Multiple Extranodal Sites at Presentation inNon-Hodgkin’s Lymphoma

Devinder Singh, Atul Sharma,* B.K. Mohanti, Sanjay Thulkar, Sudhir Bahadur, S.C. Sharma,and S. Dutta Gupta

All India Institute of Medical Sciences, New Delhi, India

About one-third of NHL patients present lymphomas at extranodal sites. Involvement ofmultiple noncontiguous extranodal sites at presentation without lymph node involvementis unknown. Herein we present two such cases. The first patient had sinonasal tract andurinary bladder NHL at presentation, while the other patient presented with bilateraladrenal involvement in addition to sinonasal tract NHL. Clinical presentation and patho-physiology are discussed, and a brief review of literature is included. Am. J. Hematol.74:75–77, 2003. © 2003 Wiley-Liss, Inc.

Keywords: sinonasal tract; extranodal NHL; urinary bladder; adrenal

CASE REPORT

Case 1

A 60-year-old male presented with 2-months’ historyof epistaxis, nasal obstruction, and headache. He also hadhematuria once. There were no B symptoms (fever,weight loss, night sweats). On examination, a mass wasfound in the right nasal cavity. The rest of the physicalexamination was normal. CT scan of the paranasal si-nuses (PNS) confirmed the finding of a right nasal cavitymass with extension into the nasopharynx and both eth-moid sinuses (Fig. 1). Biopsy of the nasal mass wasreported as NHL—diffuse large B-cell (DLBC) type(Fig. 2). On CT scan of the abdomen, a urinary bladdermass was detected. Cystoscopic transurethral resection ofthe bladder mass was done, the biopsy of which was alsoreported as NHL-DLBC (similar in histology to the nasalmass). Routine hemogram, biochemistry, bone marrowexamination, chest X-ray, and HIV tests were noncon-tributory. Final Ann Arbor staging was IVA with twoprimary extranodal sites. A combined modality treatmentwith chemotherapy and radiotherapy was planned for thepatient, who received 6 cycles of CHOP (standard doses)chemotherapy and IFRT to PNS 45 Gy/25 doses/5weeks. Repeat CT scan of the PNS and abdomen re-vealed complete resolution of the masses. The patientcontinues to be in complete remission 11 months aftercompletion of treatment at the time of reporting in Oc-tober 2002.

Case 2

A 60-year-old female presented with a 1-month his-tory of headache without B symptoms. On examination,a right nasal cavity mass was detected. There was nolymphadenopathy or hepatosplenomegaly. CT scan ofthe paranasal sinuses revealed a soft tissue mass in theright nasal cavity extending into the left nasal cavity,right maxillary antrum, nasopharynx, and both ethmoidsinuses. The mass was resected, and histopathology re-vealed NHL-DLBC. As a part of workup for staging, aCT scan of abdomen was performed, in which bilateraladrenal masses were detected. The patient received acombined modality treatment with chemotherapy and ra-diotherapy. Initially 6 cycles of CHOP chemotherapywere given followed by IFRT to PNS 45 Gy/25 doses/5weeks. Repeat CT scan of the PNS was normal, whereasCT scan of the abdomen showed a residual adrenal masson the right side and complete resolution of left adrenalmass. In the meantime, the patient developed multiple

Correspondence to: Dr. Atul Sharma, M.D., D.M., Assistant Professorof Medical Oncology, IRCH, All India Institute of Medical Sciences,Ansari Nagar, New Delhi 110029, India. E-mail: atul1@hotmail.com

Received for publication 18 October 2002; Accepted 15 April 2003

Published online in Wiley InterScience (www.interscience.wiley.com).DOI 10.1002/ajh.10357

American Journal of Hematology 74:75–77 (2003)

© 2003 Wiley-Liss, Inc.

nodules in skin, biopsy of which confirmed relapse ofNHL. She was given salvage chemotherapy with MINE(Mitoxantrone, Ifosfamide, VP-16), but she died of dis-ease while on treatment.

DISCUSSION

About one-third of NHL cases occur at extranodalsites, but involvement of multiple noncontiguous extra-nodal sites at presentation without lymph node involve-ment is rare. Moreover, primary urinary bladder or adre-

nal involvement in addition to sinonasal tractinvolvement at baseline has not been reported in the En-glish literature to the best of our knowledge. This makesour cases all the more interesting.

