====== Pineal Germinoma Case Reports ====== A case of bifocal pineal and suprasellar germinoma with posterior fossa metastases in a 15-year-old male patient. The involvement of the third ventricular floor and nonthickened inferior pituitary stalk of the suprasellar lesion suggest that it is a metastasis of a primary pineal lesion rather than a dual-primary. This distinction, with the presence of posterior fossa metastases, favors the use of more aggressive treatment with combination radiation therapy and chemotherapy for a better outcome ((Kumar V, Mahdi E, Moore N, Vorona G, Mishra C, Jones K, Urbine J. Bifocal pineal and suprasellar germinomas with posterior fossa metastases in an adolescent patient. Radiol Case Rep. 2022 Sep 27;17(12):4537-4541. doi: 10.1016/j.radcr.2022.08.102. PMID: 36189158; PMCID: PMC9520090.)). ---- A 23-year-old adult male with no underlying pathologies, drug allergies, or family history of cancer, presented for medical evaluation with blurred vision, diplopia, forgetfulness, and weight loss starting 3-4 months before the evaluation. Clinical examination indicated [[Parinaud's Syndrome]]. [[Magnetic resonance imaging]] (MRI) and computed tomography (CT) revealed a [[pineal tumor]] with ependymal dissemination in both [[lateral ventricle]]s, which was causing [[obstructive hydrocephalus]]. The patient had surgery consisting of [[ventriculostomy]], Holter shunt insertion, cisternal ventricular intubation, and cisterna magna anastomosis to improve ventricular drainage. Pathology confirmed pineal germinoma. Cerebrospinal fluid cytology and MRI of the axis were negative. Four cycles of NC were given to the patient (carboplatin, etoposide, and ifosfamide), with a reduced dosage. Once a partial volumetric response was confirmed, whole-ventricular radiotherapy (WVR) was initiated with a total tumor bed dose of 45 Gy over 25 sessions in 5 weeks. Optimum clinical results were observed, and no short-term (<90 days) radiation toxicity was observed. The patient was able to resume his normal activities soon after treatment. Follow-ups over 2 years post-surgery indicated continued control of the lesion and absence of symptoms except for mild diplopia. Although this is a [[case report]], these data suggest that a reduced [[neoadjuvant chemotherapy]] course and whole-ventricular [[radiotherapy]] may effectively treat adult [[germinoma]]. This protocol likely decreases the risk of undesirable NC and RT secondary effects while providing excellent local control; however, using a narrower RT field is not recommended ((Fernández-Rodríguez LJ, Maldonado-Pijoan X. Pineal germinoma in a young adult: A case report. Cancer Rep (Hoboken). 2022 Mar 28:e1611. doi: 10.1002/cnr2.1611. Epub ahead of print. PMID: 35347869.)). ===2015=== A clinical case of a 19-year-old male patient who developed [[pure word deafness]] due to the local compressive effect of a pineal germinoma on the [[inferior colliculus]] of the [[quadrigeminal plate]]. After percutaneous radiation therapy the size of the tumor decreased significantly, while audiometry demonstrated a complete regression of the auditory deficit. Since pure word deafness is commonly attributed to temporal lesions, the inferior colliculi represent an exceptional site for these symptoms ((Joswig H, Schönenberger U, Brügge D, Richter H, Surbeck W. Reversible pure word deafness due to inferior colliculi compression by a pineal germinoma in a young adult. Clin Neurol Neurosurg. 