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Esthesioneuroblastoma, Neuroendocrine Carcinoma, and Sinonasal Undifferentiated Carcinoma: Differentiation in Diagnosis and Treatment

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Esthesioneuroblastoma, Neuroendocrine Carcinoma, and Sinonasal Undifferentiated Carcinoma: Differentiation in Diagnosis and Treatment THIEME Review Article S149 Esthesioneuroblastoma, Neuroendocrine Carcinoma, and Sinonasal Undifferentiated Carcinoma: Differentiation in Diagnosis and Treatment Shirley Y. Su1 Diana Bell2 Ehab Y. Hanna1 1 Department of Head and Neck Surgery, University of Texas MD Address for correspondence Ehab Y. Hanna, MD, Department of Head Anderson Cancer Center, Houston, Texas, United States and Neck Surgery, University of Texas MD Anderson Cancer Center, 2 Department of Pathology, University of Texas MD Anderson Cancer 1515 Holcombe Blvd., Suite 1445, Houston, TX 77030-4009, Center, Houston, Texas, United States United States (e-mail: [email protected]). Int Arch Otorhinolaryngol 2014;18:S149–S156. Abstract Introduction Malignant sinonasal tumors comprise less than 1% of all neoplasms. A wide variety of tumors occurring primarily in this site can present with an undifferenti- ated or poorly differentiated morphology. Among them are esthesioneuroblastomas, sinonasal undifferentiated carcinomas, and neuroendocrine carcinomas. Objectives We will discuss diagnostic strategies, recent advances in immunohis- tochemistry and molecular diagnosis, and treatment strategies. Data Synthesis These lesions are diagnostically challenging, and up to 30% of sinonasal malignancies referred to the University of Texas MD Anderson Cancer Center Keywords are given a different diagnosis on review of pathology. Correct classification is vital, as ► sinonasal malignancy these tumors are significantly different in biological behavior and response to treat- ► esthesioneuroblas- ment. The past decade has witnessed advances in diagnosis and therapeutic modalities toma leading to improvements in survival. However, the optimal treatment for esthesioneur- ► sinonasal oblastoma, sinonasal undifferentiated carcinoma, and neuroendocrine carcinoma undifferentiated remain debated. We discuss advances in immunohistochemistry and molecular diag- carcinoma nosis, diagnostic strategies, and treatment selection. ► neuroendocrine Conclusions There are significant differences in prognosis and treatment for esthe- carcinoma sioneuroblastoma, neuroendocrine carcinoma, and sinonasal undifferentiated carcino- ► olfactory ma. Recent advances have the potential to improve oncologic outcomes but further neuroblastoma investigation in needed. Introduction advancement is closely tied to the improvements in diagnosis, surgery, and adjuvant treatments. The sinonasal tract is the location for a wide variety of benign Malignant sinonasal tract tumors comprise less than 1% of and malignant tumors. Per cubic centimeter, the sinonasal all neoplasms and 3% of the upper aerodigestive tract. tract gives rise to a greater diversity of neoplasms than any Sinonasal tract malignancies most commonly affect the max- other site in the human body. The diversity is partly due to the illary sinus (60%), followed by the nasal cavity (22%), ethmoid anatomic complexity and highly varied tissues in this com- sinus (15%), and frontal and sphenoid sinuses (<3%). Sino- pact area. Malignancies of the sinonasal tract have shown nasal tract tumors are diverse. The majority are squamous dramatic improvements in survival, from 20% in the 1950s to carcinomas and their variants (55%), followed by nonepithe- 60 to 80% survival as cited by the most current literature. This lial neoplasms (20%), glandular tumors (15%), DOI http://dx.doi.org/ Copyright © 2014 by Thieme Publicações 10.1055/s-0034-1390014. Ltda, Rio de Janeiro, Brazil ISSN 1809-9777. S150 Malignant Sinonasal Tumors Su et al. undifferentiated carcinomas (7%), and miscellaneous tumors myosarcoma. S-100 is variably positive, but positive cells are (3%). The spectrum of primary sinonasal undifferentiated usually limited to the periphery of neoplastic nests, corre- neoplasms has expanded because new entities specificto sponding to sustentacular cells. This characteristic pattern this region or initially described in other locations have been differentiates ENB from sinonasal melanoma. FLI1 is negative, recognized over time. as is the Ewings Sarcoma/Friend Leukemia Integration-1 Several of the malignant tumors occurring primarily in the (EWS/FLI1) chimeric transcript, ruling out the rare diagnosis sinonasal tract may present with an undifferentiated or of peripheral neuroectodermal tumor/Ewing sarcoma. Hyams poorly differentiated morphology and are composed of small, grading scheme, which covered work predating the first medium, and large round or polygonal atypical cells. These description of SNUC by nearly 10 years, captures the spec- lesions pose significant diagnostic difficulties for the surgical