Psammocarcinoma of ovary with serous cystadenofibroma of contralateral ovary: a case report
© Jain et al; licensee BioMed Central Ltd. 2009
Received: 19 September 2009
Accepted: 15 December 2009
Published: 15 December 2009
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.
We herein report a case of psammocarcinoma of ovary with serous cystadenofibroma of contralateral ovary in a 55 year old Asian Indian female.
To the best of author's knowledge, ours is the rare case describing coexistence of this very rare malignant serous epithelial tumor with a benign serous cystadenofibroma of contralateral ovary.
Literature review of primary ovarian psammocarcinomas
CA- 125 units/ml
Gilks et al 
36 to 76 (mean of 57)
TAH+ BSO (4 cases)
Oment (3/8 cases)
Y (1 patient)
3- lost FU
4- free of disease
Kelley et al 
TAH, BSO, oment,
42 months NED
Pakos et al  (German)
Mass in the lower abdomen
Powell et al 
Abdominal pain and increasing abdominal girth
TAH, BSO, oment
12 months NED
Family history of epithelial cancer positive
Poggi et al 
Abdominal Pain nausea vomiting
Recurrence 18 months
Aggressive, with Cystadenofibromata
Cobellis et al 
Referred for leiomyomata uteri
TAH, BSO, oment
2 years NED
Omental and peritoneal implant
Giordano et al 
TAH, BSO, oment,
NED after 1 year
Bilateral, omental nodule showed the features of invasive implant
Rattenmaier et al 
Malaise and abdominal discomfort
Bilateral with cysadenofibromata
Radin et al 
Diffuse abdominal pain, bloating, diarrhea, and low back pain,
Laparotomy, tumor debulking
Vimplis et al
Abdominal discomfort and increasing abdominal girth
BSO,, SH, oment,
Hiromura et al 
Lower abdominal distention and pain
AH, BSO, and oment
4 months stable
Akbulut et al 
Vaginal bleeding and abdomino-pelvic pain
10 years with recurrent and metastatic disease
Pusiol et al 
10.5 years, free of disease.
Bilateral, psammoma bodies in cervical smear, Presence of psammocarcinoma
in the tubaric lumen
Alanbay et al 
Adnexal mass, pelvic pain
6 years free of disease, 2 months
Tiro et al 
Shortness of breath
Implants in pleural cavity and pericardium
Chase et al 
Mediastinal, pulmonary, subcutaneous, and omental metastases
Poujade et al 
Y except in one case
18-45 months NED, one case has persistent disease
Menorrhagia, abdominal discomfort and pain
Total abdominal hysterectomy with bilateral salpingo oophorectomy
6 months, free of disease
Psammocarcinoma of ovary is a rare serous neoplasm which can arise from both peritoneum and ovaries . Extensive search showed presence of only 28 cases of psammocarcinoma of ovary reported till date [1–17] (Table 1). Age ranged from 18 to 76 years [1, 2]. Many of these cases presented with abdominal discomfort and increasing abdominal girth.
This tumor can be misdiagnosed as other calcifying tumors of abdomino-pelvic region especially calcified leiomyomas, by radiological investigations, similar to our case. However if calcified abdomino-pelvic tumors are seen in association with an elevated CA-125 level, then a diagnosis of ovarian neoplasm can be made . In most of the reported cases CA-125 levels were raised ranging from 65-25,000 units/ml [7, 8]. Our patient had elevated levels of CA-125 just after the surgery.
All but one, cases reported in the literature had FIGO stage III tumors showing clinical behavior similar to that of borderline serous tumors . Our case fits in to stage I C as patient had malignant ascitis, however no pelvic extension was seen.
Despite the apparent low malignant potential of this tumor, there remains a need for patient's follow up data as aggressive behavior has been described in the literature [12, 15, 16]. In the case reported by Poggi et al  no adjuvant therapy was given because of the supposed indolent behavior. However, recurrence of disease has been noted after eighteen months. Our patient received post operative adjuvant therapy and is on follow up.
Histopathologic differential diagnosis of psammocarcinoma involves other serous epithelial tumors. Some rare cystadenofibromas have mild cytologic atypia and may also exhibit psammoma bodies, but they are generally inconspicuous. In the present case we have seen cystadenofibroma in the contralateral ovary with an occasional psammoma body formation. Earlier, association of psammocarcinoma with cystadenofibroma has been documented [5, 8]. Although these tumors show clinical behavior similar to that of borderline serous tumors, the presence of destructive stromal invasion excludes serous borderline tumors. Moderate cytologic atypia helps differentiating from high grade serous carcinomas. Psammocarcinomas differ from well-differentiated serous adenocarcinomas by numerous psammoma body formations.
Potential mechanisms responsible for the characteristic, extensive psammoma body formation include the accumulation of successive layers of calcium on single necrotic or degenerated tumor cells. One hypothesis states that they arise due to accumulation of hydroxyapatite in degenerating cells . Psammoma bodies are described as multiple, discrete, laminated calcified bodies and are found in many neoplastic and non neoplastic lesions. Characteristic calcifications on radiological investigations and psammoma bodies on cervicovaginal smears should alert the clinician for this unusual tumor. Recently Pusiol et al  have reported a case of psammocarcinoma associated with presence of psammoma bodies in the cervicovaginal smears.
The molecular features of psammocarcinoma include mutations of a gene belong to the cancer related RAF family, that is BRAF .
Hiromura et al  described imaging features of psammocarcinoma including extensive minute calcifications on CT scan that were not detected on the abdominal x-ray. The tumor appears sandy and coarsely granular on enhanced T1-weighted MR images due to scattered clusters of psammomatous calcifications. Radin et al  have shown its high avidity for the Tc-99m MDP radiopharmaceutical.
Like with other serous epithelial tumors of the ovary, aggressive debulking surgery has been the initial treatment modality in nearly all cases. Postoperative therapies have included observation, tamoxifen and cytotoxic chemotherapy (generally using cyclophosphamide with cisplatin or carboplatin) .
Psammocarcinomas are rare serous neoplasms. Due to the rare aggressive nature of the tumor, it is important to follow up the patient closely. Our case adds a new case of psammocarcinoma with contralateral cysadenofibroma in the literature.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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