19th Apr 2021

ICRI CASE OF THE WEEK

Contributed By : Dr Jwala Srikala DMRD, DNB

Senior Consultant Radiologist
Krishna institute of medical sciences, Hyderabad

Copyright of the Case belongs to : Dr Jwala Srikala

Clinical Details

• 25 year old, unmarried female presents with progressive retraction of
the right nipple with a mass in right breast from the last 3 months

• No associated nipple discharge

• No associated trauma / or prior surgery to the right breast

• No family history of breast cancer

Mammogram - CC Views

Mammogram –MLO Views

Shearwave Elastography

USG of Right Axilla

• What is your diagnosis ?
• What is the BIRADS category ?
• What will you do next ?

Mammogram – CC View and MLO View of the right breast show an irregular high density
mass in the central and lower aspect of right breast. No associated microcalcifications seen.
There is retraction of the nipple areolar complex

a bc

High resolution ultrasound of the right breast shows an irregular hypoechoic
mass at 6 ‘o’ clock position in right breast. The mass is abutting the pectoralis
muscle but not infiltrating it (Arrow in fig b and c)

Shearwave Elastography of the lesion shows very high velocities within the
lesion and in the surrounding parenchyma suggestive of a stiff lesion

USG of right axilla shows a benign appearing lymph node
with normal thickness of cortex and preserved fatty hilum

• Irregular high density mass in right breast with retraction
of the nipple areolar complex is suggestive of a BIRADS V
lesion with a recommendation for core biopsy.

USG Guided Core Biopsy

• HPE – Low grade Spindle Cell Tumor
• IHC

Beta catenin - negative

CD-34 - negative

Pancytokeratin - negative

Possibility of Myofibroblastic tumor was given with advise for
excisional biopsy

• Is this RAD-PATH concordance or discordance?

CE MRI of both the Breasts

• Irregular mass in central quadrant of right breast with mild
progressive enhancement with a type-I time intensity curve.

• There is no invasion of the chest wall posteriorly.

MRI – BIRADS – IV C

Patient underwent a wide local excision of the tumor with
oncoplastic procedure with a frozen section biopsy which was
reported as fibromatosis

Final Histology with Immunohistochemistry

Fibromatosis of the breast

FIBROMATOSIS

• Mammary fibromatosis is a rare, benign stromal tumor of the breast
that constitutes less than 0.2% of all breast tumors

• This tumor is locally aggressive and may recur in up to 29% of cases
• It mostly affects women during their reproductive years, often

following trauma or various surgical breast procedures such as breast
reduction or breast augmentation with saline or silicone implants
• Initially, mammary fibromatosis was reported in a patient with
familial adenomatous polyposis syndrome, or Gardner syndrome.
• Unlike desmoid tumor of abdomen, this is not associated with
pregnancy.

Clinical findings

• Mammary fibromatosis presents as a palpable mass that is clinically
suspicious for malignancy. Dimpling or retraction of the skin may be
present and the mass may adhere to the chest wall

• Breast is an uncommon site

Imaging

• Mammography - Fibromatosis presents as a spiculated mass that is
suspicious for malignancy (BI-RADS category 5) Calcifications are
extremely rare

• USG - Fibromatosis typically appears as a solid, spiculated or
microlobulated, irregular hypoechoic mass with straightening and
tethering of Cooper ligaments, which is very difficult to differentiate
from a malignant lesion

• Involvement of the pectoralis muscle or intercostal muscles may be
identified, indicating the locally aggressive nature of fibromatosis

• MRI is the best imaging technique for evaluating tumor extent and, in
particular, chest wall involvement. The masses are typically irregular
and are isointense to muscle on T1-weighted images and of variable
high signal intensity on T2- weighted images

• The enhancement pattern is of a benign progressive enhancement, as
opposed to the typical washout kinetics of breast carcinoma.

Pathology

• Microscopically-An infiltrative stromal process occurs, composed of
fibroblasts or myofibroblasts, or both, with little to no nuclear
pleomorphism and variable amounts of collagen. Mitoses are rare to none.
Lymphoid aggregates often may be seen at the periphery of the lesion.

• The Differential Diagnosis includes

 Metaplastic carcinoma
 Spindle cell type
 Low grade fibrosarcoma
 Nodular fasciitis
 Scar or biopsy-site reaction.

Immuno-Histochemical Markers in differentiating Spindle Cell Tumors

Fibromatosis Beta catenin SMA CD 34 PAN CK STAT- 6
+ + _ _ _

Myofibroblastic _ +/- +_ _
tumor _ +/- +_ +
_ + __ _
Solitary fibrous
tumor (SFT)

Nodular fascitis

Spindle cell _ __ + _
carcinoma

Treatment

• The recommended treatment is complete surgical resection. Margins may be
difficult to assess clinically during the surgical procedure because of the
infiltrative nature of fibromatosis. Frozen sections may be helpful to
determine clear margins.

• Recurrence rates are high in patients with margins positive for mammary
fibromatosis, although recurrence may develop in patients with margins
negative for the entity. Recurrence rates of up to 29% have been reported.

• Radiation therapy has been used to obtain local control in recurrent
fibromatosis.

Further Reading

• Fibromatosis of the Breast Diagnostic Accuracy of Core Needle Biopsy M. Gabriela Kuba,
MD,1 Susan C. Lester, MD, PhD,1 Catherine S. Giess, MD,2 Monica M. Bertagnolli, MD,3
Tad J. Wieczorek, MD,4 and Jane E. Brock, MB, PhD1

• Wuyts, L and De Schepper, A. Desmoid-type Fibromatosis of the Breast Mimicking
Carcinoma. Journal of the Belgian Society of Radiology. 2019; 103(1): 13, 1–3. DOI:
https://doi.org/10.5334/jbsr.1612

• Ultrasound Elastographic Findings of Mammary Fibromatosis Ping He,1 Li-Gang Cui,1
Yu-Tao Lei,2 Jian-Ying Liu,3 and Jin-Rui Wang1

• Mammary Fibromatosis-Katrina N. Glazebrook1 Carol A. Reynolds2