Case Of Papillary Tumor Of The Pineal Region Research Articles

Papillary tumor of pineal region: Case reports

Background: The papillary tumor of the pineal region (PTPR) was a new distinct entity described in 2003 WHO classification and later included in the subsequent 2007 and 2016 WHO classification of Central Nervous System tumours. They are commonly associated with obstructive hydrocephalus and are difficult to distinguish from other clinical entities, more particularly pineocytoma. Due to the limited number of reported cases, treatment strategies are not well established. Case report: We report two cases of PTPR who presented with headache impaired gaze and diplopia. In first case MRI showed a cystic mass in the third ventricle and pineal region whereas the tumor had displaced the tectal plate in second case. The initial squash suggested a neoplastic lesion of probable glial origin favoring ependymoma. On microscopy, the tumor displayed papillary architecture with perivascular pseudo rosettes displaying multi layering of cuboidal to columnar cell with vesicular nucleus. Based on histological features the differential diagnostic conditions considered were choroid plexus tumor, papillary ependymoma, choroid plexus carcinoma, PTPR, and metastatic carcinoma. PTPR was confirmed on immunohistochemistry workup. Both the patients had recurrence, after one year and six months respectively. Conclusion: PTPR is an important differential diagnosis to be considered in cases where the tumoris arising in the vicinity of pineal region showing papillary architecture. Keywords: pineal region papillary tumor; PTPR; pineal region; subcommissural organ

Papillary tumor of the pineal region-report of three cases with literature review.

Papillary tumor of the pineal region (PTPR) is a rare tumor of the pineal region. Not much is known about the pathogenesis, prognosis, and treatment protocol of this uncommon entity. We present three cases of PTPR with follow-up from 8 months to 98 months. All patients presented with headache and visual disturbance. One patient also had amenorrhea. Radiology revealed an heterogeneously enhancing mass arising from the pineal region with associated hydrocephalus. Histopathologically, all cases showed the papillary architecture, strong pan cytokeratin and cytokeratin 18 positivity, and faint positivity for synaptophysin and neuron-specific enolase. All cases received postoperative radiotherapy. One case showed tumor recurrence after 7 years. Other two cases did not show any recurrence till the last follow-up.

Clinicopathologic features of papillary tumors of the pineal region

To study the clinicopathologic features of papillary tumor of the pineal region (PTPR). Three hundred and eighty six cases of pineal region and posterior third ventricle tumors, two newborn and two adult pineal glands were analyzed by HE, PAS and immunohistochemistry of 16 antibodies (EnVision method). Five cases of PTPR were diagnosed with mixed papillary features and densely cellular areas, and included one recurrent case. In the papillary areas, the vessels were lined by one or several layers of cuboidal/columnar cells; the vessel wall was hyalinized. In the densely cellular areas, sheets or nests of tumor cells were seen. The tumor cells of these five cases were immunoreactive to CK, CK8/18, synaptophysin, MAP2, nestin, S-100, and vimentin. Four cases were immunoreactive to NSE and CgA; and 2 cases were immunoreactive to NF. All five cases were negative for EMA, CK5/6, CEA, and NeuN. Ki-67 labeling index ranged from 1% to 6%.Three patients were alive, and the recurrent one died. PTPR occurs in patients with over a wide age range, from children to adults, and is more commonly found in male than female. PTPR is composed of both papillary and solid areas, characterized by epithelial cytology, and needs to be differentiated from ependymoma. PTPR may originate from the specialized ependymocytes of the subcommissural organ. The prognostic factors are early diagnosis, complete surgical resection and radiotherapy.

Papillary tumor of the pineal region in a 15-month-old boy

The papillary tumor of the pineal region (PTPR) is a distinct entity that is particularly rare in the pediatric population. The authors document the youngest reported patient with this clinicopathological entity to date. A case of PTPR in a 15-month-old boy is described. Initially thought to be a tectal glioma, the tumor was later identified as a pineal region tumor after demonstrating growth on routine imaging. Diagnosis of PTPR was established by histopathological evaluation of biopsy samples, which revealed papillary, cystic, and solid tumor components. The patient's postoperative course was complicated by tumor growth despite several debulking procedures and chemotherapy, as well as persistent hydrocephalus requiring 2 endoscopic third ventriculostomies and eventual ventriculoperitoneal shunt placement. After a 15-month follow-up period, the patient has received proton-beam therapy and has a stable tumor size. The PTPR is a recently described tumor of the CNS that must be included in the differential diagnosis of pineal region masses. The biological behavior, prognosis, and appropriate treatment of PTPR have yet to be fully defined.

Bcl‐2 expression in a papillary tumor of the pineal region

Neuroepithelial papillary tumor of the pineal region (PTPR) has been described by several groups and recognized by the 2007 World Health Organization Classification of Tumors of the Central Nervous System. The proto-oncogen Blc-2 can function as an apoptosis suppressor and can promote neoplastic transformation. It may also be involved in neuroendocrine differentiation in some tumors. As PTPRs express neuroendocrine markers, we investigated the expression of Bcl-2 in tumoral cells of a new case of PTPR in a 42-year-old woman. Bcl-2 immunostaining was detected in the cytoplasm of the tumoral cells; staining intensity was heterogeneous from cell to cell and more intense in papillary areas. This intense expression of Bcl-2 in one case of PTPR with a high proliferation index (8%) might be related to the malignancy of this neoplasm. It will be interesting to investigate the prognosis impact of Bcl-2 expression in a large series of PTPRs.