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OJHAS Vol. 9, Issue 2:
(2010 Apr-Jun) |
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| Ovarian Fibroma with Meigs Syndrome associated with
Elevated CA125 - A Rare Case |
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Prasad K Shetty, UD Bafna, K Balaiah, Gnana Prakash S,
Departments of Pathology, Gynaconcology and Radiology,
Bhagwan Mahaveer Jain Hospital, Bangalore, India. |
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Address For Correspondence |
Surgical Pathologist,
Bhagwan Mahaveer Jain Hospital,
Millers Road, Vasanth Nagar,
Bangalore - 560052, INDIA.
E-mail:
dr.pkshetty@gmail.com |
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Shetty PK, Bafna UD, Balaiah K, Gnana Prakash S. Ovarian Fibroma with Meigs Syndrome associated with
Elevated CA125 - A Rare Case. Online J Health Allied Scs.
2010;9(2):17 |
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Submitted: May 3,
2010; Accepted: Jul 15, 2010; Published: Jul 30, 2010 |
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| Abstract: |
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Postmenopausal
women with solid adnexal masses, ascites and pleural effusion with elevated
CA 125 are highly suggestive for malignant ovarian tumor. However in
literature 28 cases Meigs syndrome (Benign ovarian tumor, ascites and
right pleural effusion) with raised CA 125 have been reported. We report
a case of Meigs syndrome caused by right ovarian fibroma with elevated
serum CA125 level in a postmenopausal woman
Key Words: Ascites, Meigs
syndrome, Carcinoembryogenic Antigen
(CA) 125. |
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An
elevated serum Carcinoembryogenic antigen (CA) 125 level in association
with a ovarian mass, pleural effusion and massive ascites usually signifies
a ovarian malignancy in a post menopausal woman. Benign ovarian tumor
in association with high CA125 is quite rare and very few cases have
been reported in literature. We present such a case which mimicked ovarian
malignancy.
A 72 year-old
woman came with history of breathing difficulty, abdominal distention
since 1 year she also gives history of breathing difficulty and oliguria
since 1 month. She is a known case of Diabetes and Hypertension since
11 Yrs. On abdominal examination moderate ascites was noted with a palpable
and freely mobile mass in the left side abdomen crossing mid line and
extending to right iliac fossa, measuring 15x15 cms with irregular surface.
Chest X- ray revealed
moderate right pleural effusion.
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Figure 2: Abdominopelvic
plain CT showing huge isodense Left adnexal mass; moderate ascites noted in
peritoneal cavity |
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Figure 1: Chest X- ray showing Right pleural effusion |
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Abdominopelvic
plain Computer Tomography (CT) scan reveled a large isodense abdominopelvic
mass measuring 193x120mm probably arising from the left adnexa.
Hepatitis B surface antigen
was Positive 213.54 (<2 Normal) and serum CA 125 level was high 205.3U/ml
(<35U/ml normal). Pleural and ascitic tap fluid cytology reveled
low cellular fluid comprising of lymphocytes and mesothelial cells.
No evidence of malignancy.
A preoperative
diagnosis of left ovarian carcinoma was made and an exploratory laparotomy
with total abdominal hysterectomy and bilateral salpingo-oophorectomy,
pelvic lymphnodes dissection and omentectomy was performed.
Grossly uterus
with cervix measured 9x6 cms and was unremarkable, right ovary measured
2.5x1.3 cms, cut surface was unremarkable,
Left ovarian tumor measured 20x15 cms with a smooth outer surface and
fallopian tube running over it, tumor was gritty to cut and was solid,
lobulated, firm and uniformly gray white.
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Figure
3: Gross photography of uterus with left ovarian solid gray
white mass. |
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Figure
4: Cut section photograph of left ovary showing solidly
uniform gray white tumor. |
Pelvic lymphnodes
were 21 in number largest measuring 1.4cms across, Omentum measured
18x11 cms. Cut surface was Unremarkable.
On Microscopy
left ovary showed closely packed tumor cells arranged in storiform
pattern, cells show small spindle shaped nuclei. No evidence of atypia/
mitosis.
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Figure 5: Microscopy
40x shows tumor cells arranged in storiform pattern with spindle shaped nuclei |
We made a diagnosis
of Ovarian Fibroma. The postoperative period was uneventful and the
patient was discharged on the seventh postoperative day.
