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Paraganglioma Presenting as a Nasal Septal Mass: Case Report and Literature Review.

1. Introduction

Paragangliomas are rare, slow-growing neuroendocrine tumors arising from cells of neural crest origin. They typically originate from the adrenal gland, but occur extraadrenally in 5-10% of cases [1]. In the head and neck region, paragangliomas make up only 0.6% of all tumors, most commonly in the carotid body and jugular-tympanic regions [1-3]. Though rare, there are few reported cases of paragangliomas arising in the nasal cavity and paranasal sinuses [4-8]. To our knowledge, there has only been one previously reported case of a paraganglioma originating from the nasal septum [8].

2. Case Report

A 70-year-old female complaining of persistent nasal congestion and obstruction presented to our clinic for evaluation. She denied any headache or epistaxis. Nasal endoscopy was performed which showed a posterior septal mass approaching the sphenoid sinuses bilaterally and partially obstructing the view of the nasopharynx. The overlying mucosa was intact except for a small area superiorly which showed a soft granulomatous mass protruding into the left nasal cavity.

The patient was taken to the operating room for septoplasty with biopsy of the mass at an outside institution. Microscopic examination of the biopsy specimen demonstrated clusters of epithelioid-appearing cells separated by bands of fibrillary stroma. The epithelioid cells were noted to have abundant amphophilic cytoplasm, uniform, rounded nuclei with "salt and pepper" chromatin, and small nucleoli. No mitotic activity, invasion, necrosis, or calcification was seen. Immunohistochemical staining demonstrated positivity for neuron-specific enolase (NSE), chromogranin A, synaptophysin, and CD56 cell markers within the epithelioid cells. Fibrillary cells were positive for NSE, chromogranin A, S-100, glial fibrillary acid protein (GFAP), and CD56 cell markers. Based on the histological appearance and immunohistochemical staining, a diagnosis of paraganglioma was made.

The patient was referred to our institution for further management. Preoperative CT imaging showed a smoothly marginated, soft tissue density mass centered at the posterior nasal septum with extension into the nasopharynx and bulging into the right sphenoid sinus (Figure 1). Severe thinning and smooth remodeling of the anterior wall of the sphenoid sinus and anterior clivus were seen. MRI imaging demonstrated hyperintense signaling of the mass on T1weighted images with a peripheral rim of hypointense signaling on T2-weighted imaging suggestive of a capsule.

An endoscopic resection of the mass was performed. Intraoperatively, a large mass was seen in the posterior aspect of the septum, bulging into the bilateral nasal cavities and extending into the sphenoid sinuses (Figure 2). Erosion of the bone of the rostrum and anterior face of the sphenoid were also seen. Complete resection of the mass was achieved through a posterior septectomy and bilateral sphenoidotomy with tissue removal. Postoperative histologic analysis of the specimen was consistent with a paraganglioma (Figure 3).

The patient has been symptom free without local recurrence 3 months following tumor resection.

3. Discussion

Paragangliomas arising from the nasal cavity and nasal sinuses are extremely rare. Only 48 total cases of paragangliomas occurring in the nasal cavity or paranasal sinuses have been reported to date, occurring in patients with ages ranging between 8 and 89 years old, with an average age of 49 years old. These tumors are slightly more prevalent amongst females (60.4%). Twelve were reported to be malignant in nature, demonstrating either intracranial extension or metastasis to the cervical lymph nodes, brain, and bone. These masses were most frequently reported to originate from the ethmoid sinuses, middle turbinate, and maxillary sinuses. Other less common locations of origin reported include the superior and inferior turbinates, and the sphenoid and frontal sinuses. To the best of our knowledge, there has only been one other reported case of a paraganglioma originating from the nasal septum (Table 1) [8].

The most commonly reported symptoms included nasal obstruction, headache, and recurrent epistaxis. The vast majority of reported cases were functionally inactive, with no evidence of catecholamine or adrenocorticotropic hormone (ACTH) secretion. However, there have been a few reported cases of metabolically active tumors secreting ACTH and catecholamines, causing hypertension and other cushingoid features in the affected patient [12, 13, 30]. As such, testing for catecholamine and metanephrines is recommended in symptomatic patients [29, 33].

