Enormous cystic tumor of peritoneal psammocarcinoma exhibiting complete response to Cisplatin and cyclophosphamide after suboptimal cytoreduction: case report and review of the literature.
TL;DR: This case implies that CP therapy is a potential regimen of postoperative remission-induction therapy for suboptimally resected primary peritoneal psammocarcinoma.
Abstract: Psammocarcinoma is a serous peritoneal tumor arising from the ovary or the peritoneum and characterized by low-grade nuclear features, extensive psammoma bodies, and invasiveness Only 62 cases have ever been documented, 30 primary peritoneal and 32 primary ovarian, most of which presented as small tumors Adjuvant therapies, including chemotherapy and radiation, were performed in 12 of the primary peritoneal cases, without any clear evidence of benefit We present a case of an unusually large primary peritoneal psammocarcinoma with unexpected outcome The patient was a 38-year-old woman with a tumor of the peritoneum which adhered densely to the uterus and rectum and developed into the intra-abdominal cavity and retroperitoneal space After adhesiolysis of the tumor and rectum, suboptimal surgical reduction left a 4 cm × 2 cm tumor segment Postoperative chemotherapy, consisting of paclitaxel and carboplatin (TC) for 1 course, and cyclophosphamide and cisplatin (CP) for 5 courses, was conducted The residual tumor responded completely to the chemotherapy and the patient is alive today, with no evidence of disease 15 months after the surgery Our case implies that CP therapy is a potential regimen of postoperative remission-induction therapy for suboptimally resected primary peritoneal psammocarcinoma
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TL;DR: Complete cytoreductive surgery is the cornerstone of the PK's management as a primary treatment and was associated with a better OS and DFS in a univariate analysis.
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.
3 citations
TL;DR: A rare case of PPP in a 74-year-old female, discovered fortuitously within a laparotomy for gallbladder lithiasis, found that both ovaries were intact having a normal size and died of SARS-CoV-2 six days after surgery.
Abstract: Psammocarcinoma is an uncommon subtype of low-grade serous carcinoma. It is characterized by the presence of extensive psammoma bodies and can have either an ovarian or peritoneal origin. To our knowledge fewer than 30 cases of primary peritoneal psammocarcinoma (PPP) have been reported in the English literature. We report a rare case of PPP in a 74-year-old female, discovered fortuitously within a laparotomy for gallbladder lithiasis. At laparotomy, multiple nodular implants involving the omentum, the peritoneum and a magma of intestinal loops in the right iliac fossa were noted. A biopsy from nodules was performed. Gross examination showed multiple nodules of different sizes in the fat tissue. Pathologic examination showed massive psammoma bodies representing more than 75% of the tumor. The final diagnosis was psammocarcinoma. Our patient was referred to the gynecologic department for further investigation and to ascertain whether the tumor arose from the ovaries or peritoneum. Hysterectomy, bilateral adnexectomy and omentectomy were performed. Macroscopic examination showed that both ovaries were intact having a normal size. No invasion of ovarian stroma was shown in microscopic examination. The patient died of SARS-CoV-2 (COVID-19) six days after the surgery. PPP is a rare type of low-grade serous carcinoma. The behavior of this tumor is unclear, and the treatment is not standardized because of its rarity and lack of long-term follow-up. More cases need to be studied for better understanding and improvement of the management protocols.
2 citations
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01 Jan 1992
TL;DR: The publication of this edition marks the transition in diagnosis of lower genital tract lesions from a largely morphologic activity to one based upon integrated assessment using microscopy and molecular biology, and emphasise the importance of immunohistochemistry and molecular in situ hybridisation as adjuncts to morphology in routine clinical diagnosis.
Abstract: The publication of this edition marks the transition in diagnosis of lower genital tract lesions from a largely morphologic activity to one based upon integrated assessment using microscopy and molecular biology. Furthermore, the ability to integrate these diagnostic approaches with clinical data for patient management has been heightened. The introduction of prophylactic vaccines for preventing human papillomavirus (HPV) infections and cervical cancer precursors also thrusts pathology into a new unprecedented public health role with regard to disease monitoring, which will likely expand over time. Finally, the realisation that cervical cancer is becoming increasingly preventable carries with it both a sense of accomplishment and the recognition that this must now be translated into an achievable goal of cancer prevention in underdeveloped nations. Although the text has remained concise in order to maintain the primary function of the book as an atlas rather than a comprehensive textbook, it has been substantially updated to reflect the changes in the field since the previous edition was published in 1992. In this regard, the number of illustrations, all of which are in colourr, has almost doubled. Both the text and the photomicrographs emphasise the importance of immunohistochemistry and molecular in situ hybridisation as adjuncts to morphology in routine clinical diagnosis. A binary (low-grade and high-grade squamous intraepithelial lesion) classification for histology, analogous to TBS for cytology, replaces the four-tiered dysplasia/CIS system and the three-tiered CIN system. This Atlas will be a useful reference for pathologists, residents, and students for years to come.
232 citations
TL;DR: The data suggest that EPSPC is clinically similar to PSOC and support the need for a prospective clinical trial to compare these two entities further, and there were no significant differences in tumor response to therapy, disease-free interval, and actuarial survival between cases and controls.
