Open Journal of Obstetrics and Gynecology, 2012, 2, 206-209 OJOG Published Online September 2012 (
Primary fibrom a tosis of t he breast in a 13 years old girl
Elhassani Mehdi1, Kouach Jaouad2*, Achenani Mouna2, Babahabib Abdellah2, Moussaoui Driss2,
Dehayni Mohamed2, Bakkali Hicham3
1Gynecology-Obstetrics Department, ElRapaha Polyclinic, Libreville, Gabon
2Gynecology-Obstetrics Department, Mohamed V Military Hospital for Instruction, Rabat, Morocco
3Anesthesiolog y Dep a r t ment, ElRapaha Polyclinic, Libreville, Gabon
Email: *
Received 13 May 2012; revised 20 June 2012; accepted 5 July 2012
The mammary fibromatosis is a rare clinical entity.
The etiopathogeny remains unclear. The clinical and
radiological characteristics of this injury are often
worrying within the main differential diagnosis of the
mammary carcinoma; however only the histological
examination can confirm the diagnosis. It is charac-
terized by a strictly local evolution with an infiltra-
tion and recidivism tendency without giving metasta-
sis. The treatment is basically surgical. We report
herewith an observed case of 13-year-old girl, pre-
senting with a huge tumor located in the left breast
with ulceration of the nipple-areolar plate. The diag-
nosis is confirmed by the histological study. The treat-
ment consisted of a mastectomy. Suites are simple. In
view of the clinical evidence of the literature, we call
back the difficulties of management of this tumor.
Keywords: Breast; Fibromatosis; Desmoid Tumor;
Extraabdominal Desmoid Tumors; Gardner’s Syndrome
The agressive fibromatosis or desmoid tumor is a mes-
enchymal tumor that corresponds to à fibroblastic pro-
liferation, having an intermediate biological behavior
between the benign fibroblastic lesions and the low grade
fibrosarcoma. It tends to recur, but with no metastas
anymore. According to its location, we distinguish the
abdominal fibromatosis (25% - 49%), the extra-abdo-
minal fibromatosis (43% - 60%) as well as the intra-
abdominal fibromatosis (8% - 15%) [1,2].
The mammary location represents only a particular
aspect of these tumors, its frequency is less than 0.2% of
the primitive tumors of the breast and 4% of the extra-
abdominal fibromatosis. She can arise from the fascia
musculo-aponeurotic of the chest wall or from fibroblasts
and myofibroblasts of the mammary parenchyma [1-3].
We report herewith a new case of desmoid tumor in a
13-year-old teenager, and In view of evidence of the
literature, we focus on the clinical aspects, the diagno stic
and therapeutic d ifficulties of this tumor.
Miss P.K., 13-year-old, of Gabonese origin, having a
regular cycle for two years, she is being admitted to the
department for a large ulcerated tumor by the left breast.
Her personal and family histories were without peculiarity
in particular no breast cancer. The beginning of the dis-
ease goes back up to one year by a self-discovery of a
tumor by the left breast having quickly increased in
volume with ulceration of all the nipple-areolar plate. All
this evolved in preservation context of the general state.
The examination finds the left breast increased in volume
with palpation of a mobile firm mass with regard to the
profound plan, the skin with regard to the nipple-areolar
plate is hurt with purulente serosity, covering the whole
breast and being 15 cm of diameter (Figure 1). The ex-
amination of the breast contralateral is without pecul ia rity.
The palpation of the ganglionic areas is without abnor-
Figure 1. Large tumor of the left breast with ulceration of the
nipple-areolar plate.
*Corresponding a uthor.
E. Mehdi et al. / Open Journal of Obstetrics and Gynecology 2 (2012) 206-209 207
malities. The mammography was not able to be achieved
and the echog raphy showe d a heter ogeneous tissular m ass
of which the big diameter > 14 cm. The cyt oponction been
not decisive, biopsies were realized. The histological
examination noticed a proliferation of well differentiated
regular fibroblasts, in the nucleus (kernel) sometimes
dystrophic with rare normal mitoses, the fibroblastic tis-
sue was arranged in beams in support of a desmoid tumor
of the left breast (Figur e 2). Given t he tumour size and t he
risk of a second recurrence, the patient benefited from a
left breast mastectomy. The histological study of this part
confirmed the diagnosis of desmoid tumor with healthy
limits. The post-operative evolution was favorable. The
clinical supervision did not show local second recurrence
with a recession of 6 months. A mammary plasty is ex-
pected after 03 years of evolution without a local recur-
The mammary fibromatosis is a rare lesion, often de-
scribed as isolated cases or small series.
