Primary T-cell Lymphoblastic Lymphoma Research Articles

Primary orbital precursor T-cell lymphoblastic lymphoma: Report of a unique case.

Primary T-cell lymphoblastic lymphoma (T-LBL) in the eye region is very rare. The present study described a unique case of T-LBL involving the extraocular muscles. A 22-year-old male patient presented with a 3-week history of headache, reduced visual acuity and edema of the left eye. Clinical examination revealed left-sided exophthalmus, periorbital edema, chemosis, and reduced motility of the left eye. A magnetic resonance imaging scan revealed thickening of the left orbital muscles and a positron emission tomography-computed tomography scan also demonstrated activity in a subclavicular lymph node. Histopathological analysis of both lesions revealed infiltration by medium-sized neoplastic lymphoid cells with a high nuclear-cytoplasmic ratio and a high mitotic index. Immunostaining revealed positivity for CD2, CD3, CD99, Tia-1, and GranzymB, and variable positivity for CD4. There was no involvement of the bone marrow. Based on the clinical and histopathological findings, a diagnosis of T-LBL was made. There was no evidence of NOTCH1 mutation or rearrangements of the ETV6 and MLL genes and high-resolution array-based comparative genomic hybridization (arrayCGH) analysis revealed a normal genomic profile. The patient received chemotherapy according to the high-risk NOPHO protocol, followed by myeloablative allogenic bone marrow transplantation. At 35 months after diagnosis, the patient remained in complete first remission, but without light perception on his left eye. To the best of our knowledge, this is the first report of a case of T-LBL involving the extraocular muscles. Although primary T-LBL in the eye region is very rare, our findings demonstrate that lymphoma should be considered in the differential diagnosis of patients with similar symptoms.

T‐cell lymphoblastic lymphoma/leukemia presenting as a pituitary mass lesion: A case report and review of the literature

We present a rare case of primary T-cell lymphoblastic lymphoma of the pituitary gland. A 58-year-old woman presented with headaches, right-sided ptosis and cranial nerve III palsy. She subsequently developed polyuria, polydipsia, and hyperglycemia and was found to have hypopituitarism. MRI revealed a large, heterogeneously enhancing intrasellar/suprasellar lesion displacing the optic chiasm and extending into the right cavernous sinus. Radiologically, these findings were thought to represent an invasive pituitary adenoma. Pterional craniotomy was performed with subtotal tumor resection. Histopathological examination revealed a T-cell lymphoblastic lymphoma/leukemia (T-LBL) admixed with pituitary corticotrophic cell hyperplasia. CT scans of the chest, abdomen and pelvis showed no evidence of systemic disease. Analysis of peripheral blood and bone marrow, including flow cytometry, demonstrated no involvement by T-LBL. Follow-up MRI of the spine revealed abnormalities in the distal thoracic spinal cord and conus medullaris, raising suspicions of leptomeningeal dissemination. Only five case reports of T-cell primary pituitary lymphoma (PPL) have been previously described, four of which were associated with hypopituitarism and/or concurrent pituitary adenoma. We present the first report of a T-cell PPL associated with adenohypophyseal hyperplasia and the third documented occurrence of a primary pituitary T-LBL.

Primary T-cell lymphoblastic lymphoma of the cavernous sinus

The rare occurrence of T-cell lymphoblastic lymphoma as a primary tumor in the cavernous sinus is described. The patient, a 17-year-old girl, presented with right-sided ophthalmic and maxillary neuropathy and diplopia due to neuropathies of cranial nerves III and VI. An enhancing mass in the cavernous sinus was identified on MR imaging. Dexamethasone was prescribed but did not provide symptomatic relief. Rapid progression of symptoms led to open biopsy, and a diagnosis of T-cell lymphoblastic lymphoma was made. The patient promptly underwent aggressive chemotherapy in which a modified hyper-cyclophosphamide, vincristine, and dexamethasone without doxorubicin regimen with concurrent radiotherapy was used. The patient achieved complete remission and is currently completing the 2-year maintenance phase of chemotherapy.