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HIV infection initially presenting as sinonasal Burkitt's lymphoma
American Journal of Otolaryngology–Head and Neck Medicine and Surgery 27 (2006) 433 – 435 www.elsevier.com/locate/amjoto
HIV infection initially presenting as sinonasal Burkitt’s lymphoma Chih-Wei Lianga, Hsueh-Yu Lia, Kai-Ping Changa, Chi-Kuan Chenb, Ying-Lin Chen, MDa,4 a
Department of Otolaryngology-Head and Neck Surgery, Chang Gung Memorial Hospital and Chang Gung University, Taiwan, ROC b Department of Pathology, Chang Gung Memorial Hospital and Chang Gung University, Taiwan, ROC Received 17 November 2005
Abstract
Burkitt’s lymphoma is a common comorbidity of infection with the human immunodeficiency virus, but rarely the initial clinical manifestation of undiagnosed AIDS, especially for the otolaryngologist. We report the case of a patient with recalcitrant rhinosinusitis subsequently diagnosed with sinonasal Burkitt’s lymphoma and HIV. D 2006 Elsevier Inc. All rights reserved.
1. Introduction Burkitt’s lymphoma (BL) is a small, noncleaved, highly aggressive B-cell lymphoma. The incidence of BL may increase with coexisting HIV infection; however, sinonasal BL is rarely encountered as the initial defining illness of AIDS. Likewise, otolaryngologists infrequently play a major role in the diagnosis of HIV/AIDS. We report a case of sinonasal BL with symptoms mimicking chronic rhinosinusitis and a computed tomography (CT) scan demonstrating sinus inflammatory changes with extensive polyposis. The patient was subsequently shown to be HIV seropositive. In addition to the case report, which serves to increase awareness of the variety of manifestations of HIV/ AIDS, we have briefly reviewed the most common presentations of HIV infection–associated neoplasms as encountered by the otolaryngologist. Early recognition of HIV infection facilitates prompt and adequate treatment for the patient and helps prevent inadvertent infection of health care personnel. 2. Case report A 39 year-old man sought evaluation from a neurologist in January 2003 because of an escalating headache over a 2-week period. A comprehensive examination failed to reveal an apparent neurologic etiology. The patient also had nasal obstruction, orbital pain, and a mucopurulent nasal 4 Corresponding author. No. 5, Fu-Hsing St. Kuei-Shan, Tao-Yuan, Taiwan, ROC. Tel.: +886 3 3281200x3967; fax: +886 3 3979361. E-mail address: [email protected] (Y.-L. Chen). 0196-0709/$ – see front matter D 2006 Elsevier Inc. All rights reserved. doi:10.1016/j.amjoto.2006.01.013
discharge. Rhinogenic headache was suspected, and he was therefore referred to the ENT clinic for further evaluation. A thorough physical examination disclosed a bilateral polyplike mass in the nasal cavities. Swelling in the left periorbital area and mild exophthalmos were noted as well. A CT scan of the sinus displayed complete bilateral opacification of the maxillary, frontal, ethmoid, and sphenoid sinuses, with the presence of abnormal soft tissue in the nasal cavities bilaterally (Figs. 1 and 2). No evidence of an orbital wall defect was seen. The initial diagnostic impression was chronic rhinosinusitis with extensive nasal polyposis. However, intranasal neoplastic lesion was not dismissed in the differential diagnosis because of the left periorbital swelling and exophthalmos. The treatment plan included an endoscopic sinonasal exploration with biopsies of any suspicious lesions; routine functional endoscopic sinus surgery was planned absent any lesions. The patient was thus admitted for surgical intervention 3 weeks after initial presentation to the neurologist. After removal of several polyps, the sinoscopic examination revealed a gray, round tumor interspersed among benign nasal polyps. The mass differed from typical nasal polyps by its opacity and friability. The mass was biopsied, and frozen section examination suggested a lymphoma. The operation was thus terminated. More detailed pathologic evaluation of the specimen demonstrated that the mucosa was infiltrated by lymphoid cells with small round nuclei and nucleoli (Fig. 3), and malignant lymphoma, specifically BL, was highly suspected. Immunohistochemical studies verified the diagnosis of BL. Meanwhile, a HIV test revealed that the patient was seropositive. Therefore, chemotherapy (ie, cyclophospha-
434
C.-W. Liang et al. / American Journal of Otolaryngology–Head and Neck Medicine and Surgery 27 (2006) 433 – 435
Fig. 1. Coronal view of sinus CT scan revealed diffuse opacification of bilateral maxillary, frontal, sphenoid, and ethmoid sinuses.
