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Journal ArticleDOI

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

15 Dec 2009-Journal of Medical Case Reports (BioMed Central)-Vol. 3, Iss: 1, pp 9330-9330

TL;DR: This is the rare case describing coexistence of this very rare malignant serous epithelial tumor with a benign serous cystadenofibroma of contralateral ovary in a 55 year old Asian Indian female.

AbstractIntroduction Psammocarcinoma of ovary is a rare serous neoplasm characterized by extensive formation of psammoma bodies, invasion of ovarian stroma, peritoneum or intraperitoneal viscera, and moderate cytological atypia. Extensive medlar search showed presence of only 28 cases of psammocarcinoma of ovary reported till date.

Topics: Psammoma body (53%), Ovary (51%)

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Citations
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Journal ArticleDOI
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

1 citations


Journal ArticleDOI
TL;DR: Both CT corrected and uncorrected PET images showed hypermetabolism in the massively calcified lymph nodes in the neck, mediastinum, axilla and abdomen, indicative of active residual disease.
Abstract: The contribution of positron emission tomography/computed tomography (PET/CT) with 18F-fludeoxyglucose (FDG) in evaluating ovarian cancer recurrence even after a prolonged disease-free interval, and in therapy response is well-described. Calcifications observed in CT, although usually attributed to benign conditions, may actually represent active disease. Such an example of calcified formations is psammoma bodies. We present a case of 56-y. o. patient with ovarian cancer relapse at the supraclavicular area 18 years after complete response and disease-free interval. The patient received chemotherapy and underwent 18F-FDG-PET/CT for the evaluation of treatment response. Both CT corrected and uncorrected PET images showed hypermetabolism in the massively calcified lymph nodes in the neck, mediastinum, axilla and abdomen, indicative of active residual disease.

1 citations


Cites background from "Psammocarcinoma of ovary with serou..."

  • ...Although the exact mechanism of psammoma body formation is yet unclear, they are known to be associated with increased apoptotic cell death, BRAF mutation, and normal TP53 function, all of which are more profound in low-grade ovarian serous adenocarcinoma (22,23,24)....

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  • ...Cases reported in the literature include primary psammocarcinomas of the ovary or peritoneum, or psammoma deposits in benign and malignant conditions such as serous adenocarcinoma of the ovaries, thyroid, meningioma, pancreas and calcifications in metastatic lesions (3,6,14,15,16,20,21,22,25,26)....

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  • ...The later arise from the ovaries or peritoneum, and certain criteria must be fulfilled in order to characterize a carcinoma with psammoma bodies as psammocarcinomas (22)....

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  • ...They are also likely to represent a biologic process which leads to degeneration of cancer cells, with consequent death and delay of tumor growth (14,19,21,22)....

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Journal ArticleDOI
TL;DR: A 60-year-old postmenopausal woman who presented with abdominal distension and elevated serum CA-125 suggested malignant ovarian neoplasm is reported, which showed bilateral serous psammocarcinoma of ovary with invasive implants on omentum.
Abstract: Serous psammocarcinoma is a rare variant of serous carcinoma arising from either ovary or peritoneum, characterized by massive psammoma body formation, low grade cytologic features, and invasiveness. Its clinical behavior is similar to serous borderline tumors with relatively favorable prognosis. We report herein a case of a 60-year-old postmenopausal woman who presented with abdominal distension. Contrast enhanced computed tomography (CECT) revealed calcified pelvic masses with ascites. Elevated serum CA-125 (970 U/mL) suggested malignant ovarian neoplasm. Patient underwent exploratory laparotomy with primary debulking surgery. Histopathology showed bilateral serous psammocarcinoma of ovary with invasive implants on omentum. Adjuvant chemotherapy was advised in view of advanced stage disease, although its benefits are poorly defined due to rarity of the tumor. However, patient opted out of it and is now on follow-up.

1 citations


Cites background from "Psammocarcinoma of ovary with serou..."

  • ...These tumors are usually unilateral, but rarely may these be bilateral as in our case [11]....

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01 Jan 2012
TL;DR: A new case of aggressive psammocarcinoma of ovary in 65 years old woman is reported, linked to a better prognosis than conventional serous adenocarcine.
Abstract: Psammocarcinoma of ovary is a rare serous neo- plasm characterized by extensive formation of psammoma bodies with ovarian stromal invasion. Rarely aggressive with invasion of the peritoneum or intraperitoneal viscera, it is linked to a better prognosis than conventional serous adenocarcinoma. It is characterized by a tumor cells with little cytological atypia and a low nuclear grade. Extensive medlar search showed presence of only 29 cases of psammo- carcinoma of ovary reported till date. A new case of aggres- sive psammocarcinoma of ovary in 65 years old woman is reported.

