Ovarian and peritoneal psammocarcinoma: Results of a multicenter study on 25 patients

doi:10.1016/j.ejso.2019.12.005

European Journal of Surgical Oncology

Volume 46, Issue 5, May 2020, Pages 862-867

https://doi.org/10.1016/j.ejso.2019.12.005 Get rights and content

Abstract

Purpose

Psammocarcinoma (PK) is a rare disease of unknown origin. We aimed to report the characteristics, management and survival of patients operated on for PK within the French Network for Rare Peritoneal Malignancies (RENAPE) expert centers.

Patients and methods

All consecutive cases of PK operated within all 26 RENAPE centers between 1997 and 2018 were retrospectively analyzed.

Results

Twenty-five patients were identified. The median age was 53 years [range 17–78]. None of the patients had extra peritoneal metastases at diagnosis. A median of 6 cycles of carboplatin-based systemic chemotherapy was delivered in 52% preoperatively (n = 13) and 56% postoperatively (n = 14); associated with placlitaxel for 12 patients. All patients were operated on. The median PCI was 23 [0–33]. Eighty-four percent had a complete cytoreductive surgery through digestive (n = 7), spleen (n = 3), pancreas (n = 1) resections and/or multiple peritonectomies (n = 11). Five patients (20%) had intraperitoneal chemotherapy. Morbidity (Dindo-Clavien ≥3) was 12%. No postoperative death occurred. After a median follow-up of 42 months (range [2–194]), the median overall (OS) and progression-free (DFS) survival times were respectively 128 months and 31 months. Eighteen patients recurred (72%), mainly in the peritoneum (n = 16). Four of them (22%) were reoperated. The 5 and 10-year DFS rates were both 20.3%. The 5 and 10-year OS rates were 62% and 51.7%, respectively. A complete cytoreductive surgery was associated with a better OS and DFS in a univariate analysis.

Conclusion

Complete cytoreductive surgery is the cornerstone of the PK's management as a primary treatment. Recurrence remains common and new adjuvant strategies seem needed.

Introduction

Psammocarcinoma (PK) is a rare serous carcinoma arising either from the ovary or the peritoneum, for which the lack of epidemiological data renders its real incidence unknown [1,2]. PK are characterized by low grade cytological features with massive psammoma body formation occupying >75% of the tumor. Psammoma bodies are extra cellular multiple, discrete and laminated bodies formed by the accumulation of calcium on necrotic or degenerated tumor cells [3,4]. Most cases presents at diagnosis with asymptomatic or incidental detection or with non-specific clinical signs such as abdominal discomfort and increased abdominal perimeter [4,5]. The computed tomography (CT) scan aspect is quite typical with extensive calcified pelvic and peritoneal masses [6]. CA 125 levels are commonly moderately elevated [3,7].

The prognostic importance of psammoma bodies in serous carcinomas is controversial [8]. PK is described as a low grade disease with more favorable prognosis compared to invasive serous adenocarcinoma [3] but some metastatic cases have been described, suggesting an heterogeneous disease with, in some cases, aggressive behavior [9]. The medical literature comes down to a few clinical case reports and series of less than 10 patients [1] with miscellaneous treatment strategies. A total of 67 cases of ovarian and peritoneal PK have been reported in the literature [3,7]. Without any comparative study and long-term follow-up, the treatment is not yet standardized.

Thus, we aimed to report the characteristics, management and survival of patients operated on for PK within the French Network for Rare Peritoneal Malignancies (RENAPE) expert centers in order to determine the best treatment for these patients.

Section snippets

Study population – RENAPE database – eligibility criteria

Data were collected from the French RENAPE (French National Network of Peritoneal Surface Malignancies) database [10], including patients operated on from 26 expert centers in peritoneal surface malignancies between 1997 and 2018. All cases of pathologically documented PK were selected for the present study. Serous ovarian PK was defined as a variant of low grade serous carcinoma characterized by massive psammoma body formation >75% of the tumor mass and low-grade cytological atypia with not

Patient characteristics

A total of 25 patients were selected for the study, their characteristics are shown in Table 1. The median age was 53 years [range 17–78]. Initial surgery was performed in a reference center for 20 patients (80%). For the other 5 patients, the median interval between PK diagnosis and surgery in a reference center was 27.6 months [7.7–120.1]. None of the patients had extra peritoneal metastases at diagnosis and 5 patients had radiological signs of intra-abdominal lymph node invasion. Eight

Discussion

We report here the largest series of PK with peritoneal involvement and the longest follow-up ever published. Due to the retrospective nature of this study, we acknowledge some limitations and selection biases. Namely, as we analyzed data of patients from several centers treated over a relatively long period of time (21 years) in which surgical indications, techniques, teams’ experience, learning curves and systemic treatments evolved and regularly improved at the same time as medical

Conclusions

Complete cytoreductive surgery is the cornerstone of the PK's management as a primary treatment and at recurrence. However, recurrence remains common and new adjuvant strategies seem needed.

