ICRI CASE OF THE WEEK
Contributors : Dr. G.Yuvageetha, Dr. D. Ankamma Rao
NRI General hospital, Chinakakani, Guntur
Copyright of the case belongs to : Dr. G. Yuvageetha
Clinical details:
39 year old female patient presented to general
surgery OPD with chief complaint of right lower
abdominal pain since 8 months
Pain was insidious in onset, progressive in nature,
colicky type of pain and associated with nausea.
No history of vomiting
No history of major previous surgeries
Menstrual history:-
She attained menarche at the age of 13 years with
regular cycles- 3-5/30, moderate flow
Obstetric history:-
Age of marriage was at 21 years with two living
children
Age at first child birth was 22 years
General Examination of Abdomen :
Visible mass in right iliac fossa extending into
hypogastrium, umbilical and left iliac region.
Umbilicus normal in position. No scars, no visible
pulsations and no engorged veins are seen.
Tenderness present more in right iliac region
A single palpable mass in right iliac region extending
into hypogastrium, umbilical, left iliac region.
Mass of size approximately 15 x 10 cm with smooth
surface, well defined margins, hard to cystic swelling
Skin over the swelling was normal.
Blood Investigations
Initial laboratory investigations revealed elevated
CA -125 levels
CA -125 : 352 U/mL
(Normal value : <46 U/mL)
(a) Plain CT (c) Plain CT
What are the findings ?
(b) X-ray Chest PA view
NECT
What are the findings ?
NECT CECT
What are the findings ?
(a) (c)
(a & b) CT plain section and
X-ray showing right sided
pleural effusion
(c.) CT abdomen section
showing free fluid in pelvis
(b)
NECT
NECT : Shows isodense mass lesion anterior to uterus, in the pelvis
extending into lower abdomen
NECT CECT
(a) On Post-contrast scan, the
lesion shows very subtle
heterogenous enhancement.
(b) Mass can be seen
separately from uterus and left
ovary. Adjacent pelvic free
fluid can be appreciated.
What is the working diagnosis ?
Mildly heterogenously enhancing pelvic mass
Ascites and right sided pleural effusion with elevated
CA-125 levels
No peritoneal thickening, no omental nodes and no
other lesions in abdomen and chest
What are the imaging findings ?
(a) (b)
(c) (d)
(a) Pelvic mass with ascites. (b) Right sided pleural effusion (c) On contrast administration, the mass
shows mild heterogeneous enhancement and is seen separately from uterus. Left ovary seen
separately. Right ovary not seen separately from mass
Histopathology Findings :
Chest X-ray 2 weeks after surgical resection
Right pleural effusion has resolved.
What is the final diagnosis ?
Benign Ovarian Fibroma with Meigs Syndrome
Ascites and Right sided pleural
effusion
Pleural effusion subsided after
surgical resection of the
Ovarian Fibroma
Discussion
Meigs' syndrome is diagnosed based on a triad of an
ovarian fibroma, pleural effusion and ascites.
It resolves spontaneously after the resection of the
fibroma
4 characteristics of Meigs syndrome:
The tumour is a benign fibroma or a fibroma-like tumour of
the ovary (such as thecoma or granulosa cell tumour)
Ascites
Pleural effusion
Removal of tumour must cure the patient.
