Psammocarcinoma of the ovary: a case report and review of the literature

Psammocarcinoma is a rare form of serous carcinoma of the ovary, associated with extensive psammoma body formation and invasion of surrounding structures. We report the case of a 63-year-old woman who presented with a highly calcified abdominopelvic mass and a raised CA-125 marker and who, following a full staging laparotomy, was diagnosed with stage IIIB ovarian psammocarcinoma. Serous ovarian psammocarcinoma is characterized by massive psammoma body formation, and despite the limited number of cases reported, it appears that the clinical prognosis is much more favorable than for the usual serous carcinomas and is similar to that of serous borderline lesions of the ovary. A summary of all the reported cases is provided to highlight the clinical and prognostic features of this rare tumor.     

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

Psammocarcinoma is a rare form of serous ovarian and peritoneal carcinoma, characterized by abundant psammoma bodies, invasiveness, and low-grade cytological features. Many of the cases which have been reported had extraovarian spread and peritoneal seeding at the time of presentation. We present a case of 45-year-old woman with ovarian psammocarcinoma who initially presented with a metastatic subcutaneous nodule, and was found to have pulmonary and mediastinal metastases. We believe this to be the first report of a psammocarcinoma with these metastases. Ovarian and peritoneal psammocarcinomas are quite rare, and because of this, knowledge of their behavior is limited. Although most seem to follow an indolent course similar to that of borderline lesions of the ovary, this case demonstrates that some of these tumors may be clinically aggressive with distant metastases.     

Primary peritoneal psammocarcinoma: A case presenting with an upper abdominal mass and elevated CA-125.

Primary peritoneal serous adenocarcinoma with predominating psammoma bodies, psammocarcinoma, is a very rare tumor with only seven cases documented in the English literature. Pathological classification of this entity was established in 1990 and clinical behavior of this tumor is uncertain. Based on limited data these tumors appear to behave similarly to low malignant potential tumors of the ovary. This case describes a 59-year-old woman who underwent exploratory laparotomy for a large upper abdominal cystic mass. Findings included a large tumor mass involving the gastrocolic omentum and dense small bowel adhesions. The patient had normal ovaries and was debulked to no macroscopic disease. Final pathologic diagnosis confirmed a stage IIIC primary peritoneal psammocarcinoma. The patient has received no adjunctive therapy and is without evidence of disease 2 years after surgery. Primary peritoneal psammocarcinoma is a neoplasm which can mimic serous adenocarcinoma of the ovary. In contrast, primary peritoneal psammocarcinoma appears to behave in an indolent fashion. Primary surgical debulking should be attempted, while the utility of postoperative chemotherapy remains unknown.     

Primary psammocarcinoma of the peritoneum

Abstract.   Bılgın T, Özuysal S, Cankiliç H. Primary psammocarcinoma of the peritoneum. Int J Gynecol Cancer 2006; 16(Suppl. 1): 129–131.
Psammocarcinoma is a rare type of serous carcinoma arising from the ovaries or the peritoneum. Histopathologically, it has large deposits of psammoma bodies, invasion to surrounding tissues, and low-grade cytologic atypia. Biologic behavior is similar to borderline serous tumors with a favorable survival time. Primary surgical debulking with complete resection of the tumor should be the main surgical approach, while the benefit of postoperative chemotherapy remains unknown. A new case of primary peritoneal psammocarcinoma with survival after an initial diagnosis of 5.5 years is presented with a literature review.     

