Extremely Late Recurrence 21 Years after Total Removal of Immature Teratoma: A Case Report and Literature Review

Abstract

Immature teratoma (IMT) is normally treated by resection and adjuvant therapy. The present unusual case of recurrent germinoma occurred 21 years after total resection of pineal IMT. A 3-year-old boy presented with headache, disturbance of consciousness, and Parinaud’s syndrome. Magnetic resonance (MR) imaging revealed a pineal mass lesion, and total resection of the tumor was achieved. The histological diagnosis was mature teratoma. He did not receive further treatment, and did well without recurrence for 20 years. However, he suffered headache 21 years after resection, and MR imaging revealed a homogeneously enhanced pineal mass with low minimum apparent diffusion coefficient value and proton MR spectroscopy showed a huge lipid peak. The levels of tumor markers were not elevated. Cerebrospinal fluid (CSF) cytology found atypical cells with large nuclei and irregularly shaped nucleoli. To elucidate the relationship between the primary and recurrent tumors, we reviewed the histological specimens and CSF cytology at the initial treatment and found a subset of incompletely differentiated components resembling fetal tissues in the histological specimen and atypical large cells in the CSF. Based on these radiological and histological findings, we presume that the recurrent disease was disseminated germinoma after the resection of disseminated IMT. He received chemotherapy and craniospinal radiation therapy, and the enhanced lesion and atypical cells in the CSF disappeared. This case demonstrates that disseminated IMT can be controlled for the long term without adjuvant therapy, but may recur as germinoma. Tumor dormancy may account for this unusual course.

Multiple ectopic recurrent germ cell tumors after total pineal mature teratoma removal: A case report and literature review

“…Therefore, the suprasellar tumor is likely to be a metachronous tumor, which is also consistent with the theory of the multi-center origin of GCTs. Cases of new GCTs occurring long after total removal of mature teratomas have been similarly reported (19,20). In these "recurrent tumors", germinoma seems to be more common (19).…”

Pituitary germinoma after resection of a mature third ventricular teratoma: illustrative case

“…Contrary to de novo occurrences, miGCTs, most likely recurrences of the primary neoplasm, have been described. 6 , 7 , 14 , 16 , 17 , 19 Recurrences are usually, but not always, marked through a shorter time interval between occurrence of the primary lesion and that of the iGCT (< 2 years) or attributed to regrowth of microscopically residual tumors. 6 , 17 , 21 Recurrent miGCTs can be explained through primary tumor dissemination before diagnosis and surgical treatment, remaining dormant until a later stage, or, if systemic treatment was applied, by nonradiosensitive cells surviving radiation.…”

Intracranial immature teratoma invading the nasal cavity mimicking olfactory neuroblastoma

“…[ 21 ] The prognosis of congenital intracranial immature teratoma is usually poor because the tumor is usually large and is accompanied by extensive invasion. [ 7 – 10 , 14 ] Late recurrence is uncommon in these patients, [ 22 ] but Mano et al reported a case of intracranial immature teratoma that recurred 21 years after the surgery of the primary tumor. [ 22 ] There are few reports of tumors in adults, and the prognosis in these cases remains to be studied.…”