mide, doxorubicin, vincristine, and prednisone) and highly active antiretrovial therapy were initiated. The patient began experiencing a gradual impairment of visual acuity on the left 1 week after diagnosis of HIV. Orbital CT scan showed a tumor that had invaded the bilateral orbital fossa and cavernous sinus, possibly causing left optic neuropathy, resulting in the visual impairment. Emergent radiotherapy was arranged, which the patient chose to undergo at a regional hospital. 3. Discussion Common AIDS-related neoplasms involving the head and neck include Kaposi sarcoma (KS) and lymphoma. Around one third of KS cases involve the oral cavity. Kaposi sarcoma is most commonly in the form of a patch, plaque, or nodular lesions, and may resemble granulation tissue. Typically, KS is located on the palate, gingiva, tongue, and lymph nodes. In contrast, AIDS-associated lymphoma, especially high-grade B-cell lymphoma, usually present with fever of unknown origin, night sweats, and unexplained weight loss [1,2]. Hence, patients among highrisk group with lymphomatous lesions and any of the preceding systemic symptoms should be tested for the presence of HIV infection.
Fig. 2. Axial view of sinus CT scan. The image shows soft tissue in bilateral nasal cavities, suggestive of nasal polyps.
Fig. 3. Biopsy specimen hematoxylin-eosin staining. Diffusely infiltrated lymphoid cells with small round nuclei and distinct nucleoli are visible, as well as scattered by pale-staining, benign macrophages, which gives the characteristic bstarry skyQ appearance of BL (original magnification 200).
Burkitt’s lymphoma is a highly aggressive, malignant, non-Hodgkin’s lymphoma. The incidence of BL is higher in HIV-seropositive patients, and BL accounts for 30% to 40% of all HIV-associated lymphomas [3]. Other common lymphomas associated with HIV infection are high-grade B-cell non-Hodgkin’s lymphomas, which include diffuse large B-cell lymphomas, primary effusion lymphomas, and plasmablastic lymphomas [4,5]. AIDS-associated BL is often diagnosed shortly after confirmation of HIV infection, when compared with other types of AIDS-associated lymphomas. Such patients have higher CD4+ T-cell counts, suggesting that BL occurs in less severe immunodeficiency states. HIV-associated BL mainly targets extranodal sites, especially the gastrointestinal tract. HIV-associated sinonasal involvement is rare, and in fact, a Medline search uncovered but a single case of paranasal sinus BL in a HIVpositive patient [6]. In that particular case and in distinction from the case we have reported herein, HIV infection was diagnosed before presentation of sinonasal BL. The most common presenting features of sinonasal lymphomas are nasal obstruction or other nonspecific symptoms consistent with chronic rhinosinusitis [7]. Furthermore, CT scans are sometimes unable to definitively discriminate tumors from other inflammatory processes. A complete differential diagnosis is generated by maintaining a high level of suspicion of clinical signs and symptoms, such as the unusual periorbital swelling described herein. All suspicious findings should be biopsied and analyzed with frozen sections, as was done in the present case with respect to the abnormal-appearing mass adjacent to benignappearing polyps. Frozen section analysis is of great importance in enhancing treatment decision making. Inverted papilloma, for example, is commonly found in conjunction with nasal polyps. If inverted papilloma is identified in frozen sections,
