Int J Hematol (2014) 99:790–793 DOI 10.1007/s12185-014-1582-2

CASE REPORT

Primary B-lymphoblastic lymphoma of gallbladder involving mandibular bone Hee-Jun Kim • Tae Jin Lee • Yoo Shin Choi

Received: 1 October 2013 / Revised: 7 April 2014 / Accepted: 7 April 2014 / Published online: 1 May 2014 Ó The Japanese Society of Hematology 2014

Abstract We report the case of a 75-year-old man who presented for evaluation of painless hematuria persisting for more than 1 month. At the time of presentation, the patient did not report any systemic symptoms and had no fever, weight loss, or dysuria. Computed tomography showed several enhancing, sessile polyps in the gall bladder (1.5 cm or smaller). There was no associated stone or biliary dilation. Since no other abnormality was evident, we performed laparoscopic cholecystectomy. He was diagnosed as having B-cell lymphoblastic lymphoma (BLBL) after surgical resection of the gall bladder (GB). As the left mandibular swelling was developed after the diagnosis of the B-LBL involving GB, facial magnetic resonance imaging (MRI) was added to the imaging scan. Facial MRI revealed mass formation in the left mandible, left medial pterygoid, masticator, and buccinator muscles. The biopsy samples from the mandibular bone were also diagnosed as B-LBL. The definitive pathological diagnosis was B-LBL, stage IV. Systemic chemotherapy was done with subsequent response in size of the left mandible mass.

Keywords B-cell lymphoblastic lymphoma (B-LBL)  Gall bladder (GB)  Mandible

Introduction

H.-J. Kim Division of Hemato-Oncology, Chung-Ang University College of Medicine, Seoul, Korea

Lymphomas presenting as gallbladder tumors are uncommon and may occur as de novo or secondary as a part of systemic disease. Primary lymphoma of the gallbladder is an extremely rare disease, accounting for 0.3 % of all gallbladder neoplasms [1]. Several types of lymphomas occur in the gallbladder such as diffuse large B-cell lymphoma and follicular lymphoma. Lymphoblastic lymphoma (LBL) is a highly aggressive subtype of nonHodgkin’s lymphoma (NHL), and it accounts for about 2 % of all NHLs [2]. The natural history of LBL has not been well defined and it is known that B-cell phenotype of LBL has a predilection for children [3]. Clinically, extranodal sites are frequently affected: skin (33 %), lymph node (22 %), bone (19 %), and mediastinum (5 %) [4]. On the other hand, infiltration of the neoplastic cells into the visceral organ is relatively infrequent. We recently treated an old patient with B-lymphoblastic lymphoma who had extensive extranodal involvement including the gallbladder, vertebra, bone and regional muscle of mandible. Herein, we report a case of B-cell lymphoblastic lymphoma with extensive extranodal involvement incidentally found after cholecystectomy for gallbladder polyp.

T. J. Lee Department of Pathology, Chung-Ang University College of Medicine, Seoul, Korea

Case report

Y. S. Choi (&) Department of Surgery, Chung-Ang University College of Medicine, 102 Heukseok-Ro, Dongjak-gu, Seoul 156-755, Korea e-mail: [email protected]

A-75-year-old man presented with gross painless hematuria. The patient was referred to our center for evaluation of the cause of hematuria. He had been taking

H.-J. Kim Department of Internal Medicine, Chung-Ang University College of Medicine, Seoul, Korea

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Fig. 1 CT scan image demonstrating several enhancing, sessile polyps in the gallbladder (1.5 cm and smaller)

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B

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Fig. 2 a A dense, diffuse lymphocytic infiltration without lymphoepithelial lesion (HE, 9400), b immunohistochemical staining for CD79a (B-cell marker) showed many positive staining cells (9200), c immunohistochemical staining for TdT showed many positive staining cells (9200), d immunohistochemical staining for CD3 (T cell marker) showed a few positive staining cells (9200)

antihypertensive agents for hypertension and had no previous history of coagulopathy or genitourinary disease. Initial laboratory signs were within normal levels, except for hematuria in the routine urinalysis. Cystoscopy showed normal findings. However, abdomen and pelvis computed tomography (CT) revealed a sessile polyp in the gallbladder measuring 1.5 cm (Fig. 1). Gross hematuria disappeared spontaneously without any treatment. Based on radiologic finding of gallbladder mass exceeding 1 cm, elective laparoscopic cholecystectomy was performed without complication. Lymphadenopathy was not identified intraoperatively in other sites. Pathologic examination revealed a B-cell phenotype of lymphoblastic lymphoma of