Journal ArticleDOI
Jean-Baptiste Delhorme1, Jean-Baptiste Delhorme2, Jordan Ohayon1, Sebastien Gouy1, Gerlinde Averous2, Catherine Genestie1, Léopold Gaichies1, Olivier Glehen, Jean-Marc Guilloit, Denis Pezet, François Quenet, Gwenael Ferron3, Cécile Brigand2, Philippe Morice1, Charles Honoré1, Julio Abba4, Karine Abboud5, Mohammad Alyami6, Catherine Arvieux4, Naoual Bakrin6, G. Balague, Vincent Barrau, Houda Ben Rejeb, J.M. Bereder, Isabelle Berton-Rigaud, Frédéric Bibeau7, Isabelle Bonnefoy6, Dominique Bouzard, I. Bricault4, S. Carrere, Cécile de Chaisemartin, Madleen Chassang, Anne Chevallier, Thomas Courvoisier, Peggy Dartigues1, Anthony Dohan, Julien Dubreuil6, Frédéric Dumont, Clarisse Eveno, Marie Faruch-Bilfeld, Juliette Fontaine6, Laure Fournier, Johan Gagnière, Delphine Geffroy, Laurent Ghouti, François-Noël Gilly6, Laurence Gladieff, Diane Goéré1, Aymeric Guibal, Frédéric Guyon, Bruno Heyd, Christine Hoeffel8, Constance Hordonneau, S. Isaac6, Peggy Jourdan-Enfer6, R. Kaci, R. Kianmanesh8, Catherine Labbé-Devilliers, Joëlle Lacroix, Bernard Lelong, Agnès Leroux-Broussier, Yoann Lherm, Rea Lo Dico, Gérard Lorimier, Caroline Malhaire9, Frédéric Marchal10, Pascale Mariani9, Emilie Mathiotte, Pierre Meeus, Eliane Mery, Simon Msika, Cédric Nadeau11, Pablo Ortega-Deballon, Guillaume Passot6, Olivier Pellet, P. Peyrat, Nicolas Pirro, Marc Pocard, Flora Poizat, Jack Porcheron, Anaïs Poulet6, Patrick Rat, Pierre Rousselot, P. Rousset6, Hélène Senellart, Martine Serrano6, Vincent Servois9, Olivia Sgabura, Andrea Skanjeti6, Magali Svrcek, Raphaël Tetreau, Emilie Thibaudeau, Yann Touchefeu, Jean-Jacques Tuech, Séverine Valmary-Degano, Delphine Vaudoyer6, Stéphane Velasco, Véronique Verriele-Beurrier, Laurent Villeneuve, Romuald Wernert, Franck Zinzindohoué 
01 May 2020-Ejso
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.

References
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Journal ArticleDOI
TL;DR: A case of the chondroid variant of chordoma is presented and the differentiating features of this entity are discussed on a clinical and histopathologic basis.
Abstract: A case of the chondroid variant of chordoma is presented. The differentiating features of this entity are discussed on a clinical and histopathologic basis. A review of the topic of chordoma is also provided.

1,126 citations


Journal ArticleDOI
TL;DR: The finding that at least 60% of serous borderline tumours harbour mutations in two members of the ERK‐MAP‐kinase pathway compared with 12% of high‐grade serous carcinomas (BRAF 0%, KRAS 12%) indicates that the majority of serious borderline tumoured tumours do not progress to serious carcinomas.
Abstract: Genes of the RAF family, which mediate cellular responses to growth signals, encode kinases that are regulated by RAS and participate in the RAS/RAF/MEK/ERK/MAP-kinase pathway. Activating mutations in BRAF have recently been identified in melanomas, colorectal cancers, and thyroid and ovarian tumours. In the present study, an extensive characterization of BRAF and KRAS mutations has been performed in 264 epithelial and non-epithelial ovarian neoplasms. The epithelial tumours ranged from adenomas and borderline neoplasms to invasive carcinomas including serous, mucinous, clear cell, and endometrioid lesions. It is shown that BRAF mutations in ovarian tumours occur exclusively in low-grade serous neoplasms (33 of 91, 36%); these included serous borderline tumours (typical and micropapillary variants), an invasive micropapillary carcinoma and a psammocarcinoma. KRAS mutations were identified in 26 of 91 (29.5%) low-grade serous tumours, 7 of 49 (12%) high-grade serous carcinomas, 2 of 6 mucinous adenomas, 22 of 28 mucinous borderline tumours, and 10 of 18 mucinous carcinomas. Of note, two serous borderline tumours were found to harbour both BRAF and KRAS mutations. The finding that at least 60% of serous borderline tumours harbour mutations in two members of the ERK-MAP-kinase pathway (BRAF 36%, KRAS 30%) compared with 12% of high-grade serous carcinomas (BRAF 0%, KRAS 12%) indicates that the majority of serous borderline tumours do not progress to serous carcinomas. Furthermore, no BRAF mutations were detected in the other 173 ovarian tumours in this study.

245 citations


"Psammocarcinoma of ovary with serou..." refers background in this paper

  • ...Sieben NL, Macropoulos P, Roemen GM, Kolkman-Uljee SM, Jan Fleuren G, Houmadi R, Diss T, Warren B, Al Adnani M, De Goeij AP, Krausz T, Flanagan AM: In ovarian neoplasms, BRAF, but not KRAS, mutations are restricted to low-grade serous tumours....

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  • ...The molecular features of psammocarcinoma include mutations of a gene belong to the cancer related RAF family, that is BRAF [19]....

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Journal ArticleDOI
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.

