Associated Actinomycosis and Rhinopharyngeal
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Le Infezioni in Medicina, n. 3, 164-172, 2008 Casi clinici Associated actinomycosis and rhinopharyngeal Case reports adenocarcinoma during HIV infection: diagnostic and therapeutic issues Associazione patologica tra actinomicosi ed adenocarcinoma del rinofaringe in corso di infezione da HIV. Aspetti diagnostici e terapeutici Sergio Sabbatani1, Ciro Fulgaro1, Gino Latini2, Marcellino Burzi3, Roberto Manfredi1 1Department of Infectious Diseases, “Alma Mater Studiorum” University of Bologna, S. Orsola-Malpighi Hospital, Bologna, Italy; 2Department of Otolaryngology, Maggiore General Hospital, Bologna, Italy; 3Department of Radiology, Maggiore General Hospital, Bologna, Italy ■ INTRODUCTION years, after the availability of cART [1-5]. The d- ifferential diagnosis and therefore the treatment hanks to the introduction of potent regi- of these disorders are usually delayed and ham- mens of combined antiretroviral therapy pered by co-existing disorders, especially chron- T(cART), since mid-1996 the frequency of op- ic-relapsing infectious complications, related or portunistic infections had a dramatic decline a- unrelated to the eventual, underlying HIV-asso- mong HIV-infected patients, and the life ex- ciated immunodeficiency [1-5]. pectancy of this patient population significantly In particular, among neoplastic complications increased. At the same time, due to the increased of the ear, nose, and throat (ENT) districts, mean age of HIV-infected population, and a se- those affecting the nose and the rhinopharynx ries of potential supporting factors (i.e. persist- remain somewhat rare among HIV-infected pa- ing immune system imbalance, exposure to tients, with extranodal lymphomas burdened drugs and carcinogenic substances, continued la- by a series of possible local complications, pre- tency of potentially oncogenic viruses, HIV in- vailing over squamous adenocarcinoma and fection itself), haematological and especially sol- other solid tumours, differently from the dis- id organ malignancies showed a proportional in- ease distribution found in the general popula- crease during time. As a consequence, besides tion [6-8]. the traditionally reknown AIDS-related cancers The diagnosis of tumour-like rhinopharyngeal (i.e. Kaposi’s sarcoma, non-Hodgkin’s lym- lesions remains difficult when an expected lym- phoma, and primary central nervous system lym- phocyte-monocyte hyperplasia involving the phoma, together with cervical cancer added to local lymphoid districts occurs in HIV-infected the list since the year 1993), there is a worldwide subjects, who are prone to show a reactive lym- emergence of broad-spectrum haematological phoid proliferation descending from HIV dis- and especially solid organ neoplasms, which al- ease itself, and eventual concurrent infections, so seem to occur at a younger age, and encom- like that caused by Epstein-Barr virus [6-8]. pass a greater aggressivity and a more frequent Moreover, slowly progressive infections possi- and more rapid mortality compared with the bly caused by a large spectrum of pathogenic general population [1-3]. The possible occurrence microorganisms (especially atypical mycobac- of multiple (concurrent or subsequent) malig- teria, and Mycobacterium tuberculosis), may oc- nancies, has been also reported during recent cur as space-occupying masses of the nose 164 2008 and/or the rhinopharynx [9]. They tend to ■ CASE REPORT mimick other local complications, including neoplastic ones, and remarkably complicate the A 49-year-old male ex-i.v. drug user was diag- differential diagnostic process of these lesions. nosed with HIV infection since 13 years, while Finally, similarly to the occurrence of tobacco a concurrent chronic HCV hepatitis was dis- smoking as a known risk factor of upper closed since 10 years. Due to a very low com- aerodigestive tract malignancies, also a pro- pliance to clinical and laboratory controls, both longed exposure to recreational substances (in- antiretroviral therapy and chemoprophylaxis cluding inhalation of drugs like cocaine and against the most common HIV-associated op- heroin), has been described as a possible sup- portunistic infections were denied by our pa- porting factor of rare, anecdotal cases of het- tient during his first three years of follow-up, erogeneous tumours of the nasal cavity among when active drug addiction was still present. HIV-infected subjects, with or without local su- Ten years ago, our patient was hospitalized ow- perinfections caused by the coexistence of ul- ing to the development of an AIDS-defining ill- cerative mucosal lesions, and a varied resident ness (neurotoxoplasmosis). At that time, he ex- flora colonizing the upper respiratory tract [10- perienced his nadir of