Sinonasal malignancies

Lisa A. Spacek, M.D., Ph.D.

PATHOGENS

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

CLINICAL

  • Malignant tumors of sinonasal tract are rare and make up 3-5% of all head and neck cancers.
  • Although sinonasal tract involves a limited area, the complex anatomy gives rise to histologically distinct solid tumors complicated by high-grade and/or undifferentiated morphology.[3]
    • Most common sinonasal malignancies are squamous cell carcinoma, adenocarcinoma, mucosal melanoma, and sinonasal minor salivary gland tumors.[5]
  • 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 nonspecific and result in delay of diagnosis.
    • Presentation may include fever, weight loss, and malaise.
  • Kaposi sarcoma (KS) and non-Hodgkin lymphoma (NHL) are associated with HIV infection.
    • KS: sinonasal involvement less common than at other sites. May involve any mucosal site of upper aero-digestive tract. Symptoms may include pain and bleeding. Associated with concurrent cutaneous lesions. Similar indolent course.[7]
    • NHL: occurs 25-60x more frequently in PLWH. 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 (refer to ENT). 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.[2]
  • MRI: Excellent to delineate tumor from surrounding inflamed tissue or secretions and to evaluate intracranial spread.[4]
  • Bx: Primary lesion and any nodal metastasis. Transnasal intraoperative biopsy preferred. Evaluate tissue using histopathology and immunohistochemical stains.[3]
  • 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 may improve outcomes for treatment in select patients.[1]
  • 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.[6] Non-responders may require local RT or rarely surgical resection. Poor prognosis.

FOLLOW UP

  • Epithelial tumors require regular follow-up 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. Carlton DA, David Beahm D, Chiu AG. Sinonasal malignancies: Endoscopic treatment outcomes. Laryngoscope Investig Otolaryngol. 2019;4(2):259-263.  [PMID:31024998]

    Comment: Due to advances in endoscopic techniques, endoscopic endonasal approaches compared to open craniofacial resection may offer similar survival outcomes and lower complication rates. Given sinonasal cancers frequently have a focal attachment point, a primary goal of endoscopic approach is piece-meal debulking with resection of tumor pedicle en bloc. Review includes listing of 5 recent systematic reviews and meta-analyses.

  2. Cheson BD, Fisher RI, Barrington SF, et al. Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification. J Clin Oncol. 2014;32(27):3059-68.  [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. Taverna C, Agaimy A, Franchi A. Towards a Molecular Classification of Sinonasal Carcinomas: Clinical Implications and Opportunities. Cancers (Basel). 2022;14(6).  [PMID:35326613]

    Comment: Given great heterogeneity of sinonasal carcinomas, molecular profiling may be used to determine management. For example, HPV positivity in sinonasal SCC suggests a favorable prognosis compared to HPV negative SNSCC.
    Rating: Important

  2. Famuyide A, Juliano A, Moonis G. MRI of Sinonasal Malignancies. Top Magn Reson Imaging. 2021;30(3):139-149.  [PMID:34096897]

    Comment: Role of MRI in evaluation of sinonasal tumors includes soft tissue delineation, evaluation of multicompartmental extension, intracranial spread, and perineural spread.

  3. Contrera KJ, Woody NM, Rahman M, et al. Clinical management of emerging sinonasal malignancies. Head Neck. 2020;42(8):2202-2212.  [PMID:32212360]

    Comment: Authors review emerging sinonasal malignancies (Table 1) and provide clinical details and imaging findings.

  4. Lamar ZS, Fino N, Palmer J, 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. 2016;16(2):76-81.  [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%.

  5. Mouden K, Khmou M, Loughmari S, et al. Primary Kaposi's sarcoma of the nasal cavity: a case report and review of the literature. Clin Sarcoma Res. 2016;6:4.  [PMID:26998222]

    Comment: Report of 56 yo 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.

Sinonasal malignanciesis the Johns Hopkins Guides Word of the day!

Last updated: October 19, 2022