Sinonasal tract lymphomas are more common in Asiathan in Western populations and are predominantly T-cell lymphomas with angiocentric features [1,2]. Both ofour patients had the B-cell phenotype. Within the sino-nasal tract, the maxillary sinus is more commonly in-volved than the nasal cavity in Western populations, butin Asia, the nasal cavity is more commonly involved [2].In our report, the nasal cavity was the primary site in bothpatients.

Involvement of other extranodal sites at presentationin sinonasal tract NHL is very rare. However, involve-ment of lymph nodes, liver, spleen, bone marrow, orbit,stomach, testis, brain, and meninges has been mentioned[3], but in our patients, none of these sites was involvedat presentation. In one series of 70 patients with sinonasaltract (SNT) NHL by Logsdon et al., none of the patientshad urinary bladder or adrenal involvement [4]. Simi-larly, in another series of 120 cases of SNT NHL byAbbondanzo et al., none had bladder or adrenal involve-ment [5].

Among the approximately 30 cases of primary adrenalNHL reported so far, none had simultaneous SNT in-volvement [6]. Similarly, in the approximately 50 casesof primary urinary bladder NHL reported in various se-ries so far, none had SNT as another site of NHL in-volvement [7,8]. Thus simultaneous involvement of SNTalong with urinary bladder (case 1) and adrenal gland(case 2) at presentation is unique in our report.

Extranodal lymphomas in general have poorer prog-nosis than do normal lymphomas. The presence of mul-tiple extranodal sites at presentation further adds to poorprognosis. Our 1st case is disease-free after combinedmodality treatment with chemotherapy and radiotherapy,but our 2nd case had only a partial response to chemo-therapy and radiotherapy and died of disease.

Combined modality treatment with chemotherapy andradiotherapy should be offered initially with SNT lym-phomas. In stage IV disease, radiotherapy should be usedat sites of initial bulky disease in addition to localizedresidual disease after chemotherapy. Autologous bonemarrow or peripheral blood stem cell transplant shouldbe offered to patients with refractory or residual disease.

In any case of primary extranodal NHL, the patientshould be fully investigated to look for other unusualsites. CT of the chest, abdomen, and pelvis and bonemarrow examination should be done in all cases. CSFexamination needs to be done in patients with PNS, bonemarrow, or testicular involvement. In this way a greaternumber of asymptomatic sites can be detected with bettertreatment results. In our 2nd case, the adrenal mass was

Fig. 1. Photomicrograph showing nasal tissue and tumorcells (original magnification 10×).

Fig. 2. CT scan abdomen showing bilateral adrenalmasses. [Color figure can be viewed in the online issue,which is available at www.interscience.wiley.com.]

76 Case Report: Singh et al.

asymptomatic and was detected on a CT scan of abdo-men done as a part of staging work up.

REFERENCES

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2. Ng CS, Chan JK, Lo S, et al. Immunophenotypic analysis of non-Hodgkin’s lymphoma in Chinese: a study of 75 cases in Hong Kong.Pathology 1986;18:419–425.

3. Liang R, Todd D, Chan T, et al. Treatment outcome and prognosticfactors for primary nasal lymphoma. J Clin Oncol 1995;13:666–670.

4. Logsdon MD, Ha CS, Kavadi VS, et al. Lymphoma of the nasal cavity

and paranasal sinuses: improved outcome and altered prognostic fac-tors with combined modality therapy. Cancer 1997;80:477–488.

5. Abbondanzo SL, Wenig BM. Non-Hodgkin’s lymphoma of the sino-nasal tract: a clinicopathological and immunophenotypic study of 120cases. Cancer 1995;75:1281–1291.

6. Wu HC, Shih LY, Chen TC, et al. A patient with bilateral primaryadrenal lymphoma, presenting with fever of unknown origin andachieving long-term disease-free survival after resection and chemo-therapy. Ann Hematol 1999;78:289–292.

7. Bates AW, Norton AJ, Baithun T, et al. Malignant lymphoma of theurinary bladder: a clinicopathological study of 11 cases. J Clin Pathol2000;53:458–461.

8. Ohsawa M, Aozasa K, Horiuchi K, et al. Malignant lymphoma ofbladder: report of three cases and review of literature. Cancer 1993;72:1969–1974.

Case Report: Sinonasal Tract NHL With Other Extranodal Sites 77