Diagnostic and management challenges of atypical sinonasal non-Hodgkin lymphoma in a patient with acquired immunodeficiency syndrome: a case report

¹Cameroon Baptist Convention Health Services, Bamenda, Cameroon, ²Nkwen Baptist Hospital, Ear Nose and Throat (ENT) Department, Bamenda, Cameroon, ³Nkwen Baptist Hospital, Ophthalmology Department, Bamenda, Cameroon, ⁴Mbingo Baptist Hospital, Radiology Department, Bamenda, Cameroon, ⁵Mbingo Baptist Hospital, Oncology Department, Bamenda, Cameroon, ⁶St. Pius Medicalized Health Centre, Outpatient Department, Tatum, Cameroon, ⁷University of Bamenda, Department of Public Health, Bamenda, Cameroon

^&Corresponding author
Bonko Neville, Cameroon Baptist Convention Health Services, Bamenda, Cameroon

Introduction    

Malignant lymphomas commonly occur in lymphoid tissues and rarely in extranodal sites. Non-Hodgkin lymphomas of the head and neck region are rare constituting about 0.4 to 2% of all extranodal lymphomas and 6.4 to 13% of all lymphomas occurring in this region [1]. Non-Hodgkin lymphomas are among the most common malignancies in patients infected with human immunodeficiency virus (HIV) and occur 25 to 60 times more compared to the general population. In this group of individuals, NHL tends to be highly aggressive with a poor prognosis and tends to develop in extranodal sites, such as the central nervous system, digestive tract, and bone marrow. Non-Hodgkin lymphomas involving the paranasal sinuses is rare in HIV-infected patients and is likely to be confused clinically and radiographically with sinusitis [2]. We present the case of a 22-year-old female, newly diagnosed with HIV infection with an unusual presentation of sinonasal NHL. The epidemiology, diagnostic challenge, and management strategies are here discussed.

Patient and observation     

Patient information: we received a 22-year-old female at our clinic, presenting with a 1-year history of nasal blockage and rhinorrhea worse on the right nostril. The patient admitted there was postnasal drip, but however denied any frequent sneezing or nasal itchiness. Initially, there were no eye symptoms but in the 1 month prior to consultation, she had started experiencing epiphora of the right eye which was somewhat increasing in frequency. Her vision was nevertheless preserved. There were no ear or throat symptoms and no neck swellings. She had lost 4kg of her weight in the last six months but to her, it was no cause for concern as this had been intentional. There were no fevers or night sweats.

Patient timeline: she had been diagnosed with HIV infection from birth for which she had been on Highly active antiretroviral therapy (HAART) and compliant with treatment. In March 2018 she was delivered of a live male child. In February 2021 she began experiencing frequent nasal blockages and rhinorrhea for which she consulted at primary healthcare centers frequently. In September 2022, a CT scan of nose and paranasal sinuses was requested which showed right maxillary sinus disease and she was booked for and underwent endoscopic sinus surgery. However, no histopathology of the resected specimen was requested.

Clinical findings: on examination, her vital signs were stable. There was an obvious anterior bulge of the right maxillary area. There was a polypoid mass in the right nasal cavity with markedly reduced nasal patency. Rigid nasal endoscopy of the left side showed normal left nasal cavity and nasopharynx. Ear and throat examinations were normal. Visual acuity was 6/6 in both eyes and extraocular motility was free in all directions of gaze in both eyes. There were no palpable lymph nodes.

Diagnostic assessment: a CT scan of the nose and paranasal sinuses was requested which showed complete soft tissue opacification of the right maxillary sinus with widening of the maxillary ostium and extension into the nasal cavity with thinning of the sinus walls. There was near to complete opacification of the left maxillary sinus, left frontal, left ethmoidal, and left sphenoidal sinuses suggestive of chronic sinus disease (Figure 1). Differential diagnosis includes mucocele, inflammatory polyp, possible fungal colonization of chronic sinus disease, and inverted papilloma. A punch biopsy of nasal mass was done which showed an inflammatory nasal polyp. Table 1 below summarizes the complete blood count and other blood tests done.

Therapeutic intervention and follow-up: the patient only showed up for surgery 2 months later with a draining sinus on the anterior maxillary wall. The open surgical approach was preferred over endoscopic excision based on the extent of the disease and its aggressive nature. Right-sided partial maxillectomy was done through a lateral rhinotomy and vertical lid split incision. Intraoperative findings included erosion of the anterior maxillary wall and orbital floor. Complete excision of mass was done up to the posterior ethmoids and sphenoid sinusotomy was done. The orbital floor was reconstructed with a titanium mesh plate (Figure 2). An excised specimen was sent for histopathological correlation. Microscopic sections revealed monomorphic, discohesive, small round cell tumors invading soft tissue and bone. Tumor cells were small to medium-sized size with coarse chromatin, small single or multiple nucleoli, and high nucleus-to-cytoplasm ratios. The mitotic rate was at 14/10 high power fields. Few macrophages were identified and there was no necrosis. This was in favor of aggressive non-Hodgkin lymphoma. The disease was staged as stage 1E according to the Ann Arbor staging system. The patient was referred to the oncology department for further management postoperatively. The patient, however, only showed up for treatment with a more advanced disease. The initial treatment regimen was a combination of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP). She defaulted on 3 cycles and returned with progressive disease and was treated empirically( due to lack of funds for proper investigations for staging) as stage III with a second-line combination treatment of dexamethasone, high-dose cytarabine, and cisplatin (DHAP) was initiated. With 3 cycles of treatment, there was marked improvement and treatment is currently ongoing.

