Author
Information
Anuranjani
L*, Ostwal P**, Chauhan AR ***.
(*
Fourth year resident, ** First year resident, *** Additional
Professor and Unit Head.
Department
of Obstetrics and Gynecology, KEM Hospital, Mumbai, India.)
Abstract
Ovarian
fibromas are benign ovarian tumorswith incidence of 1-4.7%. Generally
found in perimenopausal and menopausal females, they are rarely found
in premenarchal teenagers. As they present with nonspecific symptoms
they are often misdiagnosed as fibroid or malignant ovarian tumours.
Clinical features, ultrasonography in adjunction with tumorsmarkers
remain the best preoperative approach to aid diagnosis. Though benign
in nature, occasionally raised carcinoma antigen 125 (CA-125) and
Meig’s syndrome may be associated and result in poor prognosis.
Surgical excision is the treatment of choice and histology remains
the gold standard for diagnosis. We report an atypical case of large
ovarian fibroma encountered in a premenarchal teenage girl. The case
highlights the potential of misdiagnosis due to its presentation, age
of occurrence and absence of characteristic symptoms.
Introduction
Innumerable
pelvic masses confront the gynecologist and often lead to diagnostic
dilemma. Among these, masses arising from ovaries are not easily
identified due to their anatomical location or dynamic nature until
they present with symptoms. Amidst the classification of ovarian
tumors, ovarian fibromas belong to the group of sex cord stromal
tumors; histologically they have stromal origin and are composed of
fibroblastic spindle cells which produce collagen. Ovarian fibromas
are benign neoplasms of ovary with occurrence of 1 to 4.7%.[1, 2]
Though the incidence is low, they are the most common benign solid
primary tumors of the ovary.[1] Presenting age is perimenopausal or
postmenopausal and rarely below 20 years of age.[1, 2] Even
though there is rare documentation of occurrence in a 13-and a
8-year-old patient, till now very few cases have been reported in
premenarchal age [1]. Though
mostly asymptomatic they may present with nonspecific symptoms like
abdominal pain or mass.[1] Generally unilateral and solid in nature,
cystic fibromas may also occur.[1, 2] Being solid tumors they are
often misdiagnosed as fibroid or as malignant ovarian tumor if
ascites and raised CA-125 is found.[3, 4] Due to these reasons, it
poses a diagnostic dilemma if encountered in teenage years.
Case
Report
A
13 year old unmarried, premenarchal girl presented in our OPD with
intermittent pain in abdomen since 20 days. She noticed a lump in the
lower abdomen 15 days ago and complained of intermittent fever since
1 month. There was no history of breathlessness, vomiting, edema or
any bowel or bladder complaints. No significant medical, surgical or
family history was found. On
general examination, the patient was afebrile with stable vital
signs. Pallor was present while no icterus, cyanosis, lymphadenopathy
or edema was found. Both breast and pubic hair were Tanner stage
III. Systemic examination did not reveal any abnormality. On
abdominal examination, a midline mass around 16 weeks, tender, firm
to hard in consistency with restricted mobility was present. No
ascites was demonstrated. On local examination hymen was intact.
Urine pregnancy test was negative and preliminary diagnosis of
fibroid or remote possibility of a hematometra was made.
Trans-abdominal
sonography revealed a solid ovarian tumor of 13 x 10 x 9 cm in the
right iliac fossa with arterial and venous flow having resistance
index (RI) of 0.3. Uterus was normal in size with an ill-defined
endometrial echo. The left ovary could not be visualized. A possible
diagnosis of malignant ovarian tumor was made. Computed
tomography scan reported a smooth surfaced, solid-cystic lesion on
left side measuring 6.5 x 9.4 x 11 cm with vascular pedicle arising
from left ovarian vessel. Right ovary and uterus were normal. Few
sub-centimeter lymph nodes and minimum free fluid in the pelvis was
present. A left ovarian neoplasm of malignant nature was suspected. Based
on these suspicions, tumor markers were investigated: CA-125, CEA,
β-hCG, AFP were found to be within normal limits. Apart from the
routine investigations, chest X-ray, ESR and serum testosterone were
also within normal limits. LDH was raised (491.6 IU/L).
