinealoms hervorgerufen hatte, was bisher ebenfalls noch nicht mit Sicherheit beobachtet wurde.

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With 2 Figures in the Text     

Spinal metastasis from renal cell carcinoma, 31 years following nephrectomy--case report

Most renal cell carcinomas recur or metastasize within 2 years. We present a 62-year-old lady with metastatic renal cell carcinoma in the spine, 31 years following nephrectomy of the primary tumor. This is the longest interval between diagnosis of primary renal cell carcinoma and metastasis reported in the literature.     

松果体区肿瘤的临床病理研究

目的 研究松果体区肿瘤(PRTs)的病理分布特点及不同病理类型的临床特点.方法 回顾性分析南方医院神经外科自2000年1月至2009年1月经手术治疗并取得完整病理资料的133例PRTs患者的临床资料及病理特点.结果 133例患者中生殖细胞肿瘤61例(45.9%),松果体实质肿瘤17例(12.8%),神经上皮肿瘤28例(21.1%),其他肿瘤27例(20.2%).生殖细胞肿瘤中男女比例为14.25:1,平均15.3岁.松果体实质肿瘤中男女比例为2.4:1,平均24.7岁.神经上皮肿瘤中男女比例为1.15:1,平均28.1岁.有33例血清免疫学检查异常,除1例为转移瘤外,其余均为生殖细胞肿瘤.结论 PRTs病理类型多样,以生殖细胞肿瘤为主;影像学检查、血清免疫学检查及活检均不能准确判断病理类型,积极手术获得完整病理标本对于PRTs的病理研究有重要意义.     

松果体区肿瘤的临床病理研究

目的 研究松果体区肿瘤(PRTs)的病理分布特点及不同病理类型的临床特点.方法 回顾性分析南方医院神经外科自2000年1月至2009年1月经手术治疗并取得完整病理资料的133例PRTs患者的临床资料及病理特点.结果 133例患者中生殖细胞肿瘤61例(45.9%),松果体实质肿瘤17例(12.8%),神经上皮肿瘤28例(21.1%),其他肿瘤27例(20.2%).生殖细胞肿瘤中男女比例为14.25:1,平均15.3岁.松果体实质肿瘤中男女比例为2.4:1,平均24.7岁.神经上皮肿瘤中男女比例为1.15:1,平均28.1岁.有33例血清免疫学检查异常,除1例为转移瘤外,其余均为生殖细胞肿瘤.结论 PRTs病理类型多样,以生殖细胞肿瘤为主;影像学检查、血清免疫学检查及活检均不能准确判断病理类型,积极手术获得完整病理标本对于PRTs的病理研究有重要意义.     

松果体区肿瘤的临床病理研究

目的 研究松果体区肿瘤(PRTs)的病理分布特点及不同病理类型的临床特点.方法 回顾性分析南方医院神经外科自2000年1月至2009年1月经手术治疗并取得完整病理资料的133例PRTs患者的临床资料及病理特点.结果 133例患者中生殖细胞肿瘤61例(45.9%),松果体实质肿瘤17例(12.8%),神经上皮肿瘤28例(21.1%),其他肿瘤27例(20.2%).生殖细胞肿瘤中男女比例为14.25:1,平均15.3岁.松果体实质肿瘤中男女比例为2.4:1,平均24.7岁.神经上皮肿瘤中男女比例为1.15:1,平均28.1岁.有33例血清免疫学检查异常,除1例为转移瘤外,其余均为生殖细胞肿瘤.结论 PRTs病理类型多样,以生殖细胞肿瘤为主;影像学检查、血清免疫学检查及活检均不能准确判断病理类型,积极手术获得完整病理标本对于PRTs的病理研究有重要意义.