Serous psammocarcinoma of the ovary: a case report and review of literature

BACKGROUND: Serous psammocarcinoma is a form of ovarian carcinoma, characterized by massive psammoma body formation, invasiveness, and low-grade cytological features. We reported a new bilateral case of serous psammocarcinoma. We also reviewed the literature in order to delineate clinical, pathological, and prognostic features of this rare neoplasm. CASE HISTORY: A 66-year-old white woman was admitted to our Institution for a voluminous abdomino-pelvic mass, which was suspected to be a leiomyoma of the uterus. Abdominal computed tomography scan revealed a heavily calcified abdominopelvic mass with cystic areas. The origin of this lesion was unclear in diverse scanning of computed tomography. Elevated serum CA-125 levels suggested the eventuality of an ovarian neoplasm. Thus, the patient underwent an exploratory laparotomy. Intraoperatory findings showed two ovarian calcified lesions. The surface of omentum was covered with a white nodule. Pathological findings were consistent with serous psammocarcinoma with invasive implant of the omentum. CONCLUSION: Serous psammocarcinoma is a rare form of ovarian carcinoma with only 13 cases reported in literature. Patient's age can range from 18 to 76 years. Prognostic factors suggest that this neoplasm has more favorable prognosis than usual serous carcinomas. It is important to differentiate these two neoplasms and other pelvic mass, such as calcified leiomyoma.     

Clear cell carcinoma of the ovary: a clinicopathologic analysis of 34 cases

Abstract. The clinical and pathologic aspects of 34 cases of pure clear cell carcinoma of the ovary are discussed. These tumors represented 12.1% (34/280) of all ovarian cancers. The ages of the patients ranged from 34 to 78 years (mean 51.6 years) and 59% were postmenopausal. The clinical stages (FIGO) of these patients were as follows; I 22, II 5, III 6, and IV 1. Thirty-nine percent of the patients were nulligravidas. The diameters of the tumor varied from 6 to 31 cm (mean 16 cm), and 47% of the patients had endometriosis. Thirteen tumors were directly connected with endometriosis and this suggests that some ovarian clear cell carcinoma arose from the epithelium of an endometriotic cyst. Three architectural patterns (solid, papillary, and tubulocystic) were recognized. Forty-seven percent of the tumors were predominantly papillary and 24% contained mixtures of these three patterns. Four cell types such as clear, hobnail, eosinophilic, and flattened were also seen. Thirty-eight percent of the tumors had psammoma bodies. Histological patterns and cell types were not prognostic factors, and the stage at presentation was the most important prognostic factor. The 5-year survival rate was 54% (7/13) which was better than that in patients with serous adenocarcinoma of the ovary. The estimated survivals in patients with clear cell carcinoma however, were worse than those in patients with serous adenocarcinoma when compared stage for stage.     

Advanced-stage serous borderline tumors of the ovary: a clinicopathological study of 49 cases.

There is controversy about patient outcomes and pathological parameters of prognostic significance in patients with stage II or stage III ovarian serous borderline tumors. Forty-nine cases of stage II and III ovarian serous borderline tumors were identified on review of the medical records at Vancouver Hospital and British Columbia Cancer Agency for the period from 1979 to 1996. Pathological features assessed included presence of micropapillary architecture, tumor cell DNA content (ploidy), and characteristics of the extraovarian implants, including invasiveness and mitotic activity. Clinical follow-up information (3-17 years of follow-up) was obtained for 48 patients. Fifteen patients had stage II tumors and 34 had stage III tumors. Fourteen patients experienced tumor recurrence 1 to 8 (mean 3.5) years after presentation and of these, six patients died of disease (2, 3, 4, 7, 10, and 11 years after presentation). Patients with gross residual disease, as assessed by the surgeon, more frequently experienced a recurrence compared with patients without gross residual disease, but this difference did not reach statistical significance (0.05相似文献   

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

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.     

Low malignant potential tumors of the ovary: a study of 76 cases

This study analyzes the clinical and pathological features of 76 patients who were diagnosed as having ovarian low malignant potential tumor over a 20-year period: 39 (51.3%) of the tumors were mucinous, 29 (38.2%) serous, three (3.9%) endometrioid, and five (6.6%) mixed. Patients with serous tumors were significantly younger (mean age 40 years) than those with mucinous tumors (mean age 50.5 years). Serous tumors were more frequently bilateral (48.3%) than mucinous tumors (12.8%). The extent of tumor (FIGO stage) at the primary laparotomy was related to the prognosis: the survival of 14 patients with stage III and six patients with stage II tumor was significantly inferior to that of the 56 patients with stage I tumors (P less than .01). Patients with stage III mucinous low malignant potential tumors and pseudomyxoma peritoneii fared badly. Four patients with stage II and 13 patients with stage III tumors had residual tumor after primary surgery. Five of these patients received no subsequent treatment of whom four are alive and well from 5.5 to 19 years after diagnosis. Three patients received pelvic radiotherapy. Eleven patients were treated with chemotherapy for residual or recurrent tumor but only one (9.1%) had an unequivocal response.     