With
a probable diagnosis of left ovarian tumour of malignant origin,
patient underwent exploratory laparotomy after a valid, well informed
consent from her parents. A smooth, solid mass of 10 x 12 cm arising
from the left ovary with dilated vessels was found as seen in Figure
1. No torsion or adhesions were found. The right ovary and both
fallopian tubes were normal. Uterus was infantile. Around 10 ml
peritoneal fluid was aspirated and sent for cytology. Omental biopsy
was done. Undersurface of liver, diaphragm and colon were normal.
Para-aortic lymph nodes could not be palpated. Patient underwent left
salphingo-opherectomy and the procedure was uneventful. Patient was
discharged on post-operative day 7.
Figure
1: Intraoperative findings.
Peritoneal
fluid cytology did not reveal any malignant cells. Histopathological
report on gross examination showed 10 x 7.5 x 4 cm ovary with smooth
surfaced, encapsulated, solid mass with dilated vessels. Cut section
showed grey white to yellow areas with few cystic lesions in the
ovarian tumor (Figure 2). The initial diagnosis of spindle cell
neoplasm suggesting sex cord stromal tumor
was given; the final diagnosis was left ovarian fibroma with no
atypia or malignancy.
Figure
2: Cut section of ovarian fibroma.
Discussion
Fibromas
are benign stromal tumors composed of spindle, oval or round collagen
producing cells arranged in storiform (cartwheel like) pattern.
Pathogenesis is obscure but trisomy and tetrasomy 12 have been
suggested.[6] These are generally unilateral, solid, mobile tumors of
variable size with bilaterality of 0-11.7% [3]. On histopathology, a
rare variant called cellular fibroma comprising of absent or minimal
nuclear atypia with 1-3 mitoses/10 HPF has been reported. Another
variant with incidence < 1% showing moderate nuclear atypia and >
4 mitoses/ 10 HPF having malignant potential is designated as a
fibrosarcoma. [2, 3]
Generally
diagnosed on ultrasonography as solid masses, fibroma usually appear
as homogeneous solid tumors with delayed enhancement on CT. MRI
appearance is of a hypo intense mass on T1-weighted images with very
low signal intensity on T2-weighted images [1]. The
treatment of these tumors is surgical where tumorectomy can be done
for young patients or radical treatment for perimenopausal and
postmenopausal patients. Even though we performed exploratory
laparotomy there are reports of laparoscopic resections with good
recovery and shorter hospital stay [3]. A
rare but well known syndrome encompassing ascites, pleural effusion
and benign solid ovarian tumor known as Meig’s syndrome is found in
1% ovarian tumors and have good prognosis.[2, 5] Ascites and
hydrothorax regress after tumour removal.[2] Meig’s syndrome has
been commonly found with ovarian fibromas (1-10%).[2, 4] Laparoscopic
removal of large ovarian fibroma with torsion and Meig’s syndrome
have been reported.[4] There
are also documentations of rare associations with Gorlin-Goltz
syndrome (multiple basal cell carcinomas, odontogenic keratocysts,
calcified falx cerebri, shortened 4th metacarpal), Gardener’s
syndrome (multiple adenomatous polyps in colon, sebaceous cysts,
osteomas) or Peutz Jeghers syndrome (hereditary intestinal polyposis,
melanosis).[1, 2] A
relationship between haemolytic anemia and benign ovarian tumors with
complete resolution of haemolytic anemia after tumor removal has been
reported;[4] however our patient had only mild anemia.
Conclusion
For
a solid or cystic adnexal mass where the primary consideration is
commonly an epithelial tumor, the possibility of a stromal tumor
should also be considered.[1] Due to its rarity and presentation it
is often misdiagnosed. Clinical findings, ultrasonography and tumor
markers remain the best preoperative approach currently available.
Surgical resection is the treatment of choice and histopathology is
the gold standard for diagnosis. Prognostic factors have not been
fully characterized.
References
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Anuranjani
L, Ostwal P, Chauhan AR. Abdomino-Pelvic Mass In a Teenager - a
Diagnostic Dilemma. JPGO 2016. Volume 3 No. 2. Available from: http://www.jpgo.org/2016/01/abdomino-pelvic-mass-in-teenager.html