Management of primary adrenal lymphoma with intracranial invasion / 中华内分泌代谢杂志

Primary adrenal lymphoma (PAL) is a rare extranodular lymphoma. It is subject to misdiagnosis due to atypical clinical and imaging features. Histopathology is required to establish the diagnosis. Patients often present to the Endocrinology Department upon revelation of adrenal incidentalomas by imaging. PAL is often accompanied by invasion of other tissues and organs, with a high mortality rate and a poor prognosis. In this paper, the clinical features of a patient with primary adrenal lymphoma and intracranial invasion were summarized, and the pathogenesis, clinical manifestations, diagnosis and treatment of this disease were reviewed.

Imaging findings of primary adrenal lymphoma and correlation with pathology / 中国医学影像技术

Objective: To explore CT, MRI manifestations of primary adrenal lymphoma (PAL) and their correlation with pathology. Methods: CT and MRI findings of 13 patients of PAL confirmed by pathology were analyzed retrospectively, among them 3 cases were confirmed by surgical operation and 10 cases by puncture biopsy, all 13 cases were diffuse large B-cell non-Hodgkin's lymphoma. The level of lactate dehydrogenase (LDH) increased in 8 cases. CT was performed in 11 cases (18 lesions), MR in 6 cases (11 lesions), and both CT and MR in 4 cases. Results: There were 22 lesions in 13 cases, 9 cases with bilateral lesions and 4 cases with unilateral lesions. The maximum diameter of the lesions was 2.60-13.40 cm. Sixteen lesions were round and oval shaped, 2 in irregular shape and 4 in adrenal shape. Eighteen lesions showed soft tissue density on CT, 9 lesions showed low signal intensity on MR T1WI and slightly high signal intensity on T2WI. CT or MR enhanced scan showed mild to moderate progressive homogeneous or uneven enhancement, of which 4 lesions showed grid enhancement in the venous phase or delayed phase of MR enhancement. Conclusion: PAL has certain imaging characteristics. Combined with the level of LDH can improve diagnostic accuracy of PAL before surgical operation.

Clinicopathology and prognosis analysis of adrenal lymphoma / 上海交通大学学报（医学版）

Objective: To investigate the clinical manifestations,pathological features and prognostic factors of patients with adrenal lymphoma. Methods: A retrospective analysis was made with the clinical and pathological data of 77 patients with adrenal non-Hodgkin's lymphoma confirmed by pathology or confirmed by imaging evidence in the Department of Hematology,Ruijin Hospital,Shanghai Jiao Tong University School of Medicine from January 2002 to June 2018. The clinical characteristics of primary adrenal lymphoma (PAL) and secondary adrenal lymphoma (SAL) were compared. Kaplan-Meier curve was used for survival analysis,and Cox regression model was used to analyze the prognostic factors. Results: Of the 77 patients,28 were PAL and 49 were SAL. The predominant pathological type was diffuse large B-cell lymphoma,accounting for 77.9%. There were 21 patients accompanied by bone and gastrointestinal involvement,respectively. The proportions of bilateral involvement (67.9%) and large mass (67.9%) were higher in the PAL patients,while the proportions of III/ stage (95.9%) and pancreas (24.5%) and bone (38.8%) involvement were higher in the SAL patients. The patients with T/NK cell-derived lymphoma or with incomplete remission after treatment had significantly shorter survival time than those with B cell-derived lymphoma or with complete remission after treatment (P=0.005,P=0.000). Conclusion: The clinical manifestations of adrenal lymphoma lack specificity. The main pathological type is diffuse large B-cell lymphoma. Bilateral involvement and large mass are more common in PAL,while advanced disease and pancreas and bone involvement are more common in SAL. T/NK cell-derived lymphoma and incomplete remission after treatment are major adverse prognostic factors.

Imaging of F-18 FDG PET/CT and follow up of bilateral invasive adrenal diffuse large B cell lymphoma mimicking adrenocortical carcinoma / 고신대학교의과대학학술지

The incidence of malignant adrenal tumors, including primary adrenal lymphoma (PAL) and adrenocortical carcinoma (ACC), is rather low. Early differentiation between ACC and PAL is necessary because the therapeutic strategies for the two differ and the prognosis of each disease is poor. Unfortunately, there is no pathognomonic characteristic of PAL, so radiologists have difficulty in differentiating it from ACC. To our knowledge, there have been only two case reports on ACC that was evaluated and followed up using F-18 FDG PET/CT. The previously reported cases were simple, involving only the bilateral adrenal glands. Here, we report on a 52-year-old man diagnosed with PAL that radiologically mimicked ACC. He had findings of heterogeneous enhancement on CT, several adjacent lymphadenopathies with internal necrosis, and abutting hepatic and bilateral crural invasion. After pathological confirmation, we monitored his prognosis using F-18 FDG PET/CT after three cycles of rituximab-CHOP and again after six cycles of treatment.