In 1934, Salmon
described the association of pleural effusion with benign pelvic tumors.
In 1937 Meigs and Cass brought out the significance of pleural effusion
and ascites in benign ovarian fibroma.[1] Meigs syndrome
is defined as the triad of benign ovarian tumor with ascites and pleural
effusion that resolves after resection of the tumor. Although Meigs
syndrome mimics a malignant condition, it is a benign disease and has
a very good prognosis. Ovarian fibroma is found in 2–5% of surgically
removed ovarian tumors, and Meig’s syndrome is observed in about 1%.[2] Ascites
is present in 10–15% of those with ovarian fibroma and pleural effusion
in 1%, especially with large lesion. [3,4] Meigs suggested
that irritation of the peritoneal surfaces by a hard, solid ovarian
tumor could stimulate the production of ascites. [5, 6]
The etiology
of pleural effusion is unclear. It is thought that the occurrence of
pleural effusion is secondary to the passage of ascitic fluid to the
pleural space through the diaphragm or diaphragmatic lymph vessels which
are more common on the right side.[7] The connection between
the pelvic tumor and ascites is confirmed by the rapid resolution of
abdominal and pleural fluid after removal of the tumor. CA125 antigen
is a glycoprotein expressed in the embryonic coelomic epithelium. The
antigen can also appear in many adult tissues such as the epithelium
of the fallopian tubes, endometrium, endocervix, and ovaries.[8]
In addition, it is found in mesothelial cells of the pleura, pericardium
and peritoneum. This tumor marker is found elevated in ovarian malignancies
and in some benign conditions such as endometriosis, peritonitis or
cirrhosis, particularly with ascites.[9] The coincidence
of Meigs syndrome with elevation of serum CA125 levels has been described
in the published literature in only 28 cases and in 15 cases the ovarian
tumor was fibroma. CA125 suggested that serum elevation of CA125 antigen
in patients with Meigs syndrome is caused by mesothelial expression
of CA125 rather than by fibroma. The mechanism is unclear, but a mechanical
irritation from a large tumor, or an increase in intraperitoneal pressure
from a large volume of ascites might be primary factors in this process.[10]
In conclusion
the association of massive abdominal ascites, pleural effusion, and
a large pelvic mass with an elevated serum CA125 level implies a ovarian
malignancy. However, a small percentage of patients with Meigs syndrome
can present with raised serum CA 125 level.
- Meigs JV, Cass JW. Fibroma
of the ovary with ascites and hydrothorax: a report of 7 cases. Am J Obstet
Gynecol 1937;33:249–67.
Scully RE. Ovarian tumors: a
review. Am J Pathol 1977;87:686–720.
Abad A, Cazorla E, Ruiz F,
Aznar I, Asins E, Llixiona J. Meigs’ syndrome with elevated CA125: case report
and review of the literature. Eur J Obstet Gynecol Reprod Biol 1999;82:97–9.
Chan CY, Chan SM, Liauw L. A
large abdominal mass in a young girl. Br J Radiol 2000;73:913–4.
Meigs JV. Fibroma of the
ovary with ascites and hydrothorax; Meigs’ syndrome. Am J Obstet Gynecol
1954;67:962–85.
Terada S, Suzuki N, Uchide
K, Akasofu K. Uterine leiomyoma associated with ascites and hydrothorax.
Gynecol Obstet Invest 1992;33:54–8.
Agranoff D, May D, Jameson
C, Knowles GK. Pleural effusion and a pelvic mass. Postgrad Med J
1998;74:265–7.
Kabawat SE, Bast RC Jr, Bhan
AK, Welch WR, Knapp RC, Colvin RB. Tissue distribution of a coelomic-epithelium-related
antigen recognized by the
monoclonal antibody OC125. Int J Gynecol Pathol 1983;2:275–85.
Jacobs I, Bast RC Jr. The CA
125 tumour-associated antigen: a review of the literature. Hum Reprod
1989;4:1–12.
Timmerman D, Moerman P,
Vergote I. Meigs’ syndrome with elevated serum CA 125 levels: two case reports
and review of the literature. Gynecol Oncol 1995;59:405–8.
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