Patient perspective: in addition to the health challenges our patient was having, she was also from a poor background, having just one surviving parent, her father, who literally had given up on her due to her numerous health needs. Also, being a single parent, further compounded her predicament. Her treatments had been funded by friends and well-wishers. She was however very satisfied with the standard of care she received and more so, knowing her face was getting back to normal, and with the positive reports from the oncologist, she had so much to hope for.

Informed consent: written consent was obtained from the patient for publication of this case report. A copy of the written consent is available for review.

Discussion     

Generally, the manifestations of lymphomas in the head and neck region are predominantly through lymph node involvement, with the Waldeyers ring being the most frequently involved extranodal site [3]. Paranasal sinus involvement is considered rare, present only in 0.2 to 5% of cases [4]. Non-Hodgkin lymphoma is 25 to 60 times higher in patients with HIV compared to the general population. Extra nodal disease is quite common in HIV and patients have been noted to develop lymphomas in tissues such as testicular, skeletal muscle, cardiac, renal, bile duct, rectal, anal and placental tissues, however rarely. Certain aggressive NHL variants have been recognised as AIDS-defining illnesses in HIV [5]. The pathological mechanism of NHL in HIV infection has been increasingly in recent times linked to Epstein Barr Virus (EBV) based on its ability to immortalize B lymphocytes and its frequent association with endemic Burkitt´s lymphoma and nasopharyngeal carcinoma. There is also a documented defect of T cell-mediated immunity to EBV with high levels of circulating EBV-infected B cells and the presence of EBV in oropharyngeal secretions in HIV-infected patients [6]. Nevertheless, the precise role of EBV in HIV-associated NHL is controversial.

In sinonasal NHL, the maxillary sinus is the most frequent site of involvement, followed by the ethmoid, sphenoid and frontal sinus. They can occur at any age, but usually, there is a prevalence of middle to older ages, with a male predominance [7]. Initial symptoms of NHL sinus disease are usually ophthalmic or oral in nature. This is partly because diagnosis is frequently missed, and partly because NHL in HIV is characteristically aggressive with rapid disease progression. That notwithstanding, contrary to the underlying disease process, it appears disease progression is largely determined by the location of the nose vis-à-vis adjacent structures and by the thinness of the bony sinus walls separating them from these structures. It may be suggested that sinonasal NHL has a high propensity for bone destruction It has long been recorded in literature that sinonasal malignancy may mimic chronic rhinosinusitis symptomatology [8]. Nasal polyps are not true neoplasms in themselves, but chronic sinus obstruction and tumour-mediated inflammation may engender polyp formation, concealing a malignancy in progress. In our patient, the diagnosis was missed in favour of chronic rhinosinusitis which led to a delay in diagnosis and treatment initiation.

Chemotherapy and radiotherapy are the mainstay of therapy for NHL depending on stage and prognosis factors. Non-Hodgkin lymphoma in HIV generally has a poor prognosis though some patients survive for relatively long periods of time [6]. Radiotherapy is used in localized forms with large tumour volumes. Chemotherapy is the treatment of choice in disseminated forms and the most usual protocol being is CHOP. Some authors recommend systematic complementary craniofacial radiotherapy, even in disseminated forms [9]. Chemotherapy is often complicated by a patient's HIV-related immunosuppression and general debility and may lead to significant morbidity or mortality. Surgical treatment is rarely applied to non-Hodgkin lymphoma except for diagnostic indications like lymph node biopsy. In sinonasal disease, surgery may be indicated for localised and locally aggressive disease [10]. In the present case, in addition to the diagnostic challenge, the disease was locally aggressive, with orbital floor erosion and risk of orbital prolapse, necessitating surgical excision and orbital floor reconstruction. HIV- infected individuals with sinonasal NHL need to be carefully assessed for disseminated disease, especially if the tumour is high-grade Early diagnosis will improve the prognosis. Continuous monitoring and close follow-up are recommended.

Conclusion     

Although NHL of the maxillary sinus is rare, otolaryngologists should keep this diagnosis in mind within the range of tumours of the paranasal sinuses. It should also be remembered that nasal polyposis could be a presentation of a malignant process in the sinuses. Early diagnosis and treatment will improve the prognosis and the patient's survival.

Competing interests     

The authors declare no competing interests.

Authors' contributions     

Bonko Neville, Kamsang Pius Beyiah, Kouya Francine, and Nguepmeni Noune Joel: patient care, and planning, design and writing of the case report. Ghislain Feudjio and Brandon Carl Monika: patient care and planning and revising of the case report. Nguepmeni Noune Joel, Chin Virginia, and Nguepmeni Noune Joel: patient care, and review and editing of the drafts. All authors were involved in the final approval for submission, including agreement to accountability for the work.

Table and figures     

Table 1: hematologic investigations

Figure 1: soft tissue window (A, B); and bone window (C, D); axial CT scan sections of nose and paranasal sinuses showing extensive mass within right maxillary sinus extending to the nasal cavity with erosion of anterior maxillary sinus wall and compression of lamina papyracea; thickened left maxillary sinus and ethmoid sinus mucosa is also seen

Figure 2: intraoperative picture showing right maxillary sinus with anterior and medial walls resected and orbital floor reconstructed with mesh plate

References