Precursor B cell lymphoblastic lymphoma presenting as periorbital swelling

Abstract

An 11-year-old girl was admitted for further investigation as to the cause of her bilateral papilloedema and periorbital swelling. She had a 2-week history of headache and unilateral eyelid swelling, and a 2-day history of right-sided groin swelling. CT and MRI scans revealed soft tissue adjacent to the lateral orbital walls within the extraconal lateral aspects of both orbits, more on the right than the left. The scans also revealed extensive lymphadenopathy above and below the diaphragm. The patient underwent bone marrow studies and biopsy of the lymph node in her groin. The results revealed normal bone marrow with no evidence of malignancy. The lymph node histology confirmed malignant lymphoma in keeping with B cell lymphoblastic lymphoma. The patient was started on the UKALL 2011 chemotherapy trial.

Hypodiploid B-Lymphoblastic Leukemia Presenting as an Isolated Orbital Mass Prior to Systemic Involvement: A Case Report and Review of the Literature

“…The actual incidence of orbital involvement in ALL is unknown, and the only consensus is that it is a rare extramedullary manifestation of ALL. Our literature search revealed seven reported cases of hematogenous masses at the initial presentation of childhood ALLs, ranging from 8 months to 11 years of age [ 8 , 9 , 10 , 11 , 12 , 13 , 14 ]. Unlike our case, these reports had systemic symptoms such as intermittent fevers, lymphadenopathy, or rhinorrhea, or abnormal CBC at presentation ( Table 1 ).…”

A case of systemic precursor T-cell lymphoblastic lymphoma presenting with single tooth mobility

“…4 However, since 2008 World Health Organization (WHO) unified LBL and ALL under the term precursor B-cell and T-cell lymphoblastic leukemia/lymphoma based on new immunogenetic, morphological, and molecular characteristics. 4,5 Precursor T-cell LBL is most common in teens or young adults compared to precursor B-cell LBL which is more common in children or young adults. 6 In general, LBLs predominantly affect males with an overall male-to-female incidence ratio of 2.5:1.…”

“…6 In comparison, 75% of precursor B-cell LBL cases develop lesions in extranodal organs including skin, breasts, liver, and bone, as well as the lymph nodes. 5,6 Apart from bone marrow, LBLs have also a predilection for central nervous system. 5 Precursor T-cell LBL has a poorer prognosis compared to precursor B-cell LBL, with an overall 5-year survival rate as low as 26%.…”

“…5,6 Apart from bone marrow, LBLs have also a predilection for central nervous system. 5 Precursor T-cell LBL has a poorer prognosis compared to precursor B-cell LBL, with an overall 5-year survival rate as low as 26%. 6 Since 1970, when the first case report on this topic was published, the most frequently reported lymphoma type in head and neck is diffuse large B-cell lymphoma, associated mainly with older males (in their seventh decade of life).…”

“…8,9 Other sites are the orbit, nasal cavity, paranasal sinuses, oral cavity (palate, gingiva, and tongue), salivary glands, mandible, and thyroid. 5,8,9 There have also been reports of "double-hit" NHLs that arise in more than one intraoral site and are associated with poorer prognosis. 10 In this article, we describe an interesting case of systemic precursor T-cell lymphoma that was initially presented with single tooth mobility and a panoramic X-ray that failed to reveal the extent of mandibular bone destruction.…”