C.-W. Liang et al. / American Journal of Otolaryngology–Head and Neck Medicine and Surgery 27 (2006) 433 – 435
it should be removed in en bloc fashion to prevent recurrence. The identification of lymphoma by frozen section examination, as in our case, is a contraindication for continued surgery. Direct mass effects of BL on cranial nerves have rarely been reported in the literature. Optic neuropathy with gradual impairment of visual acuity occurred in our patient during hospitalization. An orbital CT scan had shown a tumor invading the orbital fossa and possibly compressing the optic nerve, thereby reflecting the short doubling time of BL and its potential for rapid expansion. This case should increase physicians’ awareness of the potential risks of visual impairment in patients with sinonasal BL. If the patient complains about visual impairment, an orbital CT scan is the best modality by which to image and determine the potential involvement of a sinonasal tumor, and radiotherapy should be performed in such a critical situation. As the pace of transmission of HIV infection increases, otolaryngologists will inarguably be the first health care providers to confront a rising number of patients with HIV infection–associated lesions, and for this reason alone, a high level of suspicion is mandatory. This case has illustrated occult HIV-associated BL, masked by the more
435
common chronic rhinosinusitis and extensive polyposis. Cautious assessment of suspicious signs and symptoms before and during surgery enhances prompt and optimal patient care. References [1] Lowenthal DA, Straus DJ, Campbell SW, et al. AIDS-related lymphoid neoplasia: the Memorial Hospital experience. Cancer 1988;61:2325 - 37. [2] Kaplan LD, Abams DI, Feial E, et al. AIDS-associated non-Hodgkin’s lymphoma in San Francisco. JAMA 1989;262:719 - 24. [3] Wulff EA, Simpson DM. Peripheral neuropathy associated with acquired immunodeficiency syndrome (AIDS)-related Burkitt’s lymphoma. Muscle Nerve 2000;23:1764 - 6. [4] Fais F, Fronza G, Roncella S, et al. Analysis of stepwise genetic changes in an AIDS-related Burkitt’s lymphoma. Int J Cancer 2000;88:744 - 50. [5] Beylot-Barry M, Vergier B, Masquelier B, et al. The spectrum of cutaneous lymphoma in HIV infection: a study of 21 cases. Am J Surg Pathol 1999;23:1208 - 17. [6] Schoem SR, Morton AL. Paranasal sinus Burkitt’s lymphoma in a human immunodeficiency virus (HIV) positive male. Ear Nose Throat J 1990;69:844 - 6. [7] Shohat I, Berkowicz M, Dori S, et al. Primary non-Hodgkin’s lymphoma of the sinonasal tract. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;97:328 - 31.
HIV infection initially presenting as sinonasal Burkitt’s lymphoma Chih-Wei Lianga, Hsueh-Yu Lia, Kai-Ping Changa, Chi-Kuan Chenb, Ying-Lin Chen, MDa,4 a
Department of Otolaryngology-Head and Neck Surgery, Chang Gung Memorial Hospital and Chang Gung University, Taiwan, ROC b Department of Pathology, Chang Gung Memorial Hospital and Chang Gung University, Taiwan, ROC Received 17 November 2005
Abstract
Burkitt’s lymphoma is a common comorbidity of infection with the human immunodeficiency virus, but rarely the initial clinical manifestation of undiagnosed AIDS, especially for the otolaryngologist. We report the case of a patient with recalcitrant rhinosinusitis subsequently diagnosed with sinonasal Burkitt’s lymphoma and HIV. D 2006 Elsevier Inc. All rights reserved.