2015 Sep 9;139:62-65. doi: 10.1016/j.clineuro.2015.08.034. [Epub ahead of print] PubMed PMID: 26372938.)). ===2014=== Interferon-γ receptor 1 (IFN-γR1) deficiency is one of the primary immunodeficiencies conferring Mendelian Susceptibility to Mycobacterial Disease (MSMD). Some cases of neoplasms have been recently reported in patients with MSMD, underlying the already known link between immunodeficiency and carcinogenesis. We report the first case of intracranial tumour, i.e. pineal germinoma, in a 11-year-old patient with complete IFN-γR1 deficiency. The first clinical presentation of the genetic immunodeficiency dates back to when the child was aged 2 y and 10 mo, when he presented a multi-focal osteomyelitis caused by Mycobacterium scrofulaceum. The diagnosis of IFN-γR1 deficiency (523delT/523delT in IFNGR1 gene) was subsequently made. The child responded to antibiotic therapy and remained in stable clinical condition until the age of 11 years, when he started complaining of frontal, chronic headache. MRI revealed a solid pineal region mass lesion measuring 20 × 29 × 36 mm. Histological findings revealed a diagnosis of pineal germinoma. The patient received chemotherapy followed by local whole ventricular irradiation with boost on pineal site, experiencing complete remission, and to date he is tumor-free at four years follow-up. Four other cases of tumors have been reported in patients affected by MSMD in our knowledge: a case of Kaposi sarcoma, a case of B-cell lymphoma, a case of cutaneous squamous cell carcinoma and a case of oesophageal squamous cell carcinoma. In conclusion, in patients with MSMD, not only the surveillance of infectious diseases, but also that of tumors is important ((Taramasso L, Boisson-Dupuis S, Garrè ML, Bondi E, Cama A, Nozza P, Morana G, Casanova JL, Marazzi MG. Pineal germinoma in a child with interferon-γ receptor 1 deficiency. case report and literature review. J Clin Immunol. 2014 Nov;34(8):922-7. doi: 10.1007/s10875-014-0098-0. Epub 2014 Sep 14. PubMed PMID: 25216720. )). ===2013=== A 37-year-old gentleman presented to urology after previously being treated for a pineal germinoma with steroids and radiotherapy. On routine followup, he described symptoms of a [[testicular seminoma]]. This was managed surgically with radical orchidectomy ((Bhatty UN, Ashrafi MH, Nicholson CM, Haq A. A rare case of a pure testicular seminoma presenting 7 years after a pineal germinoma. Case Rep Urol. 2013;2013:490638. doi: 10.1155/2013/490638. Epub 2013 Oct 10. PubMed PMID: 24286014; PubMed Central PMCID: PMC3810517. )). ---- A previously healthy man underwent endoscopic biopsy for a suspected pineal germinoma. Histology and immunohistochemistry did not confirm the preoperative diagnosis, and neurosarcoidosis was hypothesised because of the presence of granulomatous reaction. The patient remained in good health for 3 years and was still asymptomatic when a control MRI showed metastasis implantation along the endoscopic route. A redobiopsy provided the diagnosis of germinoma, but was complicated by severe ventricular haemorrhage requiring emergency clot excision. Postoperative clinical conditions were so severe that the treatment of germinoma was postponed. Three months later, repeated MRI showed tumour progression. Chemotherapy promoted good tumour regression so that the treatment was completed by radiation therapy. The tumour completely disappeared on MRI, but the patient remained severely disabled because of the haemorrhage ((Talamonti G, Ligarotti GK, Bramerio M, Imbesi F. Unusual behaviour of a pineal germinoma mimicking neurosarcoidosis and metastasising along the endoscopic route. BMJ Case Rep. 2013 Aug 2;2013. pii: bcr2013200278. doi: 10.1136/bcr-2013-200278. PubMed PMID: 23912659; PubMed Central PMCID: PMC3762524.)). ===2011=== A 13-year-old patient presented with massive intra-abdominal metastasis and spontaneous acute tumour lysis syndrome, 17-months after VP shunt placement for metastatic pineal germinoma treated with cranio-spinal-irradiation. Hyperhydration/rasburicase improved renal function, allowing chemotherapy with subsequent surgery. The patient remains event-free 34-months later. Risk of intra-abdominal metastasis from VP shunts is discussed ((Murray MJ, Metayer LE, Mallucci CL, Hale JP, Nicholson JC, Kirollos RW, Burke GA. Intra-abdominal metastasis of an intracranial germinoma via ventriculo-peritoneal shunt in a 13-year-old female. Br J Neurosurg. 