Approach to the patient with primary adrenal lymphoma combined with empty sella / 中华内分泌代谢杂志

[Summary] To discuss the clinical features and treatment of primary adrenal lymphoma( PAL) with empty sella(ES).The lymphoma of the patient was nonspecific,the levels of serum sodium, cortisol, adrenocorticotropic hormone, and gonadotropin were decreased.MRI confirmed ES, CT showed bilateral adrenal enlargement.CT-guided fine-needle aspiration biopsy of the adrenal gland revealed a cytological diagnosis of diffused large B cell lymphoma( DLBCL).The patient had received R-CHOP chemotherapy, which results in a complete functional recovery and the tumoral masses disappeared.PAL combined with ES was rarely reported, which is difficult to diagnose due to complex hormone levels.

Linfoma no Hodgkin suprarrenal primario / Primary Non Hodgkin´s Lymphoma of the Adrenal Gland

El linfoma no Hodgkin primario de la glándula suprarrenal es una patología muy poco común. Su diagnóstico inicial es difícil siendo este histológico. El linfoma B difuso de células grandes es el tipo histológico más frecuente. Se presenta el caso mujer de 62 años a la que se le realiza tomografía axial computarizada abdominal que muestra una masa de 18 cms dependiente de la glándula suprarrenal y con características de carcinoma. Se realiza exéresis de la tumoración, siendo la anatomía patológica linfoma difuso de célula grande B. Este tipo de linfoma tiene mal pronóstico describiéndose supervivencias medias en torno a los 13 meses. No existe un régimen terapéutico bien definido; aunque el tratamiento más aceptado es el esquema R-CHOP. El papel de la radioterapia y de la exéresis tumoral no está claramente establecido. El linfoma suprarrenal primario debe tenerse en cuenta en el diagnóstico diferencial de pacientes que presentan una masa suprarrenal. Ante la sospecha de esta patología es preferible realizar una biopsia con aguja guiada por prueba de imagen para evitar una intervención quirúrgica innecesaria(AU)

Primary non- Hodgkin`s lymphoma of the adrenal gland is very rare. Its initial diagnosis is difficult and needs to be histological. Diffuse large B- cell lymphoma is the most common histological type. This is the report of a 62 years old woman, who was performed abdominal tomography to disclose a 18 cm tumor in the adrenal gland with carcinoma characteristics. The tumor was removed and the final pathologic diagnosis was diffuse large B-cell lymphoma. This type of lymphoma has poor prognosis since the average survival rates are roughly 13 months. There is no well-defined therapeutic regimen, although the most widely accepted treatment is R-CHOP scheme. The roles of radiotherapy and tumor resection are not clearly established. Primary adrenal lymphoma should be considered in the differential diagnosis of patients presenting with an adrenal mass. When this condition is suspected, then it is advisable to perform a imaging-guided needle biopsy to avoid unnecessary surgery(AU)

Image and pathology features of primary adrenal lymphoma / 中华泌尿外科杂志

Objective To evaluate the CT features of primary adrenal lymphoma and its relationship with pathology.Methods Pathologically proven primary adrenal lymphoma 6 cases were reviewed.There were 4 males and 2 females.The age was 18-62 years,average age was 51 years.5 cases was single tomur in unilateral,1 case was bilateral disease.The main clinical manifestations were abdominal pain,abdominal discomfort.All patients had CT scan and were treated surgically.Results CT scan showed the characters of adrenal little low-density soft tissue mass:the maximum diameter of 3-11 cm,irregularly shaped or ovalshaped,multi-state a clear,homogeneous or slightly inhomogeneous density,CT value was 30-40 HU.Lesions could be embedded or close to blood vessels and the ipsilateral kidney.The lesions were not enhanced arterial phase enhancement,CT value was 39-50 HU.The lesions showed vein phase of mild to moderate enhancement,CT value was 47-66 HU.At the delay of continuing to strengthen phase,CT value was 60-78 HU.The pathology charaters showed that:Diffuse of tumor cells under light microscope dense,more uniform size,and a large,granular chromatin,tumor stromal components was relatively small.There was no significant bleeding,necrosis and calcification.one lesion showed sheet,tumor cell necrosis.Conclusions CT scan characteristics and pathological features of primary adrenal lymphoma might have a certain correlation.