Source of support

None.

Declaration of competing interest

None.

Acknowledgments

The members of the RENAPE Network organization include: Julio Abba, MD (Department of Digestive Surgery, Grenoble University Hospital, Grenoble, France); Karine Abboud, MD (Department of General Surgery, CHU Hospital, Jean Monnet University, Saint Étienne, France); Mohammad Alyami, MD (Department of Digestive Surgery, Lyon-Sud University Hospital, Lyon, France); Catherine Arvieux, MD, PhD (Department of Digestive Surgery, Grenoble University Hospital, Grenoble, France); Gerlinde Averous, MD

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Cancer of unknown primary: ESMO Clinical Practice Guideline for diagnosis, treatment and follow-up <sup>☆</sup>
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Contemporary primary treatment of women with stage II-IV low-grade serous ovarian/peritoneal cancer (LGSOC): Determinants of relapse and disease-free survival
2022, Gynecologic Oncology
Citation Excerpt :
The diagnosis of psammocarcinoma is made once an invasive tumor contains not only at least 75% of psammoma bodies but also small papillary structures or clusters of cells with up to 15 cells across [29,30]. While several case reports suggest an excellent prognosis associated with psammocarcinoma [29,31,32], Delhorme et al. reported that 18/25 (72%) patients recurred, and the 10-year OS rate was 51.7% [30]. Clearly, further study of this histologic feature and its potential impact on outcomes is warranted.
The purpose of the present study is to describe a cohort who received contemporary primary treatment for stage II-IV low-grade serous ovarian/peritoneal cancer (LGSOC), including patient characteristics and determinants of relapse and disease-free survival.
The study included 99 patients: 1) with pathologically confirmed stage II-IV LGSOC of the ovary or peritoneum, 2) who underwent primary treatment consisting of cytoreductive surgery and either a) platinum/taxane chemotherapy followed by aromatase inhibitor maintenance therapy or b) aromatase inhibitor monotherapy, and 3) for whom there was availability of clinical data. Descriptive statistics were used to characterize clinicodemographic features. Subgroups were compared for PFS and OS. Multivariable Cox regression analyses were performed.
Median PFS for the entire cohort was 56.8 months (95% CI, 41.3-NE), and median OS was 130.7 months (95% CI, 115.0–146.4). Forty-nine of 99 (49.5%) patients have relapsed to date. For these 49 patients, median time from diagnosis to relapse was 29.6 months (95% CI, 24.6–33.1) (range, 5.4–69.1 months). Only 1/49 (2%) patients who relapsed did so >5 years from diagnosis. Fifty (50.0%) patients have not experienced disease progression or relapse. Median follow-up time for these 50 patients is 86.2 months (range, 25.3–169.0). Thirty-three of the 50 (66.0%) have been followed for >5 years from diagnosis. On regression analyses, factors associated with improved patient outcomes—either PFS, OS, or both–included no gross residual disease, normal serum CA 125 at diagnosis, primary peritoneal site, and presence of extensive psammomatous calcifications.
This is the first report to describe the clinicopathologic features and outcomes of women with stage II-IV LGSOC who received contemporary primary therapy.
Long-Term Follow-Up of Tamoxifen Treatment and the Use of Imaging in Psammocarcinoma: A Case Report, Review of the Literature and Discussion of Diagnostic and Therapeutic Challenges
2023, Current Oncology

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Ovarian and peritoneal psammocarcinoma: Results of a multicenter study on 25 patients

Abstract

Purpose

Patients and methods

Results

Conclusion

Introduction

Section snippets

Study population – RENAPE database – eligibility criteria

Patient characteristics

Discussion

Conclusions

Source of support

Declaration of competing interest

Acknowledgments

Surg Pathol Clin

Gynecol Oncol

Gynecol Oncol

Primary psammocarcinoma of the ovary or peritoneum

Int J Gynecol Cancer

Enormous cystic tumor of peritoneal psammocarcinoma exhibiting complete response to Cisplatin and cyclophosphamide after suboptimal cytoreduction: case report and review of the literature

Gynecol Obstet Investig

Bilateral serous psammocarcinoma of ovary: rare variant low grade serous carcinoma

Case Rep Obstet Gynecol

Psammocarcinoma of ovary with serous cystadenofibroma of contralateral ovary: a case report

J Med Case Rep

Serous psammocarcinoma of the ovary and peritoneum: two case reports and review of the literature

Arch Gynecol Obstet

Serous psammocarcinoma of the ovary: CT and MR findings

J Comput Assist Tomogr

Primary peritoneal psammocarcinoma

J Obstet Gynaecol Res

Primary psammocarcinoma of the peritoneum

Int J Gynecol Cancer

A unique case of ovarian psammocarcinoma with mediastinal, pulmonary, subcutaneous, and omental metastases

Arch Gynecol Obstet

The RENAPE observational registry: rationale and framework of the rare peritoneal tumors French patient registry

Orphanet J Rare Dis