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CASE REPORT Table of Contents  
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Olfactory neuroblastoma with rhabdomyoblasts: A rare case report and review of the literature


1 Department of Pathology, The First Affiliated Hospital of Dalian Medical University, Dalian, Liaoning, China
2 Department of Radiology, The First Affiliated Hospital of Dalian Medical University, Dalian, Liaoning, China

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Date of Submission24-Feb-2021
Date of Decision27-May-2021
Date of Acceptance09-Nov-2021
Date of Web Publication25-May-2022
 

   Abstract 


Herein we present a case of a 62-year-old male patient who was admitted with the chief complaints of nasal obstruction. The histopathological and immunohistochemical evaluation led to a diagnosis of olfactory neuroblastoma with rhabdomyoblasts. A review of the literature revealed that this is only the fourth case of olfactory neuroblastoma with rhabdomyoblasts. Thus, investigation of more cases and longer follow-up is necessary to understand the disease and identify the best treatment to improve prognosis.

Keywords: Immunohistochemistry, olfactory neuroblastoma, rhabdomyoblasts


How to cite this URL:
Jin Y, Zhang L, Qi W, Zhou Y, Kong J. Olfactory neuroblastoma with rhabdomyoblasts: A rare case report and review of the literature. Indian J Pathol Microbiol [Epub ahead of print] [cited 2023 Jan 29]. Available from: https://www.ijpmonline.org/preprintarticle.asp?id=345860





   Introduction Top


Olfactory neuroblastoma (ONB) is a rare neuroectodermal tumor of the nasal cavity that was first described in 1924 by Berger et al.[1] Mixed olfactory neuroblastoma, which contains areas of divergent differentiation, is even rarer. A few cases of mixed olfactory neuroblastomas have been reported.[2],[3],[4] To our knowledge, this is the fourth case of olfactory neuroblastoma with rhabdomyoblasts in the literature.[5],[6],[7] [Table 1].
Table 1: Reported cases of ONB with rhabdomyoblasts

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   Case Report Top


A 62-year-old man was admitted with a chief complaint of nasal obstruction lasting 20 days. Magnetic resonance imaging (MRI) confirmed a 3.9 × 2.5 cm mass that was hypodense on T1-weighted images [Figure 1]a, hyperdense on T2-weighted images [Figure 1]b, and enhanced by intravenous infusion of diethylenetriamine pentaacetic acid (DTPA). Sagittal and coronal contrast-enhanced images from MRI revealed an avidly enhancing nasal vault mass extending intracranially [Figure 1]c, [Figure 1]d.
Figure 1: Magnetic resonance imaging (MRI) confirmed a mass that was hypodense on T1-weighted images (a), hyperdense on T2-weighted images (b), and enhanced by intravenous infusion of DTPA. Sagittal (c) and coronal (d) contrast-enhanced images from MRI demonstrate an avidly enhancing nasal vault mass extending intracranially.

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The biopsy showed a high-grade tumor composed of lobules, sheets, and a nest of primitive cells with round or oval nuclei, pleomorphism, hyperchromatic nuclei, and scant cytoplasm [Figure 2]a, [Figure 2]b. The cellular nests were separated by vascularized fibrous stroma. Some of the larger tumor cells were diffused and had ample eosinophilic cytoplasm and a nucleus sometimes displaced toward the periphery of the cell body [Figure 2]c, [Figure 2]d. Most of the tumor cells were positive for NSE, CgA, CD56, and Syn [Figure 3]a, [Figure 3]b. MyoD1, Desmin, and Myogenin reacted positively in the eosinophilic cytoplasm of large tumor cells [Figure 3]c, [Figure 3]d. Parts of the tumor cells were positive for CK, CK8/18. S-100 protein was limited to sustentacular cells along the periphery of neoplastic lobules. A wide panel of antibodies including CK20, CK7, GFAP, TTF-1, Melan A, and HMB45 were all negative. Ki-67 staining showed a proliferation rate of about 50%. On the basis of histopathological and immunohistochemical examination, a diagnosis of olfactory neuroblastoma with rhabdomyoblasts was made. The patient went on to receive radiotherapy. The patient is now scheduled for adjuvant chemoradiation.
Figure 2: Morphological features of ONB with rhabdomyoblasts. (a and b) Tumor composed of sheets and a nest of primitive cells with round or oval nuclei, pleomorphism, hyperchromatic nuclei, and scant cytoplasm, 100× (a) and 200× (b). (c and d) Some of the larger tumor cells were diffused and had ample eosinophilic cytoplasm and a nucleus sometimes displaced toward the periphery of the cell body, 100× (c) and 400× (d).

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Figure 3: Immunophenotype of ONB with rhabdomyoblasts. Tumor cells were positive for CD56 (a) and Syn (b). The larger tumor cells were positive for Desmin (c) and Myogenin (d) (100 ×).

