FIBROTHECOMA

Maria Carmina L. Santiago, M.D.

Presenter

Erlidia T. Llamas-Clark, M.D.

Consultant Moderator

Michelle Anne M. Encinas-Latoy, M.D.

Consultant Reactor

Oliver D. Pintor, M.D.

Resident Reactor

INDEX CASE

General data

R.T.

59

Married

G4P4 (4004)

Taguig

Chief complaint: Ultrasound finding of ovarian

mass

Past medical history

No known comorbid illnesses

No prior hospitalizations or surgeries

Family medical history

No familial illnesses

Personal and social

history

No vices

High school graduate

Housewife

Roman Catholic

First coitus 19, 1 non promiscuous sexual

partner

(-) OCP/IUD use

Menstrual history

Menarche 15

Regular monthly intervals

4 days

5 pads per day

(+) dysmenorrhea

Obstetric history
G1

G2

G3

G4

FT

FT

FT

FT

SVD

SVD

SVD

SVD

Local
hospit
al
Local
hospit
al
Local
hospit
al
Local
hospit
al

Alive
(-) FMC

Alive
(-) FMC

Alive
(-) FMC

Alive
(-) FMC

History of present
illness

1 year prior to admission: (+) left upper

quadrant pain, radiating to the back,
squeezing in character, NRS 8/10,
intermittent, worsens with urination,

No abdominal enlargement, no fever, no

hematuria, no bowel/bladder changes

History of present
illness

Consult at a local hospital, holoabdominal

ultrasound: staghorn calculus of the left
kidney

Also noted an abdominopelvic mass 8.0 x

13.1 x 7.5 cm, probably ovarian

Started on Coamoxiclav 625 mg/tab BID

Advised referral to OB-GYN

Review of systems

No fever, no weakness, no pallor

No anterior neck mass, no visual disturbances

No difficulty of breathing, no cough

No chest pain, no palpitations

No jaundice, no nausea or vomiting, no melena, no

hematochezia

No vaginal bleeding, no bowel movement changes

No paresthesias, no edema

Physical
examination
Awake, alert, ambulatory, not in

General
Vital
signs
Head
and
neck

cardiorespiratory distress
BP 130/70 HR 72 RR 18 T 36.9
Anicteric sclerae, pink palpebral
conjunctivae, no anterior neck mass

Equal chest expansion, clear breath

Lungs
sounds, no rales, no wheeze
Adynamic precordium, distinct heart
Chest
sounds, normal rate, regular rhythm, no
murmurs
Soft, flabby, nontender abdomen, no fluid
Abdome wave, (+) 16x16 cm abdominopelvic mass,
n
firm, slightly movable, (-) fluid wave, (-)

Physical examination
Speculum
Cervix 3x3 cm smooth, closed, no
examinatio
bleeding or discharge per os
n
Normal external genitalia, parous
Internal
vagina, cervix smooth, closed,
examinatio
corpus and adnexal difficult to
n
assess due to AP mass
Good sphincter tone, intact rectal
Digital
vault, no intraluminal masses,
rectal
inferior pole of the mass
examinatio
nonpalpable in the cul de sac, no
n
blood or stool per examining finger
Full, equal pulses, pink nailbeds,
Extremities no edema, capillary refill time <2
seconds

Impression

Abdominopelvic mass probably myoma

uteri rule out ovarian new growth

Complicated urinary tract infection,

staghorn calculus, left

Plan

Dx

CBC

Urinalysis

BUN, creatinine, Na, K, Cl,

Ca, Alb, AST, ALT, Mg

Urine culture

Pap smear

PT/PTT

Transvaginal ultrasound

CXR PA

Tx:

Continue Coamoxiclav 625

mg/tab to complete 2 weeks

Paracetamol 500 mg/tab q6

for pain/fever

Refer to urology

Laboratory workup
Hb
Hct
WBC
Platelet
Neut
Lym
Mono
Eos

148
0.44
7.0
165
0.72
0.3
0.00
0.00

BUN

4.7

Crea

85.1

Na

141.9

4.6

Cl

102.8

Ca

2.5

Mg

0.80

PT

12.9/12.6/105/0
.97

AST

31

ALT

29

PTT

29.6/30.1

LDH

372

Laboratory workup
WBC
13
Epithelia
0
l cells
Bacteria 31

Urine culture: No
growth after 2
days

Pap smear:
Negative for
intraepithelial
lesion

TRANSVAGINAL
ULTRASOUND

Ultrasonographic
impression

Abdominopelvic mass consider ovarian

fibroma cannot entirely rule out
pedunculate subserous myopia

Normal uterus with thin endometrium

Atrophic left ovary

Chest radiograph

Linear, obliquely oriented opacities are seen in the right middle

lung field

The heart is not enlarged

Calcific densities line the aortic knob

The trachea is midline

The left costophrenic angle is shallow

The right costophrenic angle and hemidiaphragms are intact

Spurs are seen along some of the visualized vertebral end

plates

The rest of the osseous structures and soft tissues are

unremarkable

Chest radiograph

Subsegmental atelectasis vs nonspecific

pulmonary parenchymal fibrosis, right

Minimal pleural effusion vs pleural

thickening, left

Atherosclerotic aorta

Degenerative osseous changes

Course

On ffup, with resolution of left upper

quadrant pain

Seen by urology

Advised CT sonogram

Sambong 2 tabs TID

Potassium citrate 2 tabs TID

CT STONOGRAM

CT Sonogram

The left kidney measures 10.4 x 6.6 x 5.5 cm. It is

markedly hydrfonephrotic due to the presence of a
staghorn calculus. Multiple lithiasis of varying sizes
are seen in the calyces ranging in size from 0.6 to 1.2
cm.

The right kidney measures 11.8 x 6.5 x 5.8 cm. Its

pelvocalyceal system is intact.

Both ureters are not dilated. The urinary bladder is

unremarkable. There is no right nephro-, uretero-, or
cystolithiasis.

Atheromatous aorta and lumbar spondylosis are

noted.

CT Sonogram
IMPRESSION
Left staghorn calculus and multiple
calyces lithiases with marked
hydronephrosis

Normal right kidney, ureters, and urinary

bladder

Large lobulated lower abdominal/pelvic

mass

Atheromatous aorta

Lumbar spondylosis

Tumor markers
Ca 125

280.2

Ca 19-9

32.33

Impression

Abdominopelvic mass to consider ovarian

new growth, rule out malignancy vs
myoma uteri

Complicated urinary tract infection,

resolved, staghorn calculus, left

Plan

Scheduled for elective surgery

Exploratory laparotomy, total hysterectomy with

bilateral salpingooophorectomy possible surgical
staging

For referral to Urology on admission for final plan

Course

Admitted and referred to Gyne Onco and

Urology

Urology: For cystoscopy, preoperative

ureteral stenting, left nephrectomy

Cleared by medicine for surgery

Intraoperative
findings

Procedure
Preoperative cystourethroscopy, bilateral
ureteral stenting, exploratory laparotomy,
peritoneal fluid cytology, adhesiolysis, total
hysterectomy with bilateral
salpingooophorectomy, bilateral pelvic
lymph node dissection, paraaortic lymph
node sampling, infra colic omentectomy,
random peritoneal biopsy, left
nephrectomy, repair of urinary bladder
serosal tear, insertion of Jackson-Pratt drain
under epidural anesthesia

Intraoperative
diagnosis

Ovarian new growth, left, malignant,

intraoperative stage IA rule out IIB

Staghorn calculus, left kidney

Histopathology

Scanning view, ovarian mass

Low power view, ovarian mass

High power view, ovarian mass

vScanning view,
fibrothecoma
High power view, ovarian mass

Low power view, left kidney

High power view, left kidney

Final histopathologic
diagnosis

SPINDLE CELL NEOPLASM, FAVOR FIBROTHECOMA, LEFT OVARY.

REMARKS: RECOMMEND IMMUNOHISTOCHEMICAL STUDIES FOR CALRETININ TO CONFIRM

DIAGNOSIS.

SYMPLASTIC LEIOMYOMA, SUBSEROUS (1).

CYSTIC ATROPHY, ENDOMETRIUM.

CHRONIC CERVICITIS WITH NABOTHIAN CYST.

NO DIAGNOSTIC ABNORMALITY RECOGNIZED, RIGHT OVARY, ATTACHED LEFT OVARY, AND

BILATERAL FALLOPIAN TUBES.

CHRONIC PYELONEPHRITIS, LEFT KIDNEY.

NEGATIVE FOR TUMOR: OMENTUM, SPECIMEN LABELED "BLADDER", "RIGHT PELVIC SIDE
WALL", "LEFT PELVIC SIDE WALL", "CUL DE SAC", "A1", "PARACOLIC RIGHT", "PARACOLIC
LEFT", "PERITONEAL FLUID CYTOLOGY", ALL FOUR (0/4) LYMPH NODES IN SPECIMEN
LABELED "EXTERNAL RIGHT", ALL FOUR (0/4) LYMPH NODES IN SPECIMEN LABELED
"EXTERNAL LEFT", ALL THREE (0/3) LYMPH NODES IN SPECIMEN LABELED "OBTURATOR
RIGHT", AND BOTH (0/2) LYMPH NODES IN SPECIMEN LABELED "OBTURATOR LEFT".