卵巢上皮性肿瘤的临床病理特征及其细胞周期素D1和p53蛋白表达的研究

目的研究卵巢上皮性癌（卵巢癌）和交界性上皮性肿瘤的临床病理特征及其细胞周期素D1（cyclin D1）和p53蛋白表达的情况,探讨卵巢癌和交界性上皮性肿瘤在发病机制上的联系。方法分析45例卵巢癌（卵巢癌组）和54例卵巢交界性上皮性肿瘤（交界性肿瘤组）的临床病理资料,采用免疫组化法检测两组组织中cyclin D1、p53蛋白的表达情况,并分析其与临床病理特征的相关性。结果（1）临床病理特征：①年龄：交界性肿瘤组平均年龄为42．5岁（14～82岁）,中位数年龄41岁;卵巢癌组平均年龄为53．5岁（26～80岁）,中位数年龄51岁。②分期：按国际妇产科联盟（FIGO）分期标准,交界性肿瘤组Ⅰ期48例、Ⅱ期3例、Ⅲ期3例;卵巢癌组Ⅰ期6例、Ⅱ期8例、Ⅲ期26例、Ⅳ期5例。③病理类型：交界性肿瘤组以黏液型为主[占56％（30／54）],其次为浆液型[其中普通型11例,微乳头型5例;占30％（16／54）];卵巢癌组以浆液型（其中低度恶性19例,高度恶性3例）为主[占49％（22／45）]。④病理分化程度：卵巢癌组高分化5例,中分化17例,低分化或未分化23例。⑤预后：交界性肿瘤组5年生存率为98％,卵巢癌组为51％,两组比较,差异有统计学意义（P=0．000）。（2）cyclin D1和p53蛋白的表达及其与卵巢癌和交界性肿瘤临床病理特征的相关性：卵巢癌组cyclin D1和p53蛋白的阳性表达率分别为31％（14／45）和56％（25／45）,p53蛋白表达强度与病理分化程度呈正相关（r=0．320,P=0．032）;交界性肿瘤组cyclin D1和p53蛋白的阳性表达率分别为69％（37／54）和6％（3／54）。其中,普通型浆液性交界性肿瘤与高度恶性浆液性癌比较（两者cyclin D1蛋白阳性表达率分别为91％和26％,p53蛋白分别为0和58％）,差异有统计学意义（P〈O．01）;而微乳头型浆液性交界性肿瘤与低度恶性浆液癌比较（两者cyclin D1蛋白阳性表达率分别为3／5和2／3,p53蛋白分别为1／5和1／3）,差异则无统计学意义（P〉0．05）。结论cyclin D1蛋白的过度表达常见于卵巢浆液性交界性肿瘤及低度恶性浆液性癌组织中,而p53蛋白的过度表达更多见于高度恶性浆液性癌组织中。卵巢浆液性交界性肿瘤与高度恶性浆液性癌具有不同的发病机制,而微乳头型浆液性交界性肿瘤与低度恶性浆液性癌的关系可能更为密切。     