The origin of paragangliomas in the nasal cavity and paranasal sinuses remains unclear. Although paraganglionic tissue has never been demonstrated in the nasal cavity, a wider distribution ofparaganglionic tissue is thought to exist in fetuses and neonates, degenerating after birth [42, 43]. Some authors have suggested that the migration of these embryonic paraganglionic cells accounts for the occurrence of paragangliomas in areas with no known paraganglionic tissue like the nasal cavity [13,29,42,44]. Additionally, it has been shown that paraganglionic tissue exists around the terminal portion of the maxillary artery in infants and could possibly represent a point of origin for paragangliomas in the nasal cavity due to its anatomic proximity [19, 45]. Others have suggested that the paraganglionic tissue may exist in the pterygopalatine fossa due to the close relationship of paraganglionic tissue with arteries and cranial nerves [18, 19, 22].

In contrast, chondrocytes of the cartilaginous septum develop from neural crest cells between the nasal cavities by the ninth week of embryonic development [46, 47]. This is evident by the expression of various neural crest stem cell markers in nasal septum progenitor cells [48]. Several studies have demonstrated that these neural crest-derived chondrocytes in an adult septum retain pluripotent properties, with the capacity for osteogenic, chondrogenic, and neurogenic differentiation [48, 49]. It is possible that the neural crest origin of septal chondrocytes plays an important factor in the development of nasal septal paragangliomas. Despite the neural crest origins of nasal chondrocytes, paragangliomas arising from the nasal septum are extremely rare.

Masses arising from the nasal septum represent a broad spectrum of diagnoses including polyps, chondromas, hematomas, and hemangiomas. Less common septal lesions include sarcomas, melanomas, and other neoplasms [46, 50]. Patients with septal masses typically present with nonspecific symptoms of nasal obstruction or epistaxis, making it difficult to diagnose these masses based on clinical evaluation alone. CT imaging and MR imaging are useful tools in determining exact locations and boundaries of lesions, as well as identifying any erosion or extension into surrounding structures, though imaging features of most masses are nondiagnostic. Diagnosis of such masses requires a combination of clinical history and histopathologic findings. Although paragangliomas of the nasal septum are extremely rare, they should be included in the differential diagnosis when evaluating a septal mass.

The classical histologic appearance of a paraganglioma features an alveolar pattern of well-defined clusters of epithelioid chief cells with round nuclei and eosinophilic cytoplasm. These cells form a "zellballen" pattern of nests separated by vascular stroma and spindle-shaped sustentacular cells [28, 43]. Immunohistochemical staining of paragangliomas generally demonstrates positive markers for NSE, synaptophysin, and chromogranin within the chief cells and S-100 and GFAP within the sustentacular cells [8, 25, 43].

Although the majority of reported nasal paragangliomas are benign, 12 of the cases presented in this review (25.0%) were reported to be malignant, demonstrating intracranial invasion and/or metastasis to the brain, cervical lymph nodes, and bones. It is widely accepted that malignancy cannot be diagnosed by histology alone, but requires evidence of bony invasion or distant metastasis [18, 19, 22]. Certain histological features such as mitotic figures, cellular pleomorphism, necrosis, and vascular invasion can be suggestive of malignancy, though none of these features have been shown to be reliable predictors of malignancy [19, 51]. As such, evaluation with CT imaging and MRI imaging plays a key role in both localization of the primary tumor and identification of local invasion or metastasis.

Almost all of the cases presented in this review were treated surgically. Suggested management of sinonasal paragangliomas involves surgical excision with close followup, as several cases of recurrence have been reported [4, 22, 29]. Due to the rich vascularity of these masses, preoperative embolization of the vessels supplying the tumor has been suggested to reduce bleeding during surgery [4,22,24]. Many authors suggest the use of radiation therapy to slow the rate of recurrence, although radiation therapy alone has not been shown to be curative [3, 22, 33]. Chemotherapy as a primary treatment for these tumors has been shown to be largely ineffective, though 3 cases reported in this review reported the use of chemotherapy in addition to surgery and radiation therapy in the management of malignant sinonasal paragangliomas [4, 22].

4. Conclusion

We present a rare case of a paraganglioma presenting as a septal mass. To the best of the authors' knowledge, only one other case of nasal septal paragangliomas has been reported. Clinically, these lesions cause symptoms of obstruction, congestion, headache, and epistaxis. Recognizing that paragangliomas can arise from the nasal septum is crucial to accurately diagnose these tumors when evaluating septal lesions. A diagnosis of a paragangliomas can be confirmed based on the histopathologic imaging. These tumors are usually benign but require additional imaging as malignancy cannot reliably be ruled out on the basis of histology alone. Treatment of these tumors requires surgical resection, though radiation therapy has been to show slow growth and decrease recurrence.

https://doi.org/10.1155/2018/1413960

Conflicts of Interest

The authors declare that there are no conflicts of interest or financial disclosures.