It represents 0.2% of all the tumors of the breast [1,2 ].
It primarily affects women from 13 to 80 years old with
an average of 43 years, regardless of their ethnicity [2,3],
unlike the fibromatosis of the abdominal wall, the mam-
mary location is not mainly associated with the adoles-
cence. The mammary fibromatosis is more commun in
women than in men [2-4].
The etiopathogeny of desmoid tumors is not clear. It
may be the result of three factors: a cell proliferation
enhancing, possibly further to a trauma, a developing
sexual steroids and a particular genetic conditions af-
fected by a regulation problem of the fibroblast growth
Figure 2. Regular spindle-shaped cells, without atypia.
The clinical aspect is mostly about one-sided and firm
mammary gland nodule, generally painless, but with fast
and alarming increase, indistinct and irregular limits,
variable sizes (1 - 10 cm), affecting predominantly the
areal area of the right breast, sometimes with a nipple
discharge and unusual skin retraction [2,4]. Adenopathy
is usually absent [1,3-5].
The mammography is usually to have a bad opaque-
ness limited to fuzzy and irregular edges, architectural
distortion area with convergence effect and/or fibrous
divergence; a focal asymmetry of density suggestive of
malignant lesion but typically without microcalcifica-
tions. We sometimes observe a thickening or a skin re-
traction [1,3,5-7]. The literature reports other alternative
descriptions so unusual. The lesion sometimes appears as
a mass with a lobulated or not outlines, but still well de-
fined [4-8]. An ossifying lesion was also noticed. The
associated microcalcifications are rare. Finally, in some
rare cases, the lesion has no mammographic presence
The echographic appearances are various and less spe-
cific. They may include a hypo-échogènic lesion in the
irregular outlines, in the main vertical line with or with-
out post-mitigation [3,6-8]. The mammary fibromatosis
can also appear in an area with irregular and/or ill-de-
fined outlines, heterogeneous, hyper outlines and hypo-
echogenic with or without post-mitigation [6-8]. The
literature have also described the small-sized lesio ns that
are homogeneous lesions in very regular outlines [7,8].
Authors suggest the contribution of the MRI in the
preoperative diagnosis of fibromatosis mammary. In T1-
weighting, the lesion is isosignal in the fibro-glandular
tissue. In T2-weighting, with suppression of the fat signal,
we have a lesion presenting areas of low signal (collagen
tissue) and of high signal (myxoid tissue). After the in-
jection of chelated forms of gadoliniu m, in T1-weighting,
and after a fat signal saturation, we notice a heterogene-
ous raising in the ill-defined outlines. In dynamic acqui-
sition, the lesion presents a progressive raising, thing
which is not typical to the mammary carcinoma [3,4,7 ,8 ].
The magnetic resonance imaging and the scanner are
importante to estimate a possible parietal invasion [4,
The cytoponction with the fine needle could confirm
the diagnosis by isolating fibroblasts with minimal atypia;
however, it is mostly little contributory [1-4].
The diagnosis certainty is ba sed upon the post surgical
Macroscopically the legions are fibrous, brilliant,
greyish-white, firm, infiltrating, variable-sized and badly
limited [2,3,5,6].
The histological examination showed a proliferation of
spindle-shaped cells, fibroblastics and myofibroblastics,
with a variable cellularity, minimal atypia and a weak
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E. Mehdi et al. / Open Journal of Obstetrics and Gynecology 2 (2012) 206-209
mitotic activity, arranged in interlacing bundles which
infiltrate the fatty tissue, mixed (involved) with collagen
bands. A lymphoplasmacytic infiltration is mostly asso-
ciated, unlike the calcifications and the oedema that are
usually absent [1,3,5,6,9].