Ovarian epithelial tumors of borderline malignancy in Japan

Seventy-one cases of ovarian epithelial tumor of borderline malignancy have been surveyed from a clinicopathologic viewpoint. The majority of the borderline tumors (73.2%) were of the mucinous type, versus only 16.9% of the serous type. The other types--endometrioid (2.8%), Brenner (1.4%), and mixed type (5.6%)--were much rarer. Patients with mucinous tumors were significantly younger (mean age 42.6) than those with serous tumors (mean age 57.5) (P less than 0.01). Of patients with mucinous tumors, 64.2% were of reproductive age (15-45 years), compared with 17% of patients with serous tumors. Some 78.8% of mucinous and 83.3% of serous borderline tumors were FIGO stage I. Serous tumors were more frequently bilateral (66.7%) than mucinous tumors (9.8%). In mucinous borderline tumors, the extent of tumor spread at the first laparotomy had an intimate relationship to the prognosis, but in serous borderline tumors, it was insignificant. The survival rate of patients with mucinous borderline tumors was 69.3% at 5 years and 62.4% at 10 years (Kaplan-Meier method). Most patients with pseudomyxoma peritonei classified as borderline at the time of discovery died within 5 years of the operation. The prognosis of the serous type was extremely favorable. Criteria for borderline tumors of various cell types and differences in the clinicopathologic data of ovarian borderline tumors between Japan and other countries were discussed.     

Serous papillary cystadenoma of borderline malignancy of broad ligament. A report of 25 cases

The clinical and pathological features of 25 serous papillary cystadenomas of borderline malignancy of the broad ligament were analyzed. The ages of the patients ranged from 19 to 67 (average, 32) years. The clinical presentation was lower abdominal pain, pelvic pain or both in five cases, accompanied by menometrorrhagia or amenorrhea in three cases. One patient was thought to have an acute abdominal disorder. The tumors of the remaining 19 patients were discovered either on routine gynecological examination or during an evaluation of the pregnancy status of the patient. In 14 cases the tumor was located in the left broad ligament and in 11 cases it was on the right side; all the tumors were entirely separated from the ipsilateral ovary. On gross examination the tumors were 1-13 cm in greatest dimension, had smooth outer surfaces, and contained straw-colored, watery fluid. The inner lining bore single or multiple 0.3-2.5 cm excrescences. Microscopic examination revealed that the cyst walls and their excrescences were lined by simple to pseudostratified, cuboidal to columnar, focally ciliated epithelium. Slight nuclear atypism, very rare mitotic activity, and focal psammoma body formation were also found. The stroma resembled ovarian stroma but no primary follicles or follicular derivatives were identified. Twenty-three of the patients were alive and well from 0.5 to 11 years after excision of the tumor, one patient was disease-free for 8.5 years but died of an open-heart surgical procedure; and two patients were lost to follow-up examination.     

Hereditary ovarian cancer: a clinicopathological study.

Hereditary ovarian cancer (HOC) is rare and little recognized. Over the years, we have identified 37 HOC patients from HOC syndrome kindreds with documented cancers of ovary, breast, colon, or endometrium in two or more first-degree relatives. The age and clinical stage at diagnosis and overall 5-year survival of HOC patients were compared with those of ovarian cancers in the unselected patients. The gross and microscopic features of the tumors are compared with a set of 34 consecutively chosen ovarian cancer cases with documented negative family histories. The mean age of HOC patients at diagnosis was significantly lower (50.2 years) than that of the unselected control population (59 years) (p less than 0.001). Detailed pedigree analysis breaks down the HOC group into (a) site-specific ovarian cancer, 5 cases, 56.4 years mean age; (b) breast-ovarian cancer syndrome, 28 cases, 50.46 years mean age; and (c) Lynch syndrome II (colon/endometrial cancer), 4 cases, mean age 41 years. The age differences were statistically significant (p = 0.050). The most prevalent International Federation of Gynecology and Obstetrics clinical stage at diagnosis of HOC (stage III) was the same as for the control group. Histologically, all (100%) HOC tumors were surface epithelial cancers with predominance of serous papillary type moderate to high grade (89 versus 71% in control, p = 0.07). No other pathologic features appeared to be significant. In conclusion, HOC is a serous papillary tumor and characterized by early age of onset and excess of breast/ovary/colon-endometrial cancers in first-degree relatives of patients with specific HOC syndromes.     

