Case Report:
A 15-year-old female presented to family physician with a 4-months
history of left breast swelling. According to the patient, she felt a
lump in the left breast that was slowly increasing in size causing some
discomfort and burning sensation. There was no complaint of pruritus or
discharge from the nipple. On examination, a violaceous thickened
cutaneous plaque was noted without any excoriation or ulceration. The
patient was referred to the breast clinic where an ultrasound-guided
biopsy of the breast parenchyma and axillary node was performed, that
revealed chronic inflammatory changes (mixed infiltrate of lymphocytes,
histiocytes, and occasional plasma cells with stromal hyalinization)
with negative histology for bacterial/fungal stains. A diagnosis of
morphea profunda was favored, and patient was referred to the morphea
clinic at our hospital. After a thorough evaluation, it was concluded
that her symptoms and presence of histiocytes on biopsy were not
consistent with morphea and possibility of non-specific inflammation was
entertained, particularly in view of a preceding covid vaccination in
the ipsilateral arm. Clarithromycin was prescribed and inflammatory
markers (ESR, CRP, ANA) were done and found to be non-diagnostic.
Ultrasound was repeated to assess any interval change and revealed
extensive soft tissue edema of the left breast and enlarged axillary
lymph nodes, concerning for lymphoproliferative disorder (Fig-1a). In
view of the ultrasound findings and interval progression, an MRI of the
breast was done that revealed marked and diffuse inflammation of the
left breast predominantly involving the subcutaneous soft tissue with
questionable nipple retraction (Fig-1b). Repeat MRI with contrast after
2 weeks (Fig-1c) demonstrated mild interval progression with predominant
subcutaneous enhancement and enhancing axillary lymph nodes without any
discrete parenchymal mass. The right breast and axilla were normal.
An ultrasound-guided punch biopsy of the subcutaneous soft tissue of the
left breast (Fig-1d) demonstrated fibroadipose tissue with lobular
panniculitis-like lymphoid cell infiltrate, background fat necrosis,
karyorrhexis, and histiocytes containing karyorrhectic debris. Atypical
lymphoid cells were mildly enlarged and have irregular and
hyperchromatic nuclei. A helpful diagnostic feature was the rimming of
atypical lymphoid cells surrounding the individual adipocytes (Fig-1e).
By immunohistochemistry, the atypical/alipotropic lymphoid cells
expressed TCR beta-F1, CD8, and cytotoxic molecules (TIA1 and
granzyme-B) were negative for CD4 and CD56. Epstein-Barr-encoding region
in-situ hybridization was negative. PCR analysis showed TCRD gene clonal
rearrangement. The overall findings were in keeping with a diagnosis of
SPTCL. The expression of beta-F1 by the atypical cells and negative
staining for CD56 facilitated the distinction from primary cutaneous
gamma/delta (γ/δ) T-cell lymphoma. Additionally, the negative staining
for CD30 together with morphology didn’t support diagnosis of primary
cutaneous-Anaplastic large cell lymphoma (ALCL).
Subsequently, the patient developed a new lesion in the right breast.
FDG PET-CT (Fig-2a,b,c) was performed and revealed asymmetrical
increased activity predominantly affecting the subcutaneous soft tissue
of the left breast (SUVmax-8.7) and left axilla (SUVmax-10.5) as well as
hypermetabolic axillary lymph nodes. Some patchy subcutaneous soft
tissue activity was also noted in the medial aspect of the right breast,
concerning for bilateral disease.
The oncology team discussed the diagnosis and management plan with the
patient and family and started immunosuppressive therapy with prednisone
(60mg/m2/day PO divided BID) and cyclosporin (6mg/kg/day PO divided
BID). CT scan of the neck and chest was repeated after four weeks of
therapy showed resolving left breast cutaneous and subcutaneous
thickening and inflammatory-like changes as well as resolved left
axillary lymphadenopathy. The prednisone dose was tapered to 30mg/m2/day
divided BID. Repeat FDG-PET/CT (Fig 2c,d,e) for response assessment at
8-weeks of the therapy revealed significant improvement, SUV max falls
from 8.7 to 3.5 in the left breast and from 10.5 to 3.02 in the left
axilla. No activity was perceived in the right breast. Currently, the
patient is on tapering doses of prednisone with a goal to decrease it to
2.5 mg daily to complete one year of therapy.