gallbladder. Immunohistochemistry staining showed the neoplastic cells positive for TdT and CD79a, while negative for CD3 (Fig. 2). For staging work up, the patient underwent CT of the chest and neck, bone marrow (BM) biopsy, and F18-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT. As the left mandibular swelling was developed after the operation, the facial MRI was added to the imaging scan. Neck CT showed multiple lymph node enlargements in the left neck levels II and III. Facial MRI revealed mass formation in the left mandible, left medial pterytoid, masticator, and buccinator muscles (Fig. 3). PET showed an uptake in multiple foci located near the left mandibular area, right humerus, and T12 spine. There was no evidence of lymphoma involvement in the BM or cerebrospinal fluid. The biopsy samples from the mandibular bone were also diagnosed as B-cell lymphoblastic lymphoma (B-LBL). On the basis of the pathological diagnosis and clinical staging, he was diagnosed as having B-cell LBL, Ann Arbor stage IVEA. Initial treatment plan was to undergo 4 cycles of hyperCVAD regimen (hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone). After the completion of 2 cycles of hyper-CVAD, however, CT scan indicated disappearance of the tumor and whole body F18-FDG PET/CT failed to spot any hypermetabolic lesions. Unfortunately, his physical condition was deteriorated drastically by subsequent events including Pneumocystis jivorecii pneumonia, delirium, and newly diagnosed diabetes. A consensus was made among the attending physician, the patient, and his family to halt chemotherapy and have a period of watchful observation. Fortunately, he is maintaining disease-free status for 11 months after the last administration of chemotherapy.

Discussion Primary B-lymphoblastic lymphomas of gallbladder are exceedingly rare. It is uncommon and rarely presents as a

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prognostic factors. It seems that the patient in this case had responded well to the chemotherapy, although he has multiple poor prognostic factors. In conclusion, we present the first case of B-LBL in the gall bladder and multiple bones including mandible as very rare presentation. Early accurate diagnosis and aggressive chemotherapy similar to that employed in B-ALL is warranted. Delay in implementation of effective chemotherapy, along with the presence of a leukemic state, imparts a poorer prognosis. Acknowledgments This work was supported by a Chung-Ang University Research Grant. Dr. Kim receive institutional research support from Chung-Ang University.

Fig. 3 MRI of the face showing lymphoid infiltration in bone marrow and mass formation in soft tissue is noted in bone marrow of left mandible, left medial pterytoid, masticator, and buccinator muscles (arrow)

primary gallbladder tumor or solitary bone tumor. In 2001, Gravel et al. [5] reported the first case of B-LBL involving the gallbladder, which was diagnosed in a 4-year-old boy. There have been 2 additional case reports of B-LBL with involvement of gallbladder. In contrast to the previous reports that had no distant site involvement, the tumor in this case report involved not only gallbladder, but also mandibular bone, masticator, and buccinator muscles. In patients with B-LBL, the most frequently involved extranodal sites are the skin, lymph nodes, bone, mediastinum [6] and miscellaneous sites, including the parotid gland, tonsils, breast, ovary, brain, retroperitoneum, and soft tissues [7]. Involvement of B-LBL in bone lesions, such as femur and vertebra, is most frequent [8], but B-LBL in oral cavitary bone has never been reported before this one case only on mandible in adult [9]. Up to our knowledge, this is the first case of B-LBL with systemic involvement with infrequent locations such as gallbladder and oral cavitary bone. Another interesting point in this case is that B-LBL developed in an old patient. Approximately 75 % of B-LBL occurs in patients younger than 18 years [10]. For example, Lin et al. [11] reported that the median age of their 25 patients was 20 years. Occurrence of B-LBL in a 75-year-old patient is considered quite uncommon. Current chemotherapeutic modalities for lymphoblastic lymphoma are based on those used for acute lymphocytic leukemia. Hyper-CVAD is currently preferred as the most effective chemotherapeutic regimen [12]. Similar with B-ALL, advanced age, high leukocyte count, circulating blasts, elevated serum lactate dehydrogenase, increase in organ size, central nervous system involvement, and delayed response to chemotherapy are well known poor

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Conflict of interest study.

All authors had no conflict of interest on this

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Primary B-lymphoblastic lymphoma low propensity for leukemic involvement. Am J Surg Pathol. 2000;24:1480–90. 12. Kantarjian HM, O’Brien S, Smith TL, Cortes J, Giles FJ, Beran M, Pierce S, Huh Y, Andreeff M, Koller C, Ha CS, Keating MJ,

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Primary B-lymphoblastic lymphoma of gallbladder involving mandibular bone.

We report the case of a 75-year-old man who presented for evaluation of painless hematuria persisting for more than 1 month. At the time of presentati...
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