Pathologic findings in eight cases of ovarian serous borderline tumors, three with foci of serous carcinoma, that preceded death or morbidity from invasive carcinoma.

We sought to assess the frequency of previously reported adverse histopathologic findings in ovarian serous borderline tumors (SBTs) in cases that preceded a patient's death or caused serious morbidity due to invasive carcinoma. SBTs with foci of invasive carcinoma that occupied a minority of the tumor and were associated with similar outcomes were also studied for potential additional insights. Eight cases were found over a 22-year period. Ten tumors in 5 patients were purely SBT; at initial staging, 1 patient had invasive peritoneal implants; 3 had noninvasive peritoneal implants; 1 was stage I. At last follow-up 3 of the 5 patients had died of carcinoma, 1 was alive with carcinoma, and 1 had no clinical evidence of disease 4 years after a sigmoid colectomy for invasive serous carcinoma of the bowel wall. Four tumors in 3 patients had foci of invasion that were more than microinvasive; at initial staging, all 3 patients had invasive peritoneal implants, and all died of carcinoma. All 14 of the ovarian tumors in the 8 cases had surface involvement by tumor cells, and in 8 tumors in 5 cases they were confined primarily to the ovarian surface. Foci of "micropapillary serous carcinoma" accompanied more obvious areas of infiltrative carcinoma in 2 of the 4 ovarian tumors, the peritoneal implants in 1 of the cases with purely SBTs, and a recurrence in this case and 1 other case. No morphologic finding in the 10 purely SBTs was predictive of subsequent malignant behavior. We conclude that extraovarian invasive serous carcinomas, either following or concurrent with an ovarian SBT, develop from borderline foci that may originate in the ovary, but frequently are likely to have arisen independently in the peritoneum. The carcinomas may be preceded or accompanied by noninvasive-appearing micropapillary foci in the peritoneum in some cases, but micropapillary foci in the ovarian tumors are infrequent and not a necessary antecedent.     

Serous and mucinous borderline tumors of the ovary: a clinicopathologic and DNA-ploidy study of 102 cases

One-hundred and two patients with epithelial borderline ovarian tumors treated at Tampere University Hospital between January 1965 and September 1991 were evaluated. There were 48 patients with serous tumors and 54 with mucinous tumors. Ninety-three (91%) patients had clinical stage I and nine had stage III disease. Abdominal hysterectomy and bilateral salpingo-oophorectomy were performed in 70% of the patients. Forty percent of those with serous and 20% with mucinous tumors were operated conservatively. After conservative surgery six patients had a total of 10 deliveries and none of these had a recurrence. Seven patients received chemotherapy, none had radiation therapy. An aneuploid DNA pattern was identified in 8.2% of 85 specimens studied and a high S-phase fraction was found in 8.6% of 81 specimens studied. DNA measurement failed to identify the malignantly behaving tumors. Elevated preoperative serum levels of CA125 were found in 10 (63%) out of 16 cases studied. All of them dropped postoperatively to normal. During the follow-up period (mean 11.6 years, range 4.5–29.7 years) 22 patients died but in 17 of them death was unrelated to ovarian tumor. The corrected (borderline malignancies related) 5-year survival rate was 100% in patients with serous tumor and 96% with mucinous tumor, 25-year survival rate was 97% and 91%, respectively. There was no difference between the serous and mucinous groups. Our results show that ovarian borderline tumors have a good prognosis. Quite conservative therapy is often enough, especially in low-stage disease in young women who want to retain their fertility.     

Prognostic importance of psammoma bodies in adenocarcinomas of the ovaries

Microcalcifications called psammoma bodies are a common finding in serous papillary adenocarcinomas of the ovary. In a series comprising 501 cases of ovarian carcinoma the prognostic importance of the presence of these calcifications is analyzed. Psammoma bodies are confined mainly to serous papillary adenocarcinomas, particularly stage III. Using a life-table technique for survival analysis, it was not possible to demonstrate a significant difference in survival rates in cases with and without such calcifications, although the histological type, tumor stage, and grade, all known to be of prognostic importance, are kept constant. Supportive evidence favors the theory that psammoma body formation is a consequence of dystrophic calcification associated with ischemia rather than tumor-host hypersensitivity-related cell death.     