2011 Dec;25(6):747-9. doi: 10.3109/02688697.2011.566383. Epub 2011 Apr 18. PubMed PMID: 21501064. )). ===2010=== A very unique case of pineal germinoma diagnosed in an adult by [[cerebrospinal fluid cytology]]. They analyzed case reports, literature reviews, and therapy and diagnostic articles about pineal germinoma in the English literature from 1983 to 2009 through the national library of medicine, Pubmed, and OVID search engines. They used key words "pineal germinoma," "pineal gland tumor," "CNS germinoma," and "extragonodal germinomas" ((Srinivasan N, Pakala A, Mukkamalla C, Oswal A. Pineal germinoma. South Med J. 2010 Oct;103(10):1031-7. doi: 10.1097/SMJ.0b013e3181ebeeff. Review. PubMed PMID: 20818311. )). ===2002=== Pineal germ cell tumor metastasis via ventriculoperitoneal shunt ((Altundağ OO, Celik I, Kars A. Pineal germ cell tumor metastasis via ventriculoperitoneal shunt. Am J Clin Oncol. 2002 Feb;25(1):104-5. PubMed PMID: 11823710. )). ===1999=== 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 ((Uchino M, Nemoto M, Ohtsuka T, Seiki Y, Shibata I. [Extraneural metastasis of pineal germinoma through a ventriculoperitoneal shunt, following histological change]. No Shinkei Geka. 1999 Mar;27(3):269-74. Review. Japanese. PubMed PMID: 10190163. )). ===1993=== Peritoneal implantation of pineal germinoma via a ventriculoperitoneal shunt ((Blasco A, Domínguez P, Ballestín C, Argüelles M, de Agustín P. Peritoneal implantation of pineal germinoma via a ventriculoperitoneal shunt. Acta Cytol. 1993 Jul-Aug;37(4):637-8. PubMed PMID: 8328255. )). ===1991=== An 18-year-old male was admitted with headache, nausea, and vomiting. Computed tomography (CT) revealed an enhanced tumor of the pineal region and hydrocephalus. The tumor was partially resected via a parieto-occipital craniectomy. The histological diagnosis was germinoma. No serum tumor markers such as alpha-fetoprotein (AFP) and human chorionic gonadotropin (HCG) were detectable. A ventriculo-peritoneal (V-P) shunt was emplaced and radiation therapy (whole brain 59 Gy) given. The tumor and the hydrocephalus regressed completely and he returned to work. Six years later, he experienced constipation and general fatigue. CT and echotomography of the abdomen showed a large peritoneal tumor and ascites. Laboratory investigation demonstrated serum levels of AFP 7640 ng/ml and HCG 150 IU/l, and high ascitic levels of AFP 12,890 ng/ml and HCG 1030 IU/l. AFP and HCG levels regressed after combined chemotherapy. However, he died due to leukopenia and pneumonia. Autopsy found no metastasis of tumor cells to the central nervous system. The peritoneal cavity contained hemorrhagic fluid and a large tumor 4100 g in weight. The tip of the V-P shunt tube was in front of the tumor. No neoplasm was found in the testis, retroperitoneal cavity, thymus, and other organs. The microscopic appearance of the peritoneal tumor was different to the first pineal tumor. The neoplasm was confirmed as a mixed germ cell tumor with teratoma components and suspected to be a metastasis of the pineal tumor through the V-P shunt system ((Saibara T, Hashimoto T, Takahashi M, Horie S, Fukami T, Nakagawa Y. [Abdominal metastasis of a pineal region tumor through ventriculoperitoneal shunt. Case report]. Neurol Med Chir (Tokyo). 1991 Dec;31(13):1012-7. Japanese. PubMed PMID: 1726235. )). ---- A 15-year-old boy 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 initial 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 ((Pallini R, Bozzini V, Scerrati M, Zuppi C, Zappacosta B, Rossi GF. Bone metastasis associated with shunt-related peritoneal deposits from a pineal germinoma. Case report and review of the literature. Acta Neurochir (Wien). 