225 citations
TL;DR: The clinical behavior of psammocarcinoma more closely resembles that of borderline serous tumors than of serous carcinomas of the usual type, following a protracted course and being associated with a relatively favorable prognosis.
Abstract: We report 11 cases of serous psammocarcinoma, a rare variant of serous carcinoma characterized by massive psammoma body formation and low-grade cytological features. The patients ranged in age from 36 to 76 (mean of 57) years. The tumors were stage three in every case (three stage IIIA, seven stage IIIB, and one stage IIIC). Eight of the patients had ovarian psammocarcinomas with ovarian tumors measuring 5-22 (mean of 11) cm in diameter; the other three patients had primary peritoneal psammocarcinomas. The surfaces of the ovarian tumors were smooth and intact in seven of eight ovarian psammocarcinomas. Cysts were present in six of eight ovarian tumors, and the solid portions were typically gritty or granular. Necrosis was not seen. Microscopically there was destructive invasion of ovarian stroma or vascular invasion in the ovarian tumors; the extraovarian tumor implants were invasive of intraperitoneal viscera in five cases. The epithelium was arranged in small nests with no areas of solid epithelial proliferation, and at least 75% of the epithelial nests were associated with psammoma body formation. No more than moderate nuclear atypicality was identified in any case. No mitotic figures were found in 10 of the tumors, with a single mitotic figure identified in the other tumor. Follow-up data for more than 1 year was available for six patients, with two patients lost to follow-up and three patients followed without evidence of recurrence for less than 1 year. One patient died of tumor 6 1/2 years after presentation, and five patients were alive without evidence of recurrent tumor 3-10 (mean of 8.3) years after presentation. Although based on a small number of cases, these data suggest that the clinical behavior of psammocarcinoma more closely resembles that of borderline serous tumors than of serous carcinomas of the usual type, following a protracted course and being associated with a relatively favorable prognosis.
120 citations
TL;DR: Low-grade peritoneal serous carcinomas, psammocarcinoma, and serous borderline tumors ofPeritoneal origin share some clinicopathologic features that may be underrecognized at surgery and gross examination and Conservative therapy is merited for younger women with borderline tumors.
Abstract: Low-grade peritoneal serous carcinomas have been the subject of limited study, and their distinction from peritoneal serous psammocarcinomas and serous borderline tumors is not always easy. The clinicopathologic features of 14 low-grade serous carcinomas, 7 psammocarcinomas, and 19 serous borderline tumors of peritoneal origin were compared. Average ages were 58 years (low-grade serous carcinomas), 48 years (borderline tumors), and 40 years (psammocarcinomas). Typical clinical presentations were abdominal pain, abdominal mass, or both, with the tumors incidental in 37% (borderline tumors), 43% (psammocarcinomas), and 36% (low-grade serous carcinoma). Operative and gross findings varied from nodules to adhesions to a dominant mass. Treatment was surgical debulking in most cases, with biopsy alone for eight borderline tumors. Seven patients with low-grade serous carcinoma were alive when last seen, but follow-up duration is short (average, 1.2 years): five were without disease, one had recurrent disease and one persistent disease. One patient with serous carcinoma died of disease at 3.5 years, and two patients died of other causes. Three patients with psammocarcinoma were alive without disease (average 3.3 years). Fourteen patients with borderline tumors were alive (average 3 years): 10 were without disease, 2 had persistent disease, and serous carcinoma developed in 2. The low-grade serous carcinomas resembled the invasive implants of ovarian serous borderline tumors. lacked high-grade nuclear atypia, showed tissue, lymphovascular space invasion, or both and had appreciable solid epithelial proliferation. Some serous carcinomas showed abundant psammomatous calcification suggesting psammocarcinoma but had too much epithelial proliferation for that diagnosis. The psammocarcinomas showed at least 75% psammoma bodies, no more than moderate cytological atypia, tissue or lymphovascular space invasion, or both, and rare epithelial proliferation less than 15 cells across. Adequate sampling was necessary to identify invasion, with highest yields of invasive foci in omental samples; individual foci in some cases of carcinoma resembled borderline tumor. The serous borderline tumors resembled the noninvasive implants of ovarian serous borderline tumors, lacked invasion, and did not show nuclear atypia of the degree seen in grade 2 or grade 3 serous carcinoma. Low-grade serous carcinoma, psammocarcinoma, and serous borderline tumors of peritoneal origin share some clinicopathologic features and may be underrecognized at surgery and gross examination. Because of overlapping microscopic patterns, adequate sampling is mandatory to identify small foci of invasion that exclude a borderline tumor and identify significant cellularity that excludes a psammocarcinoma. Conservative therapy is merited for younger women with borderline tumors. Maximum debulking is recommended for bulky symptomatic borderline tumors, low-grade serous carcinoma, and psammocarcinoma. Although short-term outcomes for the carcinomas appear favorable, follow-up is too limited to determine long-term outcomes.
65 citations
TL;DR: This case report of an aggressive course of this disease is presented to alert others that psammocarcinoma may not always follow a benign course and patients with this disease should have optimal tumor debulking.
32 citations