Treatment and long-term outcomes in pituitary carcinoma: a cohort study

Abstract

Background Pituitary carcinoma (PC) is an aggressive neuroendocrine tumor diagnosed when a pituitary adenoma (PA) becomes metastatic. PCs are typically resistant to therapy and develop multiple recurrences despite surgery, radiotherapy and chemotherapy. Recently, treatment with temozolomide (TMZ) has shown promising results, although the lack of prospective trials limits assessment of benefit. Methods We describe a single-center multidisciplinary experience in managing PC patients over a 22-year period and review previously published PC series. Results Seventeen patients were identified. Median age at PC diagnosis was 44 years (range 16–82 years), and the median time from PA to PC transformation was 5 years (range 1–29 years). Median follow-up time was 28 months. Most PCs were hormone-positive (n = 12): ACTH (n = 5), PRL (n = 4), LH/FSH (n = 2) and GH (n = 1). All patients underwent at least one resection and at least one course of radiation after PC diagnosis. Immunohistochemistry showed high Ki-67 labeling index (>3%) in 10/15 cases. Eight patients (47%) had only central nervous system (CNS) metastases; six (35%) had combined CNS and systemic metastases. The most commonly used chemotherapy was TMZ, and TMZ-based therapy was associated with the longest PFS in 12 (71%) cases, as well as the longest period from PC diagnosis to first progression (median 30 months). The 2, 3 and 5-year survival rate of the entire cohort was 71, 59 and 35%, respectively. All patients surviving >5 years had been treated with TMZ-based therapy. Conclusions PC management benefits from multidisciplinary care and multimodality therapy. TMZ-based regimens were associated with high survival rates and long disease control.

How to Classify Pituitary Neuroendocrine Tumors (PitNET)s in 2020

“…Of note, very high Ki-67 index and high mitotic count should alert pathologists to consider other diagnosis than pituitary tumor. Indeed, Ki-67 exceeding 10% are rarely observed in pituitary tumor as shown in a recent cohort [65]. In particular, the diagnosis of null cell adenoma should be considered with caution these cases.…”

Clinical Efficacy of Temozolomide and Its Predictors in Aggressive Pituitary Tumors and Pituitary Carcinomas: A Systematic Review and Meta-Analysis

“…Encouraged by these findings, TMZ has been increasingly employed as salvage treatment for APT and PC patients after the failure of standard management with surgical, medical, and radiational treatments (9). TMZ has been demonstrated as a safe therapeutic agent offering a high clinical response rate in patients with APT and PC (10).…”

Pimozide augments bromocriptine lethality in prolactinoma cells and in a xenograft model via the STAT5/cyclin D1 and STAT5/Bcl‑xL signaling pathways

“…In the present study, increased expression of p-JAK2 and p-STAT5 was observed in bromocriptine-resistant prolactinoma tissues and cells. In our clinical practice, patients with bromocriptine-resistant prolactinoma usually suffer from hyperprolactinemia (26)(27)(28). Increased serum levels of PRL tend to bind PRLR forming a complex, and further activate the classical PRL/JAK2/STAT5 pathways (8,29,30).…”