In the surrounding area, th e lesion is badly limited and
infiltrates the mammary parenchyma with extensions in
sensor pocket. The cellularity is more abundant in young
woman and the hormonal receptors; the epithelials are
usually absent.
The differen tial diagnosis prob lem arises in a different
way depending on the cellularity degree [3,5,7].
In the weak cellularity, cases to be mentioned are:
The nodular fascist generally too deep, well bounded,
with rapid growth and including an inflammatory in-
filtrate usually non-existent in the fibromatosis;
The fat necrosis coming along with many foamy
histiocytes and sometimes with a granulomatous epi-
thelioid around n e crosed fat cells;
The three diagnosis that have been of a great help to
the clinical history; the keloid or the hypertrophic
scarring; the traumatic and post-surgical lesions; the
fibrosis (radiq ue).
In the increased cellularity cases, the malignancies to
be mentioned are:
The metaplastic carcinoma with spindle-shaped cells
showing more (pléio) morphism and many mitoses.
Besides, cells are positive keratin;
Sarcomatous tumors (fibrosarcoma of low grade, ma-
lignant (fibrohistiocytome) showing a very important
(pléio) morphism, many mitoses of necrosis and a
possible vascular spread;
Liposarcomas, generally with a big size, including
stretched or cystic epithelial elements, in or near a
strengthening of the cellularity.
The utility of a systematic research for other locations
does not make the unanimity. Mostly sporadic, the fi-
bromatosis is associated in 2% of the cases with familial
adenomatous polyposis (FAP) which is the consequence
of a transf ormati on of the AP C ge ne (fam ily aden omatous
polyposis coli). Some authors recommend to realize, as
principle, a colonoscopy to eliminate the rare combination
with a polypose in Gardner’s syndrome [3-5,8,9].
The mammary fibromatosis is a locally aggressive
tumor, but without metastatic potential. The curative sur-
gical resection remains the treatment of choice in all the
publications. All authors agree on the need of a wide and
radical surgical treatment moving to healthy areas of at
least 1 cm so as to limit the risk of recurrences. If edges
are invaded , a revision sur gery shoul d be perform ed. Most
of recurrences show up in the first three years which jus-
tifies a clinical and radiological medium-term follow-up
The literature reports rates recurrence to be from 5%
to 25%, most within three to six years after the excision
sometimes more than 10 years later. It seems appropriate
to suggest a new curative surgical resection in case of
recurrence. The mastectomy can be indicated for vast
fibromatosis or for too large recurrences [2-4,8-10]. The
node dissection has no indication in this pathology. In
case of parietal invasion, the surgery can be extremely
decaying going as far as taking away the breast, the pec-
toral muscles, the thoracic wall, and the parietal pleura.
Because of the recurrence risk, a reconstructive breast
surgery should be performed ideally after 3 years [1,
The place of radiotherapy is being discussed. The re-
sults of the various series being often discordant. It re-
quires a strong doses to be effective almost superior to 60
Gy. Some made assumptions of a malignant transforma-
tion possibility of the fibromatosis after radiotherapy
associated with the surgery. Nevertheless, other studies
have shown a beneficial effect of the radiotherapy with
60% of decreasing cases non-accessible to the surgery.
Therefore, the radiotherapy could be curative in recur-
rence cases when the surgery is impracticable [4,6,7,
Several authors propose an alternative chemotherapy
to an aggressive surgery that can leadto serious after-
effects. The treatment by anti-estrogen (tamoxifen), in
spite of some described answers, does not seem to be
justified because of the absence of hormone receptors
The use of progestin came along with an increasing
size of a mammary tumor [2,3,5,9].
The mammary fibromatosis is a benign and infiltrated
rare tumor. Its clinical and radiological aspect is sugges-
tive of malignant tumor. Only histology will bring the
diagnosis. Its étiopathogeny remains controversial. Its
treatment consists of a complete exereses with healthy
safety margins so as to reduce recurrence risk. The area
of the radiotherapy and the medical treatments, espe-
cially anti-oestrogens, are not clearly defined, and lim-
ited to this location. The realization of a colonoscopy in
principle because of its possible association with the
polypose in frame of Gardner’s syndrome.
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