1. Introduction Burkitt’s lymphoma (BL) is a small, noncleaved, highly aggressive B-cell lymphoma. The incidence of BL may increase with coexisting HIV infection; however, sinonasal BL is rarely encountered as the initial defining illness of AIDS. Likewise, otolaryngologists infrequently play a major role in the diagnosis of HIV/AIDS. We report a case of sinonasal BL with symptoms mimicking chronic rhinosinusitis and a computed tomography (CT) scan demonstrating sinus inflammatory changes with extensive polyposis. The patient was subsequently shown to be HIV seropositive. In addition to the case report, which serves to increase awareness of the variety of manifestations of HIV/ AIDS, we have briefly reviewed the most common presentations of HIV infection–associated neoplasms as encountered by the otolaryngologist. Early recognition of HIV infection facilitates prompt and adequate treatment for the patient and helps prevent inadvertent infection of health care personnel. 2. Case report A 39 year-old man sought evaluation from a neurologist in January 2003 because of an escalating headache over a 2-week period. A comprehensive examination failed to reveal an apparent neurologic etiology. The patient also had nasal obstruction, orbital pain, and a mucopurulent nasal 4 Corresponding author. No. 5, Fu-Hsing St. Kuei-Shan, Tao-Yuan, Taiwan, ROC. Tel.: +886 3 3281200x3967; fax: +886 3 3979361. E-mail address: [email protected] (Y.-L. Chen). 0196-0709/$ – see front matter D 2006 Elsevier Inc. All rights reserved. doi:10.1016/j.amjoto.2006.01.013
discharge. Rhinogenic headache was suspected, and he was therefore referred to the ENT clinic for further evaluation. A thorough physical examination disclosed a bilateral polyplike mass in the nasal cavities. Swelling in the left periorbital area and mild exophthalmos were noted as well. A CT scan of the sinus displayed complete bilateral opacification of the maxillary, frontal, ethmoid, and sphenoid sinuses, with the presence of abnormal soft tissue in the nasal cavities bilaterally (Figs. 1 and 2). No evidence of an orbital wall defect was seen. The initial diagnostic impression was chronic rhinosinusitis with extensive nasal polyposis. However, intranasal neoplastic lesion was not dismissed in the differential diagnosis because of the left periorbital swelling and exophthalmos. The treatment plan included an endoscopic sinonasal exploration with biopsies of any suspicious lesions; routine functional endoscopic sinus surgery was planned absent any lesions. The patient was thus admitted for surgical intervention 3 weeks after initial presentation to the neurologist. After removal of several polyps, the sinoscopic examination revealed a gray, round tumor interspersed among benign nasal polyps. The mass differed from typical nasal polyps by its opacity and friability. The mass was biopsied, and frozen section examination suggested a lymphoma. The operation was thus terminated. More detailed pathologic evaluation of the specimen demonstrated that the mucosa was infiltrated by lymphoid cells with small round nuclei and nucleoli (Fig. 3), and malignant lymphoma, specifically BL, was highly suspected. Immunohistochemical studies verified the diagnosis of BL. Meanwhile, a HIV test revealed that the patient was seropositive. Therefore, chemotherapy (ie, cyclophospha-
434
C.-W. Liang et al. / American Journal of Otolaryngology–Head and Neck Medicine and Surgery 27 (2006) 433 – 435
Fig. 1. Coronal view of sinus CT scan revealed diffuse opacification of bilateral maxillary, frontal, sphenoid, and ethmoid sinuses.
mide, doxorubicin, vincristine, and prednisone) and highly active antiretrovial therapy were initiated. The patient began experiencing a gradual impairment of visual acuity on the left 1 week after diagnosis of HIV. Orbital CT scan showed a tumor that had invaded the bilateral orbital fossa and cavernous sinus, possibly causing left optic neuropathy, resulting in the visual impairment. Emergent radiotherapy was arranged, which the patient chose to undergo at a regional hospital. 3. Discussion Common AIDS-related neoplasms involving the head and neck include Kaposi sarcoma (KS) and lymphoma. Around one third of KS cases involve the oral cavity. Kaposi sarcoma is most commonly in the form of a patch, plaque, or nodular lesions, and may resemble granulation tissue. Typically, KS is located on the palate, gingiva, tongue, and lymph nodes. In contrast, AIDS-associated lymphoma, especially high-grade B-cell lymphoma, usually present with fever of unknown origin, night sweats, and unexplained weight loss [1,2]. Hence, patients among highrisk group with lymphomatous lesions and any of the preceding systemic symptoms should be tested for the presence of HIV infection.
Fig. 2. Axial view of sinus CT scan. The image shows soft tissue in bilateral nasal cavities, suggestive of nasal polyps.
Fig. 3. Biopsy specimen hematoxylin-eosin staining. Diffusely infiltrated lymphoid cells with small round nuclei and distinct nucleoli are visible, as well as scattered by pale-staining, benign macrophages, which gives the characteristic bstarry skyQ appearance of BL (original magnification 200).
