Besides or in addition to breast swelling, patients present with a breast mass in 30% present of cases, enlarged lymph nodes in the armpit or around the chest clavicle bones in 20% of cases, and/or in a small percentage of cases lesions in more distant tissues. Rarely, patients have presented with skin rash or itching on or around the involved breast. Using the Ann Arbor staging system, 83% of patients present with Stage 1 localized disease while the remaining 10, 0, and 7% of patients present with what is normally regarded as more aggressive Stage II, III, or IV diseases, respectively. Thus, about 17% of individuals present with a more aggressive disease that has spread from its original breast implant site to nearby lymph nodes, to areas outside of the capsule, or to more distal tissues. Diagnosis
In most individuals with BIA-ALCL, the affected breast has a thickened capsule around the implant and effusion fluid between the capsule and implant. Neoplastic cells are located in and typically limited to the capsule and effusion. Histological examination of the capsules shows large anaplastic cells but cells with all the features of ALCL "hallmark" are often difficult to detect. In addition to these neoplastic cells, the capsule lesions contain, sometimes in a large excess that makes diagnosis difficult, a variety of non-malignant cells such as small lymphocytes, histiocytes, and granulocytes (the granulocytes are mostly eosinophils).
Chemotherapy treatments containing both anthracycline and etoposide were associated with superior overall survival rates (3- and 5-years of 76% and 69%, respectively) compared to chemotherapy treatment regiments containing an anthracycline but not etoposide (3- and 5-year overall survival rates of 56% and 44%, respectively). Progression-free survival rates with the latter two types of chemotherapy treatment regimens were not appreciably different. Primary cutaneious anaplastic large cell lymphoma
Signs and symptoms
pcALCL is the second most common lymphoma in the category of Cutaneous T cell lymphoma cutaneous T cell lymphomas that includes lymphomatoid papulosis, various borderline CD30-positive cutaneous T cell lymphomas, and mycosis fungoides. The median age at diagnosis for pcALC is 61 years. The disease has a male predominance and appears to be more common in Caucasian populations. Individuals with pcALCL typically present with reddish masses that initially appear in the skin or, much less frequently, the lymph nodes or various organs. These masses are nodules or tumors that are often ulcerated, greater than 2 cm in diameter, and localized to a single site. In 20% of cases, however, they occur in multiple sites. In about 10% cases followed for many years, pcALCL presenting as skin lesions progresses to extracutaneous sites, mainly to regional draining lymph nodes. Over these same long periods, however, the diseases lesions partially regress in about 50% of cases but then relapse in about 40% of cases. Diagnosis
pcALCL lesions exhibit large malignant T-cells or null cells (i.e. cells lacking many T-cell receptor proteins) with "Hallmark" cell features of anaplasia, pleomorphism, and kidney- and horse shaped-nuclei.