An assessment of pathologic features and treatment modalities in ovarian tumors of low malignant potential

Sixty-eight patients with epithelial ovarian tumors of low malignant potential treated at the University of Michigan Medical Center were reviewed for clinical and pathologic features related to recurrence or death. The ovarian tumor of low malignant potential represented 12.6% of all ovarian cancers and 22% of all serous or mucinous tumors. Thirty-four patients were stage I (50%), 13 were stage II (19%), 17 were stage III (25%), two patients could not be staged, and two patients developed ovarian tumor of low malignant potential in a residual ovary. The risk of recurrence was significantly related to stage III disease (P = .023), high nuclear atypia (P = .020), and high grade (P = .017); and was unrelated to capsular status, the presence of psammoma bodies, nucleoli, cribriform pattern, stratification, cystadenofibroma, tumor size, or spillage at surgery. Therapy in all stages included observation, chemotherapy, or radiotherapy. There was one recurrence in 47 patients with stages I-II, and 11 recurrences in 17 patients with stage III disease. The ovarian tumor of low malignant potential carries an extremely favorable prognosis in stage I and II regardless of therapy. Radiotherapy appeared to extend disease-free survival in stage III disease, and future randomized studies should consider this treatment modality.     

Primary peritoneal serous psammocarcinoma with recurrent disease and metastasis: a case report and review of the literature

BACKGROUND: Psammocarcinoma is a rare variant of serous carcinoma arising from either ovary or peritoneum. Although behavior of tumor is uncertain, it has been suggested to be similar to serous carcinomas of low malignant potential. CASE: A 67-year-old woman was admitted with an abnormal vaginal bleeding and abdomino-pelvic pain. Computed tomography showed a heavily calcified rectovaginal mass that was histologically characterized by numerous psammoma bodies and low-grade cytological features. Following the primary surgery, the patient received 9 cycles of chemotherapy. Five years later, she presented with diffuse recurrence and 1 year later, with metastasis to the vertebra. CONCLUSION: Although psammocarcinoma is known to behave in a more indolent course, clinicians should be aware that patients with this disease may have a clinically aggressive, recurrent, and metastatic tumor that necessitated systemic therapy.     

Aggressive ovarian psammocarcinoma: a case report

Serous psammocarcinoma is a rare form of ovarian carcinoma characterized by massive psammoma body formation. We report a new case of aggressive ovarian psammocarcinoma with omental and peritoneal implants.     

Uterine papillary serous carcinoma: A study on 108 cases with emphasis on the prognostic significance of associated endometrioid carcinoma, absence of invasion, and concomitant ovarian carcinoma

One hundred eight cases of uterine papillary serous carcinoma (UPSC) were analyzed to characterize its histologic features and biologic behavior. Special situations that could conceivably modify the behavior and therapeutic approaches were considered: (1) the occurrence of areas of endometrioid carcinoma in otherwise typical UPSC; (2) the confinement of UPSC to an otherwise benign endometrial polyp or the endometrial mucosa or absence of residual tumor at the time of hysterectomy; and (3) the coexistence of a superficial UPSC and a serous ovarian carcinoma. There was coexistence of endometrioid and UPSC in 22 cases, and tumor was confined to an endometrial polyp or endometrium in 19 cases. There was simultaneous pathologic stage I UPSC and papillary serous ovarian carcinoma in 10 cases. In patients with pathologic stages I and II UPSC the presence of areas of endometrioid carcinoma intermixed with the UPSC did not improve survival. Patients with stage I disease and no residual tumor or tumor confined to an endometrial polyp/endometrial mucosa and without vascular invasion had a survival not statistically different from those with stage I disease but with myometrial and/or vascular invasion. Patients with stage I UPSC with concomitant ovarian serous surface papillary carcinoma had survival not statistically different from patients with stage IV UPSC.