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Published by irianewsletter, 2021-05-30 00:43:43

31th May 2021

Case of the Week

Keywords: Case,week,IRIA,ICRI

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

• Lurie S. Meigs' syndrome: the history of the eponym. Eur J Obstet Gynecol Reprod
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
125 Levels: two case reports and review of the literature. Gynaecol
Oncol 1995; 59: 405–08.


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