Final diagnosis

Spindle cell neoplasm, favor fibrothecoma,

left ovary

Myoma uteri

Chronic pyelonephritis, left, secondary to

staghorn calculus

Fibrothecoma
Discussion

Sex-cord stromal
tumors

Fibrothecomas are classified under sex-cord-stomal tumors

Includes all ovarian neoplasms that contain granulosa cells,

fibroblasts, theca cells (and their luteinized derivatives),
Sertoli cells, Leydig cells, singly or in various combinations
and in varying degrees of differentiation

Account for 8% of all ovarian tumors

Approximately half are fibromas

Majority of clinically malignant are granulosa cell tumors

Great diversity of cell types and patterns with overlap

between various categories

son LH, Ronnett BM. Blausteins Pathology of the Female Genital Tract. 6 th ed. London:

Sex-cord stromal
tumors
Along with the being aware of the clinical picture
of the patient, thorough sampling of the ovarian
mass is paramount

Very important role of immunohistochemistry

Triad: inhibin, calretinin, and epithelial

membrane antigen (EMA)

Inhibin, calretinin - typically positive

EMA - typically negative

son LH, Ronnett BM. Blausteins Pathology of the Female Genital Tract. 6 th ed. London:

Lentz GM, Lobo RA, Gershenson DM, Katz VL. Comprehensive Gynecology,
6th edition. Philadelphia: Elsevier, 2012

Thecoma-fibroma group

Composed exclusively or almost

exclusively of theca cells, fibroblasts of
ovarian stromal origin, or both

The presence of occasional small nests of

granulosa cells or occasional tubules lined
by Sertoli cells does not exclude tumors
from this category

Such tumors have been referred to as

fibromas or thecomas with minor sex
cord elements

son LH, Ronnett BM. Blausteins Pathology of the Female Genital Tract. 6 th ed. London:

Fibrothecoma

Thecomas are histologically composed of lipidcontaining cells that resemble theca interna cells

Fibromas are composed entirely or almost entirely

of spindle, oval, or round cells forming variable
amounts of collagen

The differentiation between thecomas and

fibromas is occasionally imprecise because of the
histological and immunohistochemical overlap
between them. Therefore, the term fibrothecoma
has been frequently used

Have been reported to show myxoid change or

degeneration

Fibrothecomas

Account for 1% to 4.7% of organic ovarian

neoplasms

Unilateral in about 90% of the cases

Occur generally in older menopausal patients

Some authors report 2 peaks of frequency:

between 20 and 40 years and after
menopause

The occurrence of these tumors before the

age of 20 years is extremely rare

Clinical presentation

Relatively nonspecific

Physical examination: solid, mobile tumor with a

regular surface and a variable size.

Rarely, fibrothecomas present with endocrine

manifestations related to hormonally active
tumors

Estrogenic or even some rare androgenic

manifestations are reported

Pelvic pain and metrorrhagia

Clinical presentation

Clinical associations

Meigs syndrome

Ovarian fibroma, hydrothorax, and ascites

1-10% of ovarian fibromas, ~1% of

fibrothecomas

Seepage of fluid from the tumor through its serosal

surface into the peritoneal cavity, with subsequent
passage into one or both pleural cavities either via
lymphatics or through a communication between
the abdominal and pleural cavity such as the
foramen of Bochdalek.

Clinical presentation

Clinical associations

Gorlin-Goltz syndrome or basal cell nevus

syndrome

Bilateral ovarian fibromas, multiple basal

cell carcinomas of the skin, odontogenic
keratocysts, and calcified dural folds

Autosomal dominant disorder that occurs

generally in patients aged younger than 30
years

Clinical presentation

Other clinical associations

Familial polyposes

Gardner syndrome

Richard syndrome

Peutz-Jeghers syndrome

Fibrothecomas are at risk for adnexal torsion,

particularly with large tumors

and management. of ovarian fibromas and fibrothecomas. American Journal of Obstetr

n M et al. Ovarian fibrothecoma. http://radiopaedia.org/articles/ovarian-fibrothecoma