Burkitt’s lymphoma is a highly aggressive, malignant, non-Hodgkin’s lymphoma. The incidence of BL is higher in HIV-seropositive patients, and BL accounts for 30% to 40% of all HIV-associated lymphomas [3]. Other common lymphomas associated with HIV infection are high-grade B-cell non-Hodgkin’s lymphomas, which include diffuse large B-cell lymphomas, primary effusion lymphomas, and plasmablastic lymphomas [4,5]. AIDS-associated BL is often diagnosed shortly after confirmation of HIV infection, when compared with other types of AIDS-associated lymphomas. Such patients have higher CD4+ T-cell counts, suggesting that BL occurs in less severe immunodeficiency states. HIV-associated BL mainly targets extranodal sites, especially the gastrointestinal tract. HIV-associated sinonasal involvement is rare, and in fact, a Medline search uncovered but a single case of paranasal sinus BL in a HIVpositive patient [6]. In that particular case and in distinction from the case we have reported herein, HIV infection was diagnosed before presentation of sinonasal BL. The most common presenting features of sinonasal lymphomas are nasal obstruction or other nonspecific symptoms consistent with chronic rhinosinusitis [7]. Furthermore, CT scans are sometimes unable to definitively discriminate tumors from other inflammatory processes. A complete differential diagnosis is generated by maintaining a high level of suspicion of clinical signs and symptoms, such as the unusual periorbital swelling described herein. All suspicious findings should be biopsied and analyzed with frozen sections, as was done in the present case with respect to the abnormal-appearing mass adjacent to benignappearing polyps. Frozen section analysis is of great importance in enhancing treatment decision making. Inverted papilloma, for example, is commonly found in conjunction with nasal polyps. If inverted papilloma is identified in frozen sections,
C.-W. Liang et al. / American Journal of Otolaryngology–Head and Neck Medicine and Surgery 27 (2006) 433 – 435
it should be removed in en bloc fashion to prevent recurrence. The identification of lymphoma by frozen section examination, as in our case, is a contraindication for continued surgery. Direct mass effects of BL on cranial nerves have rarely been reported in the literature. Optic neuropathy with gradual impairment of visual acuity occurred in our patient during hospitalization. An orbital CT scan had shown a tumor invading the orbital fossa and possibly compressing the optic nerve, thereby reflecting the short doubling time of BL and its potential for rapid expansion. This case should increase physicians’ awareness of the potential risks of visual impairment in patients with sinonasal BL. If the patient complains about visual impairment, an orbital CT scan is the best modality by which to image and determine the potential involvement of a sinonasal tumor, and radiotherapy should be performed in such a critical situation. As the pace of transmission of HIV infection increases, otolaryngologists will inarguably be the first health care providers to confront a rising number of patients with HIV infection–associated lesions, and for this reason alone, a high level of suspicion is mandatory. This case has illustrated occult HIV-associated BL, masked by the more
435
common chronic rhinosinusitis and extensive polyposis. Cautious assessment of suspicious signs and symptoms before and during surgery enhances prompt and optimal patient care. References [1] Lowenthal DA, Straus DJ, Campbell SW, et al. AIDS-related lymphoid neoplasia: the Memorial Hospital experience. Cancer 1988;61:2325 - 37. [2] Kaplan LD, Abams DI, Feial E, et al. AIDS-associated non-Hodgkin’s lymphoma in San Francisco. JAMA 1989;262:719 - 24. [3] Wulff EA, Simpson DM. Peripheral neuropathy associated with acquired immunodeficiency syndrome (AIDS)-related Burkitt’s lymphoma. Muscle Nerve 2000;23:1764 - 6. [4] Fais F, Fronza G, Roncella S, et al. Analysis of stepwise genetic changes in an AIDS-related Burkitt’s lymphoma. Int J Cancer 2000;88:744 - 50. [5] Beylot-Barry M, Vergier B, Masquelier B, et al. The spectrum of cutaneous lymphoma in HIV infection: a study of 21 cases. Am J Surg Pathol 1999;23:1208 - 17. [6] Schoem SR, Morton AL. Paranasal sinus Burkitt’s lymphoma in a human immunodeficiency virus (HIV) positive male. Ear Nose Throat J 1990;69:844 - 6. [7] Shohat I, Berkowicz M, Dori S, et al. Primary non-Hodgkin’s lymphoma of the sinonasal tract. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;97:328 - 31.