Prognostic value of 18F-FDG PET/CT in T-Lymphoblastic lymphoma before and after hematopoietic stem cell transplantation.

PURPOSE: We aimed to evaluate the prognostic value of 18F-FDG PET/CT in patients with relapsed or refractory T-Lymphoblastic lymphoma (T-LBL) undergoing hematopoietic stem cell transplantation (HSCT). METHODS: PET/CT was performed in 21 consecutive relapsed or refractory T-LBL patients scheduled for HSCT. All PET/CT images were assessed using the Deauville criteria, and patients were divided into negative (Deauville ≤ 3) and positive (Deauville > 3) groups for comparison. The predictive value of sex, age, Ann Arbor stage, presence of B symptoms, lactate dehydrogenase level, presence of extranodal disease, and PET/CT results before and after HSCT were evaluated. RESULTS: Kaplan-Meier analysis showed that only PET/CT after HSCT (post-PET) was correlated with progression-free survival (PFS) (P = 0.030). The Cox regression model also showed that the post-PET-positive group had a higher hazard ratio (HR) than the negative group (HR = 3.884 and P = 0.049). However, none of the evaluated factors were predictive of overall survival (OS). CONCLUSIONS: Pre-PET cannot predict the PFS and OS of patients with T-LBL undergoing HSCT, which means that 18F-FDG PET/CT cannot be used for identifying patients who can benefit from HSCT. Post-PET is not predictive for OS in patients with T-LBL undergoing HSCT. However, post-PET showed strong correlations with PFS, which means that it may be useful for guiding subsequent clinical treatment decisions.

Primary Cauda Equina T-Cell Lymphoblastic Lymphoma.

BACKGROUND: T-cell lymphoblastic lymphoma (T-LBL) is a rare and aggressive form of non-Hodgkin lymphoma. This report describes, to our knowledge, the first adult case of a primary cauda equina T-LBL. Treatment consists of multiagent chemotherapy, and surgical removal of T-LBL does not improve outcome. We discuss the workup of patients with an intradural spinal mass, together with a review of the literature on primary spinal lymphoma of the cauda equina. CASE DESCRIPTION: A 54-year-old woman with Crohn's disease, for which she was taking immunosuppressive medication, presented with progressive back pain radiating to both legs and deteriorating neurologic deficits caused by an intradural, contrast-enhancing lesion in the L1-5 region. During acute surgery, the tumor was partially resected. Immunohistochemical phenotyping revealed a T-LBL. No other lymphoma localizations were found after subsequent staging. Despite extensive treatment, the patient died of disseminated disease throughout the central nervous system, 6 weeks after the diagnosis. CONCLUSIONS: Pain and progressive neurologic complaints can be symptoms of a (malignant) intradural spinal tumor. Intradural lymphoma must be considered as a differential diagnosis by clinicians because it can mimic neoplasms that often require urgent surgery. The histopathologic diagnosis should preferably be obtained by way of cerebrospinal fluid analysis or tumor biopsy because tumor resection has no beneficial effect on the oncologic outcome.

T-cell lymphoblastic lymphoma involving the ocular adnexa: report of two cases and review of the current literature.

The majority of ocular adnexal lymphomas are B-cell in origin. We report two cases of T-cell lymphoblastic lymphoma (T-LBL) involving the ocular adnexa. One patient presented with a painless pink conjunctival lesion and inferior orbital fullness. The second patient presented with a painless orbital mass. The diagnoses were confirmed by histopathology and immunohistochemistry. Both patients had extensive multifocal lesions during staging. Prompt intensified chemotherapy regimens were initiated. T-LBL is an aggressive disease with poor prognosis. This report emphasizes the importance of timely diagnosis by the ophthalmologist with co-management and treatment with an oncologist.

Nelarabine-induced peripheral and central neurotoxicity: can sequential MRI brain imaging help to define its natural history?

A 14-year-old boy with relapsed T cell acute lymphoblastic leukaemia received reinduction chemotherapy that included nelarabine, a purine nucleoside analogue known to cause dose-dependent neurotoxicity. Although he achieved aminimal residual disease negative remission after two cycles of chemotherapy he also developed severe, progressive peripheral and central neurotoxicities. Loss of grey-white differentiation was seen on a T2-weighted magnetic resonance imaging brain scan. This unusual clinical picture and previously unreported radiological findings are thought to be due to nelarabine toxicity. He was bridged with 6-mercaptopurine while transplant was deferred pending sustainable neurological improvement. This case posed clinical and ethical dilemmas while demonstrating previously unreported radiological features.

Comparison of pediatric and adult lymphomas involving the mediastinum characterized by distinctive clinicopathological and radiological features.

Lymphomas involving the mediastinum occur in a wide age range and represent heterogeneous histological subtypes with various clinical symptoms and complex radiological findings. However, studies that describe the clinical and radiological features of different subtypes among Chinese pediatric and adult patients are limited. We analyzed the clinical, radiological and pathological features of 31 pediatric lymphomas involving the mediastinum, and compared them to the features of 21 adult patients. Although several histological subtypes were identified in adults, pediatric patients presented with T-cell lymphoblastic lymphoma/T-cell acute lymphoblastic leukemia (T-LBL/T-ALL) and classical Hodgkin lymphomas (CHL) in 24 and 7 cases, respectively. Compared to adults, pediatric patients were more likely to be male (P = 0.089) and showed a higher incidence of T-LBL/T-ALL (P = 0.001), prevalence of dyspnea (P = 0.001), frequency of stage IV tumors (P = 0.008), and ratio of tumor diameter to maximum transthoracic diameter (P = 0.015). T-LBL/T-ALL patients presented with a higher frequency with stage IV disease (P = 0.000 and P = 0.001), compression of the blood vessels (P = 0.005 and P = 0.017), and pleural effusions (P = 0.001, for both) than CHL and PMBL patients. Compared to adults, pediatric patients with mediastinal lymphomas presented with exclusive histological subtypes of T-LBL/T-ALL and CHL, which showed distinctive characteristics of histological distribution, clinical presentation and radiological assessments.

