This is a 66 years old woman who had a left modified radical mastectomy in 1994 for infiltrating ductal carcinoma followed by post-operative chemotherapy. In 1999 she underwent breast reconstructive surgery with insertion of a saline filled breast implant. The implant was symptom free until March 2006 when the patient started to complain of pain at the site of the implant with deviation of the implant toward the axilla.
On examination there were no signs of infection, breakdown or ulceration but there was tenderness and some erythema. The implant was properly sized with grade II capsular contraction. There was no evidence of any mass along the mastectomy scar or any mass outside the capsule or axillary adenopathy. Mammogram and breast ultrasound did not show any evidence of recurrent malignancy but did show partial deflation of the implant with capsular contraction.
Taking into account the pain and the capsular contraction, the patient underwent capsulectomy with removal of the implant and insertion of a new implant. At the time of operation, an irregular mass with some necrosis was identified medially at 9 o'clock position. Intraoperative biopsy was taken and a frozen section was done. Frozen section showed large pleomorphic cells consistent with malignancy; hence the surgeon proceeded with excision of the whole mass with removal of the implant and the capsule on which a diagnosis of Primary ALCL was made.
Radiological studies of the chest, abdomen and the pelvis were clear. Bone marrow core examination was negative.
The patient was treated with three cycles of CHOP chemotherapy regimen and also received external beam field radiation. The patient is alive until the current date with no evidence of disease.
The excision specimen of the left breast capsulectomy specimen was fixed in 10% buffered formalin and sections from paraffin wax embedded tissue blocks were stained with hematoxylin & eosin (H&E).
Immunohistochemistry was performed using standard labeled Strep-Avidin-Biotin Technique (LSAB) using the following antibodies: LCA, AE1AE3, CAM5.2, EMA, CK7, CK20, 34BE12, Vimentin, CD20, CD79A, CD3, CD5, CD7, CD4, CD8, CD15, CD30, CD1A, CD21, CD23, ALK-1, Myeloperoxidase, CD117, EBV-LMP, Fascin, CD56, IgA, CD34, CD31, Factor VIII, S-100 protein, PNL2, Melanoma Cocktail, CD68, PLAP, Desmin, Alpha-1 Antitrypsin, Alpha-1 Antichymotripsin, Factor X111A.
Molecular analysis was performed for B and T cell gene rearrangement using standard polymerase chain reaction (PCR).
Microscopic examination of the H&E stained sections showed diffuse sheets of large atypical cells having bulky eosinophilic to foamy/vacuolated, amphophilic cytoplasm. The nuclei were large and pleomorphic with vesicular chromatin and prominent one to two nucleoli. Numerous pleomorphic multinucleate giant cells, binucleate cells, mononucleate cells and cells with horseshoe-shaped and reniform nuclei with prominent nucleoli were present (Figs 1A, B, C, D). There was brisk mitotic activity and areas of necrosis. The tumor cells were infiltrating adipose tissue but did not demonstrate angiotropism or angiodestruction. The background showed numerous histiocytes, eosinophils, plasma cells and neutrophils. Based on the morphological features, the differential diagnosis included sarcomatoid/anaplastic carcinoma, malignant melanoma, pleomorphic sarcoma NOS and hematological neoplasms such as Hodgkin's lymphoma and the rare ALCL.
Immunohistochemistry demonstrated that the large atypical neoplastic cells were focally positive for LCA (Fig 2A) and EMA (Fig 2B) and strongly positive for CD30 (Fig 2C) and Vimentin. The tumor cells were negative for all other markers including ALK-1 (Fig 2D). The background reactive lymphocytes showed positivity for T-cell markers with few cells showing positivity for B-cell markers. The histiocytes stained positive for histiocytic markers.
Molecular analysis by PCR on DNA extracted from the paraffin embedded tissue showed a monoclonal T-cell gene rearrangement for the joining and variable regions of the T-cell gamma receptors with V-gamma I and V-gamma II primers.
Based on the histology and ancillary tests a diagnosis of primary ALK negative ALCL, T-cell phenotype was made on the specimen and other entities considered in the differential diagnosis were excluded.