Other benign cysts of the ovary (such as struma
ovarii, mucinous cystadenoma, teratoma), leiomyoma
of the uterus, and secondary metastatic tumours to
ovary, if associated with hydrothorax are referred to
as ‘Pseudo‐Meigs” syndrome
Patho-Physiology :
Ascites- Filtration of interstitial liquid into the peritoneum
through the ovarian tumour capsule
Pleural effusion – Ascitic fluid moves from peritoneal
cavity to pleural cavity through diaphragmatic defects or
via lymphatic channels, eventually causing an exudative
pleural effusion
An imbalance between the blood supply to a large
tumour and its venous and lymphatic drainage may be
responsible for stromal edema and transudation
New studies suggest that the fluid accumulation may be
related to proteins such as vascular endothelial growth
factor (VEGF) that raise capillary permeability
Elevated CA 125 levels :
Immuno-histochemical staining for CA 125 suggests that serum
elevation of CA 125 antigen is secondary to mesothelial
expression of CA 125
Biochemical factors, mechanical irritation from a large tumour,
and a raised intraperitoneal pressure secondary to ascites are
possible primary factors
Differential diagnosis :
Malignant ascites and pleural effusion in the presence
of aggressive ovarian tumor
Pseudo-Meigs syndrome
Pseudo-Meigs Syndrome :
Ovarian tumor (that is not fibroma or fibroma-like)
+
Pleural effusion
+
Ascites
Pseudo-Meigs Syndrome :
Ovarian tumor (that is not fibroma or fibroma-like)
+
Pleural effusion
+
Ascites
Krukenberg tumors
colon CA metastases to ovary
Struma ovarii tumor
Uterine leiomyosarcoma: rare
Uterine leiomyoma: rare
Ovarian teratoma: rare
Pseudo-Meigs syndrome :
Ovarian tumor (that is not fibroma or fibroma-like)
+
Pleural effusion
+
Ascites
CA-125 level is elevated and hence cannot Krukenberg tumors
be used for differentiation colon CA metastases to ovary
Struma ovarii tumor
Uterine leiomyosarcoma: rare
Uterine leiomyoma: rare
Ovarian teratoma: rare
Another rare association of ovarian fibroma is
“NEVOID BASAL CELL CARCINOMA
SYNDROME”
It is also called “ GORLIN-GOLTZ Syndrome “
It presents as :
Multiple BCCs , often at puberty & adolescence
Craniofacial anomalies
Musculoskeletal anomalies
Neoplasms / hamartomas – E.g. Medulloblastoma,
Ovarian fibroma etc.
Treatment
Definitive diagnosis of Meigs' syndrome is usually post-
operative with resolution of ascites and pleural
effusions, and histological confirmation of the tumor.
The treatment of choice is exploratory laparotomy with
surgery and staging.
Frozen section of ovarian mass is performed to confirm
the benign nature of the mass.
In women of reproductive age, unilateral
salpingo‐oophorectomy is the treatment of choice,
whereas in post‐menopausal women, treatment is Total
abdominal hysterectomy with bilateral
salpingo‐oophorectomy.
Teaching Points
A triad of ovarian mass, ascites and pleural effusion
usually is considered to be aggressive neoplastic
etiology. However the chance of it being benign
tumor should be considered
Intra-operative frozen sections can help in planning
the course of surgery
Do not consider raised levels of CA-125 as
definitive for aggressive malignancy
References
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Biol 2000; 92: 199–204.
• Lallemand A. Contribution a'l'étude des fibromes de l'ovaire. Thése pourle
doctorat en médecine. Paris; 1896. No.285.
• Meigh JV, Cass JW. Fibroma of the ovary with ascites and hydrothorax: With a
report of seven cases. Am J Obstet Gynecol 1937; 33: 249–67.
• Rhoads JE, Terrell AW. Ovarian fibroma with ascites and hydrothorax (Meigs
syndrome): a case. JAMA 1937; 109: 1684.
• Meigs JV. Fibroma of the ovary with ascites and hydrothorax‐ Meigs
syndrome. Am J Obstet Gynecol 1954; 67: 962–85.
• Ishiko O, Yoshida H, Sumi T, Hirai K, Ogita S. Vascular endothelial growth factor
levels in pleural and peritoneal fluid in Meigs' syndrome. Eur J Obstet Gynecol
Reprod Biol 2001; 98: 129.
• Jones OW III, Surwit EA. Meigs' syndrome and elevated CA 125, obstet. Gynecol.
1989; 73: 520–21.
• Timmerman D, Moerman P, Vergote I. Meigs' Syndrome with elevated Serum CA
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