Linfoma adrenal primário apresentando-se como incidentaloma adrenal: relatode caso e revisão da literatura / Primary adrenal lymphoma presenting as an adrenal incidentaloma: case report and review of the literature

Introdução: O linfoma primário de glândulas adrenaisé um evento raro. Devido à agressividade dessetumor, a forma mais comum de apresentação é a insuficiênciaadrenal primária, sendo muito infrequente aapresentação na forma de um incidentaloma adrenal.Incidentalomas adrenais correspondem a tumores sóidentificados após a realização de exames de imagempara esclarecimento de alguma anormalidade semorigem, a princípio, nas glândulas adrenais. Objetivo:Descrever o caso de um paciente de 61 anos portadorde linfoma difuso de grandes células B com acometimentoprimário bilateral de glândulas adrenais, este seapresentando na forma de um incidentaloma adrenal.Material e Métodos: Relato do caso, revisão de examese discussão baseada na literatura. Os artigos selecionadospara a discussão foram buscados no PubMed edatam dos últimos 12 anos. Resultados: Paciente masculino,61 anos, previamente hígido, apresentou-se emconsulta ambulatorial com queixa de dor abdominaldifusa, perda de peso e astenia associados. Exames deimagem evidenciaram aumento volumétrico de ambasas adrenais. Não havia sintomas ou sinais clínicos sugestivosde hipofunção ou hiperfunção adrenal e osexames laboratoriais iniciais descartaram a presençade insuficiência adrenal, feocromocitoma, hiperaldosteronismoprimário e síndrome de Cushing. O pacienteevoluiu com queda do estado geral, vômitos e confusãomental, sendo diagnosticada crise adrenal, confirmadaapós laboratorialmente. O estudo histológico evidenciouneoplasia maligna indiferenciada, sendo definidaa presença de linfoma difuso de grandes células B naavaliação imunohistoquímica. Conclusões: Apesar deser uma condição rara, o linfoma primário de glândulasadrenais deve ser lembrado como um possível diagnósticono contexto de incidentaloma adrenal.

Introduction: Primary adrenal lymphoma is a rare event,Due to the agressiveness of this tumor, the most commonform of presentation is primary adrenal insufficiency ?presentation as an adrenal incidentaloma is very unusual.Adrenal incidentalomas are turmors only indentified afterradiologic evaluation in order to clarify some abnormalitythat is not originated, at the first instance, in adrenalglands. Objective: To describe a case of a 61-year-old patientwith lymphoma diffuse large B-cell involving adrenalglands bilaterally and presenting as an adrenal incidentaloma.Methods: Case report, review of tests and discussionbased on the literature. The articles choosen to thediscussion were searched in PubMed and date from thelast twelve years. Results: A 61 year-old male patient, helathypreviously, presented in outpatient service with diffuseabdominal pain, wheight loss and astenia associated.Imaging studies revealed an increase in volume of bothadrenal glands. There were no clinical signs or symptonssuggestive of adrenal hipofunction or hyperfunction andinitial laboratorial tests ruled out the presence of adrenalinsufficiency, pheocromocytoma, primary aldosteronismand Cushing?s syndrome. The patient developed then adecline in general status, emesis and mental confusionand an adrenal crisis was confirmed. Histologic study ofthe adrenal mass demonstrated an undifferenciated malignantneoplasm and the immunohistochemical evaluationshowed a large B-cell diffuse lymphoma. Conclusions:Despite being a rare condition, primmary adrenal lymphomashould be considered as a possible diagnosis in theclinical context of an adrenal incidentaloma.

A Case of Primary Adrenal Diffuse Large B-cell Lymphoma Achieving Complete Remission with Rituximab-CHOP Chemotherapy

Primary adrenal lymphoma is a very rare extranodal lymphoma; its clinical features consist of a high incidence of bilateral adrenal involvement and diffuse large B-cell lymphoma. We report a patient with primary bilateral adrenal diffuse large B-cell lyphoma who achieved complete remission with R-CHOP (rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy. A 52-yr-old man presented with fever and progressive fatigue for 3 months. Computed tomography (CT) scans of the abdomen and pelvis demonstrated large bilateral adrenal masses, and a needle biopsy of the left adrenal mass revealed diffuse large B-cell lymphoma. After 6 cycles of R-CHOP chemotherapy, CT scans showed no residual disease. To our knowledge, this is the second report to date of a patient with primary bilateral adrenal diffuse large B-cell lymphoma who achieved complete remission using R-CHOP chemotherapy.

