Contemporary Management of Pineocytoma

Pineocytoma is a rare tumor; therefore, assimilating data from case reports and small case series to generate definitive treatment guidelines is difficult. The authors recently systematically reviewed the existing literature on outcomes for patients with pineocytoma. Gross total resection is associated with significantly increased tumor control and survival compared with subtotal resection combined with radiotherapy. When gross total resection is not possible, adding radiotherapy to subtotal resection is not associated with increases in either tumor control or survival. Although aggressive surgery in the pineal region carries the risk of neurologic injury, gross total resection should be attempted for pineocytoma.

Pineocytoma is a rare tumor, accounting for 0.4% to 1% of all intracranial tumors. The literature is primarily composed to case reports and small case series, occasionally with pineocytoma analyzed together with pineal region tumors of varying histology and behavior. Consequently, reported outcomes have traditionally varied, with tumor control rates ranging from 67% to 100%. These outcomes are based on a variety of treatment modalities, including aggressive resection, subtotal resection, radiotherapy, radiosurgery, or chemotherapy. Therefore, generating definitive treatment goals and guidelines has been difficult. The authors recently systematically reviewed the existing literature on pineocytoma to generate treatment recommendations based on specific outcomes. This review comprised 64 publications describing 168 patients.

Surgical debulking improves tumor control and survival

The pineal region harbors tumors of diverse pathology, and therefore obtaining a definitive tissue diagnosis is the initial goal of surgery. Open biopsy in generally preferred relative to stereotactic biopsy for tumors in this location. Because of the unique location of the pineal gland with respect to the vein of Galen as it receives the basal vein of Rosenthal, the internal cerebral, and the inferior occipital veins, the benefits of any open surgical procedure in this area must be weighted against risk to these critical deep venous structures. These structures will be encountered regardless of approach selection. The authors showed that, in the setting of pineocytoma, surgical resection reduced the overall reported rate of progression compared with biopsy (6.3% vs 17.8%; χ ²P <.05). The 1- and 5-year progression-free survival rates for the surgical resection group versus the biopsy group were 97% versus 90%, and 89% versus 75%, respectively, which represented a statistically significant improvement on Kaplan-Meier analysis (log-rank, P <.05) ( Fig. 1 A). When comparing surgical resection versus biopsy, the 1- and 5-year overall survival rates were 89% versus 82%, and 76% versus 64%, respectively. Although the trend was toward improved survival with surgical resection, this difference did not reach statistical significance (log-rank, P = .19) (see Fig. 1 B). Thus, despite a suggestion of benefit, the data are insufficient to definitively conclude that surgical resection provides a survival benefit over a biopsy procedure combined with adjuvant therapy. Nevertheless, once committed to an open craniotomy to obtain a tissue diagnosis, tumor debulking should be attempted if the diagnosis is pineocytoma.

Gross total resection improves tumor control and survival

Aggressive surgery attempted in the pineal region carries the risk of devastating postoperative neurologic deterioration. Risk/benefit analyses based on outcomes data must be considered preoperatively. For the diagnosis of pineocytoma, no tumor recurrence was reported in patients who underwent gross total resection, which represented a significant improvement over patients receiving subtotal resection plus radiation (0% vs 9.5%, respectively; χ ²P <.05). The 1- and 5-year progression-free survival rates for the surgical resection group versus the biopsy group was 100% versus 94%, and 100% versus 84%, respectively, which represented a statistically significant improvement on Kaplan-Meier analysis (log-rank, P <.05) ( Fig. 2 A). The 1- and 5-year overall survival rates for the gross total resection group versus the subtotal resection plus radiation (subtotal resection plus radiotherapy) group was 91% versus 88%, and 84% versus 17%, respectively. On Kaplan-Meier analysis, these differences represented a statistically significant improvement (log-rank, P <.05) (see Fig. 2 B). When compared with gross total resection, the addition of adjuvant radiotherapy to subtotal resection does not seem to provide an equivalent tumor control or survival outcome. Therefore, despite the difficult location of pineocytoma and the associated inherent surgical risks, aggressive resection should be undertaken, because gross total resection can be potentially curative.

Gross total resection improves tumor control and survival

Adjuvant radiotherapy does not improve tumor control or survival

Limited data suggest a role for fractionated radiotherapy in pineocytoma management. Although the authors’ data show that gross total resection should be aggressively pursued to improve tumor control rates and extend survival, complete removal may not always be possible. In this setting, what is the role of adjuvant radiotherapy? In patients with pineocytoma, subtotal resection alone was associated with a similar rate of recurrence compared with treatment with subtotal resection plus radiotherapy (7.7% vs 11%; χ ²P = .86). The 1- and 5-year progression-free survival rates for the subtotal resection groups versus the subtotal resection plus radiotherapy group were 100% and 94% (1-year), and 100% and 81% (5-year), respectively, which were not significantly different according to Kaplan-Meier analysis (log-rank, P = .83).

The 1- and 5-year overall survival rates for the subtotal resection group versus the subtotal resection plus radiotherapy group were 77% versus 88%, and 77% versus 17%, respectively. Although a trend was seen toward decreased overall survival in patients treated with subtotal resection plus radiotherapy, this difference was not statistically significant (log-rank, P = .14). These results suggest that postoperative adjuvant radiotherapy after subtotal resection does not significantly add to tumor control rates or survival compared with subtotal resection alone. Taken together with the inability of subtotal resection with radiotherapy to replace gross total resection, these results suggest that pineocytoma is a relative radioresistant lesion and, in the absence of better evidence suggesting radiotherapy is beneficial for this lesion, that conventional fractionated radiation treatment has a minimal role as either a sole or adjuvant treatment for pineocytoma. When evaluating a patient who has previously undergone a subtotal resection, repeat craniotomy with the goal of complete removal should be considered depending on surgeon comfort.

Summary

Based on the rarity of pineocytoma and the long follow-up required to adequately document recurrence and overall survival, a prospective trial to address management issues is unlikely. In the absence of these data, current evidence indicates that surgical resection is the appropriate treatment for these tumors. When anatomically possible, gross total resection should be attempted, because this is associated with improved tumor control and longer progression-free and overall survivals. Adjuvant fractionated radiotherapy does not improve rate of tumor control or survival when used to treat a subtotally resected tumor. If possible, future studies are needed to address issues related to postoperative neurovascular complications, particularly in patients who undergo gross total resection. Particular focus on preoperative variables such as tumor size, and operative technique such as patient position, will aid in surgical decision-making guidelines.

The authors have nothing to disclose.

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