Stage I granulosa cell tumours: A management conundrum? Results of long-term follow up

Characteristics and outcome of recurrence in molecularly defined adult-type ovarian granulosa cell tumors

“…We found tumor rupture and stage IC, including both IC1 and IC2, as significant risk factors for recurrence in the patient cohort with mostly stage I tumors, similarly to a recent study [9]. In our cohort, the vast majority of these patients were primarily treated by surgery alone, which is also the current treatment protocol for all stage I AGCTs at HUH.…”

“…Prior studies focusing on AGCT recurrence have, however, lacked the diagnostic accuracy achieved with molecular validation. The 32% recurrence rate with a median time-to-relapse of 7.4 years in this study represents a slightly higher rate and longer median time than observed in most prior works, where recurrence rates vary significantly from 5% to 64% [18,20], although most studies with large patients series, extensive follow-up and tertiary-center treatment have documented a recurrence rate of 20-30% [9,17,26].…”

“…Despite the indolent disease course and relatively favorable prognosis, AGCTs exhibit an unpredictable tendency to late relapse [4][5][6][7][8][9]. Previous studies have shown that up to one-third of patients with AGCT develop tumor recurrence, even in early stage disease, leading to increased mortality [8,10].…”

“…The only clinical factor consistently related to prognosis is tumor stage [4,5,[10][11][12][13][14][15]. Tumor size, tumor rupture, presence of residual tumor, incomplete staging, and even body mass index and diabetes have been suggested as clinical prognostic factors for recurrence [9,10,[15][16][17][18][19]. Histopathologic and molecular prognosticators have included nuclear atypia, mitotic rate, diffuse growth pattern and expression of transcription factor GATA4 and human epidermal growth factor receptor HER2 [12,13,18,20,21].…”

Resisting RECIST—Uniformity Versus Clinical Validity

“…37 In diseases such as granulosa cell tumors of the ovary where the natural history is often very indolent, stable disease may be more indicative of disease biology than treatment effect, which needs important consideration. 38 In studies of patients with metastatic solid tumors treated with placebo or best supportive care, a disease stabilization rate of 25% (range, 0%Y67%) has been demonstrated. 39 Many argue that the ''degree of response'' is more relevant for assessing clinical benefit, 30 and more recently, waterfall plots have been used to chart each individual patient's response and provide a measure of the ''amount of response.''…”