Anuj Verma1, Sridhar Epari1, David Bakiratharajan1, Ayushi Sahay1, Naina Goel2, Girish Chinnaswamy3, Prakash Shetty4, Aliasgar Moiyadi4, Atul Goel5, Tejpal Gupta6, Rakesh Jalali6. 1. Department of Pathology, Tata Memorial Centre, Homi Baba National Institute, Mumbai, Maharashtra, India. 2. Department of Pathology, King Edward Memorial Hospital and Seth Gordhandas Sunderdas, Mumbai, Maharashtra, India. 3. Department of Pediatric Oncology Division of Medical Oncology, Tata Memorial Centre, Homi Baba National Institute, Mumbai, Maharashtra, India. 4. Department of Neurosurgery Division of Surgical Oncology, Tata Memorial Centre, Homi Baba National Institute, Mumbai, Maharashtra, India. 5. Department of Neurosurgery, King Edward Memorial Hospital and Seth Gordhandas Sunderdas, Mumbai, Maharashtra, India. 6. Department of Radiation Oncology, Tata Memorial Centre, Homi Baba National Institute, Mumbai, Maharashtra, India.
Abstract
BACKGROUND: Pineal gland tumors range from the well-differentiated "pineocytoma" [World Health Organization (WHO) grade I], which have a very good prognosis, to the aggressive and poorly differentiated "pineoblastoma" (WHO grade IV) with "pineal parenchymal tumor of intermediate differentiation" (PPTID; WHO grades II and III) occupying intermediary differentiation and prognosis. Papillary tumor of the pineal region (PTPR; WHO grades II and III) is a distinct entity with propensity for recurrence and spinal dissemination. However, the diagnostic criteria to differentiate these entities, especially between WHO grades II and III of both PPTID and PTPR, remain nebulous. OBJECTIVE: To evaluate the relative frequency of the individual entities and histomorphological (including the proliferation indices) features across the spectrum of pineal parenchymal tumors (PPTs) [including PTPRs] along their course. DESIGN: All cases of PPTs were retrieved, reviewed, and graded based on the histological criteria defined in the literature. RESULTS: PPTID, more commonly seen in young adults, was the most common subtype of PPT. This was followed by pineoblastoma which was more commonly seen in children. Clinical progression was seen in both grades II and III of PPTID; however, it was more commonly seen in cases with a MIB1 labeling index of >10%. PTPRs (both grades II and III) showed an aggressive histological transformation and also intraparenchymal metastasis. CONCLUSION: PPTIDs are the most common adult primary PPTs and have the potential to progress and disseminate in both grades II and III. Both grades of PTPRs have a metastatic potential. These findings suggest the need for postoperative adjuvant therapy in both grades of PPTID and PTPR.
BACKGROUND: Pineal gland tumors range from the well-differentiated "pineocytoma" [World Health Organization (WHO) grade I], which have a very good prognosis, to the aggressive and poorly differentiated "pineoblastoma" (WHO grade IV) with "pineal parenchymal tumor of intermediate differentiation" (PPTID; WHO grades II and III) occupying intermediary differentiation and prognosis. Papillary tumor of the pineal region (PTPR; WHO grades II and III) is a distinct entity with propensity for recurrence and spinal dissemination. However, the diagnostic criteria to differentiate these entities, especially between WHO grades II and III of both PPTID and PTPR, remain nebulous. OBJECTIVE: To evaluate the relative frequency of the individual entities and histomorphological (including the proliferation indices) features across the spectrum of pineal parenchymal tumors (PPTs) [including PTPRs] along their course. DESIGN: All cases of PPTs were retrieved, reviewed, and graded based on the histological criteria defined in the literature. RESULTS: PPTID, more commonly seen in young adults, was the most common subtype of PPT. This was followed by pineoblastoma which was more commonly seen in children. Clinical progression was seen in both grades II and III of PPTID; however, it was more commonly seen in cases with a MIB1 labeling index of >10%. PTPRs (both grades II and III) showed an aggressive histological transformation and also intraparenchymal metastasis. CONCLUSION: PPTIDs are the most common adult primary PPTs and have the potential to progress and disseminate in both grades II and III. Both grades of PTPRs have a metastatic potential. These findings suggest the need for postoperative adjuvant therapy in both grades of PPTID and PTPR.
Entities:
Keywords:
Pineal gland; papillary tumors of the pineal region; pineal anlage tumor; pineal parenchymal tumor of intermediate differentiation; pineoblastoma