A 27-year-old Chinese female presented with a left mammary mass to the First Affiliated Hospital, Hangzhou, China, in April 2010. There was no history of fever, weight loss, night sweats or other symptoms. The patient's medical and family histories were unremarkable. Upon physical examination, a single non-tender mass was palpable at the upper outer quadrant of the left breast and an enlarged lymph node was palpable in the ipsilateral axilla. There was no cervical or inguinal lymphadenopathy. An ultrasound examination revealed a hypoechoic, non-cystic mass with ill-defined borders in the upper outer quadrant of the left breast, which measured 3.3x2.6 cm. Computed tomography (CT) scans of the abdomen, chest X-rays, a bone marrow aspiration and a biopsy revealed no significant observations. Laboratory data, including the total white cell count, lactate dehydrogenase (LDH), beta2-microglobulin and immunoglobulin levels, were normal.
A segmental resection was performed. Examination of the hematoxylin and eosin staining revealed sheets of uniform, small and medium-sized, round, blue cells, and infiltration into ducts and blood vessels in certain regions was observed. Mitotic figures and a large number of eosinophils were observed (Fig. 1). A panel of antibodies was used for an immunohistochemical study, including CD2, CD4, CD43, CD3, CD5, CD8, p53, cyclin D1, cytokeratin, vimentin, desmin, myeloperoxidase (MPO), CD79a, CD7, CD30, anaplastic lymphoma kinase (ALK), CD34, CD10, CD56, granzyme B, B-cell lymphoma 2 (BCL2), BCL6, S100, terminal deoxynucleotidyl transferase (TDT), estrogen receptor (ER), progesterone receptor (PR) and paired box (pax)5. The tumor cells were markedly positive for CD2, CD4, CD43 and CD3 (Fig. 2) and weakly positive for CD5, CD8 and p53. Certain tumor cells were cyclin D1-positive. Reactivity for cytokeratin, vimentin, desmin, MPO, CD79a, CD7, CD30, ALK, CD34, CD10, CD56, granzyme B, BCL2, BCL6, S100, TDT, ER, PR and pax5 was negative. Based on these observations, the patient was diagnosed with PTBL, unspecified. A subsequent positron emission tomography (PET)/CT scan demonstrated multiple hypermetabolic foci in the left breast and the ipsilateral axillary lymph nodes.
The patient was administered one cycle of CHOP (750 mg/m2 cyclophosphamide on day 1, 60 mg/m2 epirubicin on day 1, 1.4 mg/m2 vindesine on day 1 and 100 mg prednisolone on days 1-5, every 3-4 weeks). A chest CT scan revealed that the left breast was enlarged with multiple soft tissue masses. Subsequently, the patient received six cycles of ECHOP (120 mg/m2 VP-16 on days 1-3 plus CHOP every 3-4 weeks). Marked tumor regression was observed following the first cycle of ECHOP and a complete clinical response was achieved following the third cycle. Six months after diagnosis and two months after chemotherapy, the patient received an autologous peripheral blood stem cell transplantation. Following transplantation, a CT scan showed no evidence of recurrence.
Six months following transplantation, two left mammary masses were identified by the patient. A PET/CT scan indicated an abnormal accumulation of fluorodeoxyglucose in the left breast, the left axilla and the right ilium, indicative of recurrence and metastasis. LDH levels were elevated to 315 U/l (normal limit, 91-250 U/l). beta2-microglobulin and immunoglobulin levels remained within the normal limits. The patient underwent an excisional biopsy of the left breast. Histopathology showed that the recurrent tumor increased levels of karyopyknosis and nuclear fragmentation compared with the primary tumor. The positive expression rates of Ki67 in the recurrent tumor (~80%) were higher than in the primary tumor (~40%). The immunohistochemical results were consistent with those of the primary tumor. Subsequently, the patient received two cycles of ECHOP and one cycle of GDP (1000 mg/m2 gemcitabine on days 1 and 8, 40 mg dexamethasone on days 1-4 and 25 mg/m2 cisplatin on days 1-3, every 3-4 weeks). The recurrent tumor was resistant to the ECHOP and GDP regimen. The individual succumbed to PTBL, unspecified, 18 months following the diagnosis.
This study was approved by the institutional ethics committee of The First Affiliated Hospital, Zhejiang University. Informed consent was obtained from the patient's family.