114 citations


Journal ArticleDOI
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.
Abstract: Background: Psammocarcinoma is an unusual variant of serous cystadenocarcinoma characterized by heavy deposits of psammoma bodies. This disease has been suggested to be similar to carcinomas of low malignant potential in its indolent clinical course. We present this case report of an aggressive course of this disease to alert others that psammocarcinoma may not always follow a benign course. Case: A 66-year-old woman underwent staging laparotomy for bilateral ovarian cystadenofibromata with rare foci of borderline serous tumors and several small bowel peritoneal surface nodules showing infiltrating psammocarcinoma. She was not recommended for adjuvant therapy because of the previously reported indolent course of this disease. Eighteen months later she represented with small bowel obstruction and underwent an exploratory laparotomy that demonstrated diffuse recurrence of the psammocarcinoma. Conclusion: Psammocarcinoma may have a more aggressive course than has been suggested. Patients with this disease should have optimal tumor debulking. There may be a role for adjuvant therapy in its treatment.

30 citations


"Psammocarcinoma of ovary with serou..." refers background in this paper

  • ...Literature search showed presence of only 28 cases of psammocarcinoma of ovary reported till date (Table 1) [1-17]....

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  • ...In the case reported by Poggi et al [5] no adjuvant therapy was given because of the supposed indolent Computed tomography scan shows calcified abdominopelvic mass (a); gross photograph shows gritty and firm tumor (b); on histological examin tion, tumor reveals extensive psammoma bodies which are surrounded by ingle layer of cytologically bland cub idal or l w colum ar epit elium (c-f) H&E cx40; dx100; ex400; fx600Figure 1 Computed tomography scan shows calcified abdominopelvic mass (a); gross photograph shows gritty and firm tumor (b); on histological examination, tumor reveals extensive psammoma bodies which are surrounded by single layer of cytologically bland cuboidal or low columnar epithelium (c-f) H&E cx40; dx100; ex400; fx600....

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  • ...The right ovary measured 7 × 6 × 2 cms. Cut Page 1 of 6 (page number not for citation purposes) P ag e 2 of 6 (p ag e nu m be r n ot fo r c ita tio n pu rp os es ) Follow up Remarks 1-died 3- lost FU 4- free of disease 42 months NED Adolescent NED 12 months NED Family history of epithelial cancer positive Recurrence 18 months Aggressive, with Cystadenofibromata 2 years NED Omental and peritoneal implant NED after 1 year Bilateral, omental nodule showed the features of invasive implant Recovered Bilateral with cysadenofibromata NA Aggressive NED 4 months stable Jo ur na l o f M ed ic al C as e R ep or ts 2 00 9, 3 :9 33 0 ht tp :// w w w .jm ed ic al ca se re po rts .c om /c on te nt /3 /1 /9 33 0 Table 1: Literature review of primary ovarian psammocarcinomas Author Year No. of cases Age Clinical features CA- 125 units/ml FIGO stage Surgery Chemothearpy Gilks et al [1] 1990 8 36 to 76 (mean of 57) Abdominal pain NA III TAH+ BSO (4 cases) LSO(2 cases) BSO(2 cases) Oment (3/8 cases) Y (1 patient) Kelley et al [2] 1995 1 18 Abdominal pain 25 IIIC TAH, BSO, oment, Y Pakos et al [3] (German) 1997 1 49 Mass in the lower abdomen IA BSO N Powell et al [4] 1998 1 59 Abdominal pain and increasing abdominal girth 118 IIIB TAH, BSO, oment N Poggi et al [5] 1998 1 66 Abdominal Pain nausea vomiting NA IIIB BSO, oment N Cobellis et al [6] 2003 1 48 Referred for leiomyomata uteri Normal IIIA TAH, BSO, oment N Giordano et al [7] 2005 1 66 Abdomino-pelvic mass Elevated IIIB TAH, BSO, oment, Y Rattenmaier et al [8] 2005 1 70 Malaise and abdominal discomfort 25,000 NA AH, BSO N Radin et al [9] 2005 1 60 Diffuse abdominal pain, bloating, diarrhea, and low back pain, 65.2 III Laparotomy, tumor debulking Y Vimplis et al[10] 2006 1 63 Abdominal discomfort and increasing abdominal girth 1,133 IIIB BSO,, SH, oment, Y Hiromura et al [11] 2007 1 73 Lower abdominal distention and pain 464 IIIC AH, BSO, and oment Y of 6 (p ag e nu m be r n ot fo r c ita tio n pu rp os es ) Jo ur na l o f M ed ic al C as e R ep or ts 2 00 9, 3 :9 33 0 ht tp :// w w w .jm ed ic al ca se re po rts .c om /c on te nt /3 /1 /9 33 0 Journal of Medical Case Reports 2009, 3:9330 http://www.jmedicalcasereports.com/content/3/1/9330 section was solid and cystic with cysts ranging in size from 0.4 to 0.5 cm....

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  • ...Earlier, association of psammocarcinoma with cystadenofibroma has been documented [5,8]....

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  • ...In the case reported by Poggi et al [5] no adjuvant therapy was given because of the supposed indolent...

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