Bilateral Adrenal Gland Lymphoma Masquerading as Vogt-Koyanagi-Harada Syndrome

PURPOSE: To report a case of bilateral adrenal gland lymphoma mimicking symptoms of Vogt-Koyanagi-Harada syndrome. CASE SUMMARY: A 57-year-old woman complained of decreased visual acuity and metamorphopsia in both eyes. She had no prior medical history, but had experienced headaches in the past week. Fundus examination revealed multiple serous retinal detachment. Fluorescein angiography of the fundus revealed multiple pinpoint leaks in early stages and subretinal dye pooling in the retinal detachment area in late stages. The most likely diagnosis was therefore Vogt-Koyanagi-Harada syndrome. Two days later, mild fever developed and a systemic evaluation was performed. Computed tomography of chest and abdomen showed bilateral adrenal gland T-cell lymphoma. Through prompt combination chemotherapy, the patient's systemic condition recovered, and her fundus recovered with improved visual acuity. CONCLUSIONS: We experienced a rare case of bilateral adrenal lymphoma which masqueraded as Vogt-Koyanagi-Harada syndrome. When there are systemic symptoms such as mild fever and headaches, systemic evaluation should be done so as not to miss more serious systemic pathology.

A Case of Primary Bilateral Adrenal Lymphoma (PAL) with Central Nervous System (CNS) Involvement

Primary adrenal lymphoma (PAL) is a very rare disease and usually does not have disease elsewhere, and if present, it is frequently extranodal. Several cases of PALs, with central nervous system (CNS) involvement, gastrointestinal tract or other endocrine organs involvement, have been reported in Western literature. We experienced a case of PAL with CNS involvement, which was previously unreported in Korea. The patient, a 61 year-old male, was admitted with left abdominal pain. After imaging study and needle aspiration biopsy (NAB), a bilateral primary adrenal lymphoma (large B-cell type), with bilateral hemorrhage, was confirmed. Combination chemotherapy, with CEOP regimen, was used. A follow-up abdomino- pelvic CT scan, after the second CEOP chemotherapy, showed the previous adrenal masses had nearly disappeared. However, about 2 months after diagnosis (day 52), he showed recent memory loss, obtundation, disorientation and drowsy mental status. The brain MRI revealed multifocal scattered lesions with increased signal intensity. The result of a brain biopsy was diffuse large B-cell lymphoma. After 5(1/2) cycles of chemotherapy, with CEOP regimen, and whole brain radiotherapy, he is still surviving 6 months later, and has become alert with nearly normalized cognitive function.

A Case of Primary Bilateral Adrenal Lymphoma (PAL) with Central Nervous System (CNS) Involvement

Primary adrenal lymphoma (PAL) is a very rare disease and usually does not have disease elsewhere, and if present, it is frequently extranodal. Several cases of PALs, with central nervous system (CNS) involvement, gastrointestinal tract or other endocrine organs involvement, have been reported in Western literature. We experienced a case of PAL with CNS involvement, which was previously unreported in Korea. The patient, a 61 year-old male, was admitted with left abdominal pain. After imaging study and needle aspiration biopsy (NAB), a bilateral primary adrenal lymphoma (large B-cell type), with bilateral hemorrhage, was confirmed. Combination chemotherapy, with CEOP regimen, was used. A follow-up abdomino- pelvic CT scan, after the second CEOP chemotherapy, showed the previous adrenal masses had nearly disappeared. However, about 2 months after diagnosis (day 52), he showed recent memory loss, obtundation, disorientation and drowsy mental status. The brain MRI revealed multifocal scattered lesions with increased signal intensity. The result of a brain biopsy was diffuse large B-cell lymphoma. After 5(1/2) cycles of chemotherapy, with CEOP regimen, and whole brain radiotherapy, he is still surviving 6 months later, and has become alert with nearly normalized cognitive function.

A case of primary bilateral adrenal lymphoma with partial adrenal insufficiency

Unilateral or bilateral non-Hodgkin's lymphomas arising primarily in the adrenal glands are extremely rare. These lymphomas are usually present with large, bilateral adrenal masses with or without lymphadenopathy, and may be accompanied by adrenal insufficiency in some cases. A review of the literature indicates that patients with primary lymphoma of the adrenal glands usually do not have disease elsewhere, and if present, it is frequently extranodal. We report here an unusual case of primary bilateral adrenal lymphoma with partial adrenal insufficiency.