Imaging

On transvaginal ultrasound: may be seen

as a homogeneous hypoechoic mass with
posterior acoustic shadowing

In most cases, the sonographic

appearance is nonspecific

Yen et al (2013): Most completely solid,

though some with mixed solid and cystic
lesions (28%)

orrelation With Computed Tomography and Magnetic Resonance Imaging: A 5-Year Single-Institutio

Chechia A, et al. Incidence, clinical analysis, and management. of

ovarian fibromas and fibrothecomas. American Journal of Obstetrics &

Imaging

CT scan

The vast majority (~80%) of ovarian fibrothecomas appear as solid

masses with delayed accumulation of contrast medium

On dynamic CT, there is an absence of arterial vessels and absence

or slight early uptake of contrast enhancement

Calcification may be present and, as these tumors enlarge, myxoid

or cystic degeneration may occur, resulting in a heterogeneous
pattern

Pelvic MRI

T1: typically shows homogeneous low signal intensity

T2: lesions show predominantly homogeneous low signal intensity

(from fibrous components) scattered high signal areas may be
present representing areas of cystic degeneration with or without
an M edema
et al. Ovarian fibrothecoma. http://radiopaedia.org/articles/ovarian-fibrothecoma

Laboratories

CA125 serum levels are usually normal

Rarely, high serum levels are noted, and

they become normal after tumor
removal

Gross findings

Gross findings

Source

Histopathologic
findings

Scanning view, fibrothecoma

High power view, thecomatous component

High power view, fibrous component

High power view, fibrothecoma (index case)

Treatment

Treatment is primarily surgical

Ascites, if present, disappears after tumor

removal

Prognosis

Fibrothecomas are generally benign:

malignant thecomas and fibrosarcomas
represent less than 1% of the cases
reported

Main histologic features are

hypercellularity arranged in a
herringbone pattern and mitotic counts
of 6 to 8 per 10 high-power fields

, and management. of ovarian fibromas and fibrothecomas. American Journal of Obste

1-Year-Old Woman With Ascites and Elevated CA 125 Level. Archives of Pathology & La

High power view, malignant fibrothecoma

Summary

Summary

Fibrothecomas fall under the classification of sexcord stromal tumors of the ovary, which accounts
for 8% of all ovarian tumors.

Fibrothecomas are an intermediate

differentiation between thecomas and fibromas,
accounting for 1-4.7% of all ovarian neoplasms,
generally occurring in menopausal patients.

Clinical presentation is nonspecific and they may

occasionally present with endocrine
manifestations.

They may be associated with Meigs syndrome

the triad of ovarian fibroma, hydrothorax, and

Summary

Immunohistochemistry specifically with inhibin,

calretinin, and EMA is essential for the
definitive diagnosis.

Transvaginal ultrasound is still the most utilized

modality, though CT scan and MRI can also be
used.

Tumor markers such as CA125 are usually

normal.

Prognosis is good, as fibrothecomas are a

benign lesion, with less than 1% malignant
degeneration.

References

Lentz GM, Lobo RA, Gershenson DM, Katz VL. Comprehensive

Gynecology, 6th edition. Philadelphia: Elsevier, 2012

Kurman RJ, Ellenson LH, Ronnett BM. Blausteins Pathology of

the Female Genital Tract. 6th ed. London: Springer; 2011.

Dhull A, Kaushal V, Mathur S, Agarwal R. Fibrothecoma of the

Ovary- A Rare Case Presentation. The Internet Journal of Third
World Medicine. 2010 Vol 9:2.

Morgan M et al. Ovarian fibrothecoma.

http://radiopaedia.org/articles/ovarian-fibrothecoma.

Chechia A, et al. Incidence, clinical analysis, and management.

of ovarian fibromas and fibrothecomas. American Journal of
Obstetrics & Gynecology (2008), 473: 1-4.

References

Kim S., Park J. A case of malignant fibrothecoma of the ovary.

Korean J Obstet Gynecol 2012:55(3):187-191.

Angeles R, Salem F, Sirota R .A Right Ovarian Mass in a 71-YearOld Woman With Ascites and Elevated CA 125 Level. Archives of
Pathology & Laboratory Medicine: May 2005, Vol. 129, No. 5, pp.
701-702.

Mallappa L, et al. Atypical (symplastic) leiomyomaa case

report. International Archives of Integrated Medicine, Vol. 2,
Issue 8, August, 2015.

Shinagare A. MRI Features of Ovarian Fibroma and Fibrothecoma

With Histopathologic Correlation. American Journal of Radiology.
2012: 198: 296-303.

Thank you!