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   Discussion Top


ONB is a rare sinonasal malignancy. It accounts for 2 to 3% of nasal cavity malignancies. The patients were aged 2 to 90 years and had bimodality in the second and sixth decades. The most common symptoms are nasal obstruction and epistaxis. Other manifestations include anosmia, headache, pain, excessive lacrimation, rhinorrhea, proptosis, and visual disturbances. Clinically, ONB usually appears as a unilateral pedunculated reddish-gray nasal mass with a smooth surface. The histogenesis of ONB is not clear. The most suggested site of origin is the basal progenitor cells of the olfactory epithelium. Histologically, the tumors are localized to the submucosa and composed of lobules, sheets, and a nest of primitive cells. The cells are classically small and round with a salt and pepper chromatin arrangement and minimal cytoplasm. The cellular nests are separated by vascularized fibrous stroma. However, in higher-grade tumors, nuclear pleomorphism with prominent nucleoli, and increased mitotic activity and necrosis may be present. Rosettes of the Homer–Wright type (pseudorosettes) can be identified in up to 30% of tumors, and rosettes of the Flexner–Wintersteiner type (true neural rosettes) can be identified in less than 5% of tumors. Calcifications, ganglion cells, melanin-containing cells, and cells of divergent differentiation have been occasionally seen in ONB. A few cases may include the presence of glandular (adenocarcinoma-like), squamous, and rhabdomyoblastic differentiation.[2],[3],[6],[7] The present study represents the fourth case of ONB with rhabdomyoblasts in the literature.

A diagnosis must be made cautiously because of the extremely rare nature of ONB with rhabdomyoblasts. Here, rhabdomyosarcoma was excluded as a diagnosis because of the observation of sheets and large nests of small, round-to-oval cells with scant cytoplasm negative for Desmin, Myogenin, MyoD1, and CD99. The number of tumor cells positive for epithelium markers further excluded rhabdomyosarcoma. Small cell carcinoma was also excluded because the tumor cells were negative for TTF-1 and CD99. Sinonasal undifferentiated carcinoma, lymphoma, mucosal malignant melanoma, and neuroendocrine carcinomas were also excluded because of the negative expression pattern of CK20, CK7, GFAP, LCA, TTF-1, Melan A, and HMB45. In general, low-grade ONB is readily recognizable and diagnostic by light microscopy. With high-grade tumors, immunohistochemistry may assist in the diagnosis.

ONBs are positive for NSE, Syn, CgA, CD56, and NFP. S-100 protein staining that is typically limited to the periphery of neoplastic lobules is characteristic of ONB. Epithelial markers, including EMA and CEA, are absent. Cytokeratin is usually negative; however, some cases can show positive cells, as seen in the present case. LCA, HMB-45, Melan A, Desmin, and CD99 are also absent.

Complete surgical excision of the mass followed by adjuvant radiotherapy is the “gold standard” for treating ONBs. The risk of recurrence is higher among those with poor prognostic factors, such as high Hyams grade, advanced Kadish staging, and positive resection margins. The highest risk is a locoregional recurrence, with the majority of recurrence between 5 and 10 years from the initial occurrence. ONB is believed to be a radiosensitive tumor. Radiation therapy is usually given in the adjuvant setting. Chemotherapy or multimodal treatment is usually recommended in patients in the advanced stage.

A diagnosis must be made cautiously because of the extremely rare nature of ONB with rhabdomyoblasts. Thus, larger numbers of cases and longer follow-up are necessary to understand the disease and identify the best treatment to improve prognosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Berger L, Luc G, Richard D. L'esthesioneuroepitheliome olfactif. Bull Assoc Franc Etude Cancer 1924;13:410-21.  Back to cited text no. 1
    
2.
Attwood JE, Jeyaretna DS, Sheerin F, Shah KA. Mixed olfactory neuroblastoma and adenocarcinoma with in situ neuroendocrine hyperplasia. Head Neck Pathol 2020;14:792-8.  Back to cited text no. 2
    
3.
Gandhoke CS, Dewan A, Gupta D, Syal SK, Jagetia A, Saran RK, et al. A rare case report of mixed olfactory neuroblastoma: Carcinoma with review of literature. Surg Neurol Int 2017;8:83.  Back to cited text no. 3
  [Full text]  
4.
Lao WP, Thompson JM, Evans L, Kim Y, Denham L, Lee SC. Mixed olfactory neuroblastoma and neuroendocrine carcinoma: An unusual case report and literature review. Surg Neurol Int 2020;11:97.  Back to cited text no. 4
    
5.
Miyagami M, Katayama Y, Kinukawa N, Sawada T. An ultrastructural and immunohistochemical study of olfactory neuroepithelioma with rhabdomyoblasts. Med Electron Microsc 2002;35:160-6.  Back to cited text no. 5
    
6.
Slootweg PJ, Lubsen H. Rhabdomyoblasts in olfactory neuroblastoma. Histopathology 1991;19:182-4.  Back to cited text no. 6
    
7.
Shintaku M, Ohta M, Kataoka K, Okabe H. Olfactory neuroblastoma associated with extensive “in situ” lesion and aberrant glandular and rhabdomyosarcomatous differentiation. Neuropathology 2021;41:273-80.  Back to cited text no. 7
    

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Correspondence Address:
Jienan Kong,
Department of Pathology, the First Affiliated Hospital of Dalian Medical University, No. 222 Zhongshan Road, Dalian - 116011, Liaoning
China
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_209_21



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