PATHOGENS

  • HHV-8: also known as Kaposi’s sarcoma-associated herpesvirus (KSHV), the agent of KS.
  • EBV: associated with lymphoma.

CLINICAL

  • Malignant tumors of sinonasal tract in general population constitute < 1% of all malignancies, and 3% of malignancies in upper respiratory tract
  • Most common sinonasal malignancies in HIV+ and non-HIV+ populations include squamous cell carcinoma (45-80%), salivary gland carcinoma (5-15%), and sarcomas (5%). Sinonasal lymphoma is rare (1.5% of all lymphomas).
  • Kaposi’s sarcoma (KS) and non-Hodgkin’s lymphoma (NHL) are associated with HIV infection.
  • Clinical features: nasal obstruction, rhinorrhea, sinusitis unresponsive to medical therapy, otitis media with effusion, mass effect on face/orbits, diplopia, and cranial nerve palsies. Complaints often non-specific and result in delay of Dx. Presentation may include fever, weight loss, and malaise.
  • KS: sinonasal involvement less common than at other sites. May involve any mucosal site of upper aero-digestive tract. Additional Sx include pain and bleeding. Associated with concurrent cutaneous lesions. Similar indolent course.[3]
  • NHL: occurs 25-60x more frequently in HIV+ pts. Sinonasal site rare. More common extranodal sites include CNS, digestive tract, and bone marrow. Often accompanied by fever, weight loss, and malaise. Highly aggressive.
  • Squamous cell carcinoma (SCC): can arise in sinonasal tract or nasopharynx. Poor prognosis if orbit or intracranial spread.

DIAGNOSIS

  • Clinical exam: Endoscopic evaluation most reliable. May appear as friable, grayish necrotic lesion (NHL) or red, purple, or black macule or papule of nasal mucosa (KS).
  • Sinus CT: Best to evaluate for bony erosion of tumor. Critical areas include bony orbits, ethmoid roof, and post. maxillary sinus to evaluate orbital, intracranial, or pterygopalantine fossa invasion. High resolution or thin cuts recommended. Contrast usually not helpful.
  • PET-CT: For initial evaluation and staging. More accurate that CT alone, particularly in aggressive NHL. Not indicated for routine surveillance.[1]
  • MRI: Excellent to delineate tumor from surrounding inflamed tissue or secretions and to evaluate intracranial spread.
  • Bx: Primary lesion and any nodal metastasis. Transnasal intraoperative Bx preferred. Evaluate tissue using histopathology and immunohistochemical stains.
  • DDx: Infectious conditions such as fungal diseases (i.e., mucormycosis, aspergillosis, Alternaria, Bipolaris) or TB may mimic malignancy. Bx with search for atypical organisms especially important in HIV infection with immunosuppression. Inflammatory nasal polyps and pyogenic granuloma (benign nasal tumor) also included.

TREATMENT

Treatment

  • Combination therapy with en-bloc resection and radiotherapy (RT) most common approach for tumors of epithelial origin (e.g., SCC). RT improves cure in this region due to difficulty obtaining wide surgical margins. Chemotherapy reserved for palliation. Prophylactic neck dissection rarely performed.
  • Minimally invasive endoscopic techniques being used to resect some sinonasal malignancies in select patients.
  • KS: ART dramatically reduces incidence of KS and contributes to resolution of disease. Treatment reserved for symptomatic tumors (severe nasal obstruction or infection, significant bleeding, impingement on vital structures). Cryotherapy, surgical excision, laser ablation, intra-lesional or systemic chemotherapy have all been described.
  • NHL: Aggressive chemotherapy treatment of choice. Regimens include CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) or EPOCH (etoposide, vincristine, cyclophosphamide, doxorubicin, prednisone). Both regimens often given with rituximab.[2] Non-responders may require local RT or rarely surgical resection. Poor prognosis.

FOLLOW UP

  • Epithelial tumors require regular F/U on monthly basis after treatment to monitor for recurrence.
  • Sinonasal KS most often asymptomatic and can be followed on a bi-annual basis. Symptomatic lesions or disseminated disease should be more frequently monitored for progression.
  • NHL requires close monitoring by oncology.

Basis for recommendation

  1. Cheson BD et al: Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification. J Clin Oncol 32:3059, 2014  [PMID:25113753]

    Comment: Data presented at 12th Annual Conference on Malignant Lymphoma defines consensus on management of HL and NHL including recommendation for PET-CT as modality for initial evaluation and staging.

References

  1. Lamar ZS et al: Dose-Adjusted Etoposide, Prednisone, Vincristine, Cyclophosphamide, and Doxorubicin (EPOCH) With or Without Rituximab as First-Line Therapy for Aggressive Non-Hodgkin Lymphoma. Clin Lymphoma Myeloma Leuk 16:76, 2016  [PMID:26725264]

    Comment: Retrospective cohort analysis of single site (Wake Forest, Salem, NC) data included 136 patients with NCI-defined aggressive NHL and treated with infusion EPOCH±rituximab as first-line therapy from 2005-2013. Ten of 100 with available serology were HIV+. Median follow-up duration was 27 mos. Overall response rate was 82%.

  2. Mouden K et al: Primary Kaposi's sarcoma of the nasal cavity: a case report and review of the literature. Clin Sarcoma Res 6:, 2016  [PMID:26998222]

    Comment: Report of 56yo woman with 1 year history of left-sided nasal swelling diagnosed with Kaposi’s sarcoma and treated with ifosfamide and doxorubicin with excellent local control at 12 months.

  3. Dutta R et al: Sinonasal malignancies: A population-based analysis of site-specific incidence and survival. Laryngoscope 125:2491, 2015  [PMID:26228792]

    Comment: US data based on National Cancer Institute Surveillance, Epidemiology, and End Results (SEER) registry from 1973 to 2011 included 13,295 patients and found that squamous cell cancer is the most common histology (42%) and nasal cavity is the most common site (46%) of sinonasal malignancy.
    Rating: Important

  4. Lombard M et al: Extranodal non-Hodgkin lymphoma of the sinonasal cavities: A 22-case report. Eur Ann Otorhinolaryngol Head Neck Dis 132:271, 2015  [PMID:26363601]

    Comment: Retrospective report of 22 patients diagnosed with lymphoma discovered by sinonasal involvement at the University Hospital Center of Nantes, France. All had non-Hodgkin lymphoma, 77% with diffuse large B-cell lymphoma and 68% involved craniofacial bones, i.e., maxillary or ethmoid sinuses. Presentation most often included unilateral nasal obstruction, mucopurulent rhinorrhea, recurrent epistaxis, or diplopia.

  5. Peng KA et al: Sinonasal lymphoma: case series and review of the literature. Int Forum Allergy Rhinol 4:670, 2014  [PMID:24760602]

    Comment: Case series included 17 patients with sinonasal lymphoma identified by pathology records from 1990-2012 at UC Los Angeles. Presenting symptoms were nasal obstruction and rhinorrhea most commonly (53%) followed by diplopia (18%). Most common type was diffuse large cell B-cell lymphoma (53%) followed by extranodal natural killer T-cell lymphoma (21%).

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Last updated: July 31, 2016