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.