trum of ENB maturation.1,2 Several groups have asserted that pathologist, especially with limited biopsy material. Up to grade 3 and predominantly grade 4 ENBs are in fact SNUCs.1 30% of sinonasal malignancies referred to our institution are Low-grade ENB forms sharply demarcated lobules of cells given a different diagnosis on review of pathology. However, embedded in a richly vascularized stroma; the cells are small correct classification by means of histology, immunohis- (slightly larger than lymphocytes), with “salt-and-pepper” tochemistry, or molecular biology is important for initiating chromatin. Neuropil (interdigitating neuronal processes) is an appropriate treatment strategy. abundant in low-grade ENB (►Fig. 1A). The cells may form There is significant debate regarding the optimal treat- rosettes with true lumens (Flexner-Wintersteiner type) or ment of esthesioneuroblastoma (ENB), neuroendocrine car- pseudorosettes (Homer-Wright type). High-grade ENBs are cinoma (NEC), and sinonasal undifferentiated carcinoma more difficult to diagnose, with increased cytologic atypia, (SNUC). The rarity of these tumors, difficulties in establishing pleomorphism, necrosis, increased mitotic activity, solid a diagnosis, and heterogeneity in treatment approaches all pattern, loss of neuropil, and abundant mitotic activity contribute to the lack of consensus. However, the past decade (►Fig. 1B). We retrospectively analyzed the records of 124 has resulted in significant progress in treatment. This review patients with ENB who had been treated at our institution for emphasizes diagnostic strategies, recent advances in immu- the association of grade and stage with prognostic outcome. nohistochemistry and molecular diagnosis, and a discussion High-grade ENB was significantly associated with poor out- of treatment strategies. comes, and advanced stage was not associated with poor outcome in this cohort. Grading should certainly be consid- 3 Review of Literature and Discussion ered in prognostication and treatment decisions for ENB. Cytogenetic data for ENB are limited. Holland et al, who Esthesioneuroblastoma performed cytogenetic characterization of one case using ENB is a tumor restricted to the area of olfactory neuro- trypsin Giemsa staining (GTG banding), multicolor fluores- epithelium, which arises from embryonic olfactory placodes cence in situ hybridization, and single-nucleotide polymor- and in adults is replaced partially by respiratory mucosa. First phism karyotyping, reported numerous chromosomal described by Berger and colleagues in 1924, ENB has been aberrations predominantly involving chromosomes 2q, 5, characterized as a rare malignant neoplasm of the sinonasal 6q, 17, 19, 21q, and 22, as well as trisomy 8.4 Bockmühl et cavity that arises in the superior portion of the nasal vault.1,2 al applied conventional comparative genomic hybridization A variety of nomenclature has been used to describe this (CGH) to 22 ENB and reported frequent deletions of 1p, 3p/q, tumor (ENB, esthesioneuroepithelioma, esthesioneurocy- 9p, and 10p/q, and amplifications of 17q, 17p13, 20p, and toma, olfactory neuroblastoma, and NEC); the accepted terms 22q.5 They also noted a specific deletion on chromosome 11 at this time are esthesioneuroblastoma and olfactory neuro- and gain on chromosome 1p, which were associated with blastoma. Arising from the neural-epithelial olfactory muco- metastasis and a worse prognosis. Three ENB were studied by sa, phenotypically ENB is intermediate between that of a pure Riazimand et al using conventional CGH, and amplification of neural neoplasm (e.g., neuroblastoma and paraganglioma) whole chromosome 19; partial gains of 1p, 8q, 15q, and 22q; and a neuroendocrine epithelial tumor (e.g., carcinoid, NEC, and deletions of 4q and 6p were detected.6 Szymas et al small cell carcinoma). studied a single ENB and found gains of whole chromosomes The differential diagnosis is broad: ENB can be confused 4, 8, 11, and 14; partial gains of 1q and 17q; partial deletions histologically with several other “small blue round cell tu- of 5q and 17q; and whole chromosome losses of 16, 18, 19, mors” of the nasal cavity and paranasal sinuses. Tumors and X.7 Guled et al applied an oligonucleotide-based array commonly confused with ENB include SNUC, sinonasal NEC, CGH to identify DNA copy number changes in 13 cases of small cell carcinoma, pituitary adenoma, melanoma, lympho- ENB.8 Novel chromosomal regions were identified that were ma, and rhabdomyosarcoma.1 Therefore, thorough pathologic frequently altered in addition to previously reported abnor- review and ancillary studies are essential to differentiating mal regions. The most frequent changes included gains at between these tumor types and properly diagnosing ENB. 7q11.22–q21.11, 9p13.3, 13q, 20p/q, and Xp/q, and losses at ENB typically shows diffuse staining with neuron-specific 2q31.1, 2q33.3, 2q37.1, 6q16.3, 6q21.33, 6q22.1, 22q11.23,
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