1991;109(1-2):78-83. Review. PubMed PMID: 2068974. )). ===1987=== a case of germinal tumor of the pineal gland, treated with a ventriculo-peritoneal shunt, cytostatics and radiotherapy. Two and a half years later, in Spite of the clinical remission of the cerebral tumor a metastasis appeared in the abdominal cavity. After reviewing the current literature, the necessity of using millipore filters in the CSF derivation systems when dealing with malignant cerebral tumors to avoid the spreading of malignant cells through the CSF ((Carcavilla LI, Gómez Perún J, Alberdi J, Eiras J, Arana T, Pisón J. [Abdominal metastasis of a pineal germinoma in a child with a ventriculo-peritoneal shunt]. An Esp Pediatr. 1987 Oct;27(4):303-7. Spanish. PubMed PMID: 3426024.)). ===1985=== The clinical findings and course of a patient with a germinoma of the pineal region is presented. After a surgical procedure and radiation treatment, ten months after, the patient develops successively peritoneal, ventricular, spinal metastasis. Effective treatment was either radiation treatment or chemotherapy. The frequency of metastasis with germinomas of the posterior part of the third ventricle are discussed, following a review of the literature, prevention and treatment are discussed ((Pelissou I, Ravon R, Moreau JJ, Mokhtari F, Vire O. [Multiple metastases during the course of a pineal germinoma]. Neurochirurgie. 1985;31(6):537-40. French. PubMed PMID: 3831777. )). ===1984=== Metastasis of pineal germinoma to the peritoneal cavity via the patient's ventriculoperitoneal shunt represents an unusual complication of this rare tumor. We report such a case with autopsy correlation of the abdominal CT findings. CT accurately delineated the extent of the metastatic mass and revealed associated complications including biliary tract obstruction, thrombosis of the inferior vena cava and a small amount of ascitic fluid. Since this tumor is very sensitive to radiotherapy and chemotherapy, the need for early detection of such metastasis is apparent. Possible implications with respect to the management of these patients are considered ((Devkota J, Brooks BS, el Gammal T. Ventriculoperitoneal shunt metastasis of a pineal germinoma. Comput Radiol. 1984 May-Jun;8(3):141-5. PubMed PMID: 6734160. )). ===1981=== A young man developed a tumor in the pineal region that grew along ventricular surfaces and caused obstructive hydrocephalus. Cytologic examination of cerebrospinal fluid demonstrated malignant cells consistent with germinoma. Following a ventriculoperitoneal shunt and radiotherapy, the tumor and the hydrocephalus regressed. Three years later, an intraperitoneal tumor appeared that was diagnosed by transcutaneous biopsy specimen as a germinoma. The abdominal germinoma also regressed after radiotherapy. While extracranial metastases of intracranial germinomas via shunts are probably rare, their occurrence, as exemplified by this case, argues for including protective filters in the shunts ((Haimovic IC, Sharer L, Hyman RA, Beresford HR. Metastasis of intracranial germinoma through a ventriculoperitoneal shunt. Cancer. 1981 Aug 15;48(4):1033-6. PubMed PMID: 7272927. )). ---- Extra-neural metastasis of pineal germinoma promoted by shunting device--a case report and review of the literature ((Sonoda H, Sano Y, Uemura S, Matsukado Y. [Extra-neural metastasis of pineal germinoma promoted by shunting device--a case report and review of the literature (author's transl)]. No Shinkei Geka. 1981 Jun;9(7):861-7. Japanese. PubMed PMID: 7266769. )). ===1979=== 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 ((Yamamoto Y, Waga S, Okada M. [Large abdominal pseudocyst as a complication of ventriculoperitoneal shunt--diagnosis by ultrasonography and whole body CT scan (author's transl)]. No Shinkei Geka. 1979 Jun;7(6):589-92. Japanese. PubMed PMID: 471170. )).