Ovarian and peritoneal psammocarcinoma: Results of a multicenter study on 25 patients
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
References (27)
- et al.
Low-grade serous neoplasia of the female genital tract
Surg Pathol Clin
(2019) - et al.
Primary peritoneal psammocarcinoma: a case presenting with an upper abdominal mass and elevated CA-125
Gynecol Oncol
(1999) - et al.
Primary peritoneal serous psammocarcinoma with recurrent disease and metastasis: a case report and review of the literature
Gynecol Oncol
(2007) - et al.
Primary psammocarcinoma of the ovary or peritoneum
Int J Gynecol Cancer
(2009) - et al.
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
(2012) - et al.
Bilateral serous psammocarcinoma of ovary: rare variant low grade serous carcinoma
Case Rep Obstet Gynecol
(2015) - et al.
Psammocarcinoma of ovary with serous cystadenofibroma of contralateral ovary: a case report
J Med Case Rep
(2009) - et al.
Serous psammocarcinoma of the ovary and peritoneum: two case reports and review of the literature
Arch Gynecol Obstet
(2009) - et al.
Serous psammocarcinoma of the ovary: CT and MR findings
J Comput Assist Tomogr
(2007) - et al.
Primary peritoneal psammocarcinoma
J Obstet Gynaecol Res
(2017)
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
Cited by (3)
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 OncologyCitation 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.