absolute CD4+ lympho- 12]. With regard to the lesions of the nasal sep- cyte count (36 cells/µL only). tum and nasal cartilage and sinusal bone tis- After successful cure of this central nervous sue, when excluding congenital lesions, all ac- system opportunistic complication, our patient quired ones usually show aspecific imaging finally accepted a combined antiretroviral ther- findings also at sophisticated study techniques, apy (cART), initially conducted with didano- like X-ray films, computerized tomography sine, stavudine, and indinavir, together with a (CT) scans, magnetic resonance imaging (MRI), secondary prophylaxis for toxoplasmosis, car- and so on. ried out with cotrimoxazole, in order to prevent These lesions may be caused by a very broad a pneumocystosis, too. Despite a limited adher- spectrum of causes, including nasal trauma (in- ence to antiretroviral therapy (not exceeding volving surgical interventions and invasive di- 70-80% of recommended doses, as assessed on agnostic procedures, accidents, but also the ground of spontaneous patient’s declara- rhinotillexomania), exposure to toxic sub- tions, and monthly drug accountability carried stances (including local decongestants and in- out at our dedicated HIV outpatient service), a haled cocaine or other drugs), inflammatory progressive recovery of the CD4+ count at lev- diseases (i.e. sarcoidosis, reparative granulo- els above 200 cells/µL, allowed the discontinu- mas, Wegener’s granulomatosis, and other col- ation of cotrimoxazole prophylaxis eight lagen vascular diseases), multiple infection months after hospital discharge. Even though a (caused by bacteria, mycobacteria, fungi, or as- complete suppression of HIV viremia was sociated microorganisms), or malignancies reached after the first six months of cART, it (whose large spectrum of disorders includes was never sustained subsequently, probably carcinomas, sarcoma, Pindborg tumor, angiofi- due to the limited patient’s adherence to differ- broma, hemangioma, neuroendocrine tumor, ent, alternative regimens of cART proposed in schwannoma, and primary or secondary le- order to enhance his adherence. During the sions of lymphoproliferative disorders) [12]. subsequent laboratory follow-up, plasma HIV- Aim of our present report is to describe an ex- RNA levels ranging from 450 and 4,300 tremely rare occurrence of associated rhinopha- copies/mL were disclosed, in absence of fur- ryngeal actinomycosis and squamous adeno- ther occurrences of negative viremia. carcinoma in a HIV-infected patients treated During the further follow-up until recently, our with cART and with some specific and local patient progressively abandoned i.v. drug ad- risk factors (cigarette smoking, long-term in- diction but resorted to a “recreational”, inhala- halatory substance abuse, and a half-profes- tory use of both cocaine and heroin. Moreover, sional mushroom-truffle search and evaluation he continued a personal semi-professional hob- by smell). by regarding frequent mushroom and truffle The histopathological and imaging diagnostic search, and continued tobacco (cigarette) smok- work-up of our patient’s disease, and its thera- ing, which lasted since the age of 16 years. peutic and outcome features, are presented and Despite low adherence levels to four different discussed on the ground of the available litera- lines of cART, the stable and sufficiently satis- ture evidences. factory immune recovery, as established by a 165 2008 CD4+ count always above 400 cells/µL (above systemic broad-spectrum antibiotic treatment, 26-30% of total T-lymphocytes, with a peak val- associated with non-steroideal anti-inflamma- ue of 486 cells/µL in the year 2003), and the tory drugs and mucolytics. After repeated ENT negligible clinical history, allowed our patient specialistic consultations, our patient under- to maintain his low-adherence cART regimen, went a contrast-enhanced CT scan and MRI of until a genotypic resistance testing of 12 the face and brain, which pointed out an ag- months ago performed with rising plasma HIV- gressive form of rhinitis and sinusitis, with a se- RNA levels (5,190 copies/mL). This last viro- vere inflammatory involvement of maxillary logical assay detected multiple nucleo- and ethmoidal sinuses (Figures 1 and 2). side/nucleotide resistance mutations, complete Subsequently, a fiberoptic rhinoscopy (Figure resistance to both available non-nucleoside re- 3) with associated multiple biopsies, culture, verse transcriptase inhibitors, and multiple pro- and histopathological examination, disclosed a tease inhibitors resistance mutations, so that a predominantly necrotic tissue with abundant cART regimen including lamivudine, abacavir, fibrin deposition, and actinomycosis-like “sul- and fosamprenavir-ritonavir was introduced phur” granules surrounded by a relevant