A solitary uterine relapse in T-cell Acute Lymphoblastic Leukaemia: CT features and pathologic correlation.

T-cell Acute Lymphoblastic Leukemia (T-cell ALL) is a rare haematological neoplasia, that affects children and less commonly adults. Female genital tract and particularly uterus involvement in acute ALL is rare. This report presents the CT features of a 64-year-old woman with uterine relapse of T-cell ALL, occurring 11 months after the diagnosis, as a second, unique relapse of disease. The patient was asymptomatic when a CT examination showed a homogenous thickness of the uterine wall in comparison with the previous CT examination. Histology from biopsy specimens, obtained through hysteroscopy, confirmed T-cell ALL localisation (TdT+, CD10+, CD3c+ and CD2+). The uterus could be a site of relapse in patients suffering from ALL. Even though an MRI examination could better demonstrate the disease in cases of suspected female genital tract involvement by ALL, the comparison of differences between a present and a previous CT examination is sufficient to suspect the diagnosis.

[Ocular manifestations of acute T-cell lymphoblastic leukemia in hematological remission--a case report]. / Objawy oczne ostrej bialaczki limfoblastycznej T-komórkowej w stadium remisji hematologicznej--opis przypadku.

The paper presents a case of a 60 year-old female referred to the Department of Ophthalmology and Ocular Oncology, Medical College, Jagiellonian University in Krakow with the sudden severe vision deterioration in both eyes. The patient was treated for T-cell acute lymphoblastic leukemia at the local Department of Hematology, at that time she was considered to be in hematological remission. Based on findings of clinical examination and additional tests, the patient was diagnosed with leukemic infiltration of the retina and optic nerve with secondary retinal detachment. Systemic and intrathecal chemotherapy as well as local radiotherapy to both eyes were administered. Ocular manifestations of T-cell acute lymphoblastic leukemia may develop in patients in hematological remission. Standard management of leukemic infiltrates involving the retina, choroid and optic nerve includes the intrathecal chemotherapy and lo- cal radiotherapy. Such therapy caused regression and cicatrization of the ocular infiltrates, but did not improve visual acuity in the described patient.

Unexpected uptake of gadoxetic acid in a hepatic metastasis from T-cell lymphoma.

We report a case of unexpected heterogeneous uptake of gadoxetic acid into a hepatic metastasis in a patient with T-cell lymphoblastic lymphoma that also lacked hypermetabolic characteristics on positron emission (PET)/computed tomography (CT) with [(18)F]fluorodeoxyglucose (FDG). Therefore, in cases of heterogeneous uptake of gadoxetic acid, infiltrative lesions must be considered.

Two entities of precursor T-cell lymphoblastic leukemia/lymphoma based on radiologic and immunophenotypic findings.

Precursor T-cell lymphoblastic leukemia/lymphoma (T-ALL/LBL) presents a mediastinal mass in one half of cases. Although the immunophenotypic features of T-ALL/LBL have been analyzed in several studies, few studies have been focused on the relationship between the anatomic distribution of lesions and immunophenotypic findings. We analyzed the clinicopathologic findings for 17 patients with T-ALL/LBL diagnosed since 1993 and whose radiologic findings were available. Data on 14 men and 3 women with a median age of 26 years (range, 10-61 years) were analyzed. On the basis of radiologic findings, the cases were divided into thymic type (n = 8) and nonthymic type (n = 9). Patients with the thymic type of T-ALL/LBL had a large mediastinal mass and minimal systemic lymphadenopathy only in the supradiaphragmatic region. Those with the non-thymic type had predominantly systemic lymphadenopathy that included infradiaphragmatic lesions. Expression of CD8 (6/7 versus 0/9) was more frequently found in the thymic type (P < .001), whereas expression of CD56 (0/7 versus 5/9) was more frequent in the nonthymic type (P = .034). In conclusion, T-ALL/LBL was divided into 2 entities, thymic type and nonthymic type, on the basis of radiologic findings and immunophenotypic features. Analysis of the expression of CD8 and CD56 would be useful for biologically classifying T-ALL/LBL into the 2 types. This study was performed in a single institution, was retrospective, and had a limited number of patients; multicenter confirmatory studies are warranted.

Orbital mass secondary to precursor T-cell acute lymphoblastic leukemia: a rare presentation.

We describe a 40-year-old woman with a history of precursor T-cell acute lymphoblastic leukemia who developed an orbital mass associated with diffuse infiltration of the paranasal sinuses. The clinical and radiologic findings suggested an orbital abscess. Examination of orbital and ethmoid sinus biopsy specimens revealed relapse of precursor T-cell acute lymphoblastic leukemia. Although orbital involvement by granulocytic sarcoma (also known as extramedullary myeloid cell tumor and chloroma) with or without concurrent acute myeloid leukemia is well described in the literature, similar presence of acute lymphoblastic leukemia of either precursor T-cell or B-cell lineage is rare.