Acknowledgments

The authors thank Dr. David Yau for providing the histopathology images and Dr. John Go for reviewing the radiology images.

References

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James H. Kim [ID], (1) Nathan Tu, (2) and Bozena Barbara Wrobel (3)

(1) Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA

(2) University of Southern California LAC+USC Medical Center Department of Otolaryngology, Los Angeles, CA, USA

(3) Associate Professor of Clinical Otolaryngology Head and Neck Surgery, Keck School of Medicine, Los Angeles, CA, USA

Correspondence should be addressed to James H. Kim; kim883@usc.edu

Received 7 August 2018; Accepted 19 November 2018; Published 6 December 2018

Academic Editor: Richard T. Miyamoto

Caption: FIGURE 1: Axial CT image showing a soft tissue mass centered at the posterior nasal septum with extension into the nasopharynx.

Caption: FIGURE 2: Intraoperative image of the mass protruding from the posterior septum within the left nasal cavity.

Caption: FIGURE 3: (a) H&E of the septal mass showing a "zellballen" pattern of clusters of epithelioid cells between bands of fibrillary stroma (20x). IHC staining showing positivity for (b) chromogranin A within epithelioid cells (40x), (c) synaptophysin within epithelioid cells (20x), and (d) S-100 within fibrillary cells (20x).
TABLE 1: Review of reported cases of sinonasal paragangliomas.

Case        Author        Year   Sex   Age

1        Harkins [9]      1957    F    52
2         Moran [10]      1962    F    89
3          Lack [3]       1976    F    50
4                                 F    50
5                                 M     8
6        Gosavi [11]      1978    M    65
7         Apple [12]      1982    F    50
8        Koegel [13]      1982    F    71
9       Himelfarb [14]    1985    M    41
10      Straehler [15]    1985    M    42
11        Ueda [16]       1985    F    31
12       Watson [17]      1988    M    56
13       Branham [18]     1989    F    25
14        Kuhn [19]       1989    M    62
15       Shimoda [20]     1989    M    58
16       Talbot [21]      1990    F    17
17       Nguyen [22]      1995    F    32
18       Biswas [23]      1999    F    45
19       Sharma [24]      1999    M    33
20     Welkoborsky [25]   2000    F    54
21                                F    56
22                                M    57
23        Scott [26]      2001    M    24
24       Lecuna [27]      2002    F    72
25      Ketabchi [28]     2003    F    72
26       Mouadeb [29]     2003    M    72
27      Lieberum [30]     2003    M    64
28        Askar [31]      2003    M    47
29        Rocha [32]      2005    M    45
30        Liess [33]      2007    F    64
31       Morales [7]      2007    F    41
32         Jin [34]       2008    F    23
33       Fasunla [35]     2008    F    39
34        Kisser [6]      2012    F    36
35       Zainine [36]     2012    F    43
36      Papaspyrou [4]    2013    F    33
37                                M    64
38                                F    56
39                                F    80
40                                M    57
41                                F    54
42       Granato [5]      2013    F    61
43       Michel [37]      2013    M    47
44     Amiraraghi [38]    2013    M    29
45        Arora [39]      2014    F    40
46      Yamaguchi [40]    2015    F    66
47        Aydin [41]      2015    F    32

48      Srivastava [8]    2016    M    60

Case            Location               Biological behavior

1             Ethmoid sinus                  Benign
2             Nasal cavity                   Benign
3           High nasal vault              Benign (local
                                         recurrence x3)
4           Middle turbinate                 Benign
5           Middle turbinate                 Benign
6           Middle turbinate                 Benign
7          Ethmoid, sphenoid,            Benign; ectopic
          and maxillary sinuses          ACTH production
8             Maxillary and           Malignant (extension
            sphenoid sinuses          into temporal lobe);
                                     catecholamine secretion
9           Middle turbinate                 Benign
10           Maxillary sinus                 Benign
11            Maxillary and                  Benign
             ethmoid sinuses
12         Inferior turbinate                Benign
13            Maxillary and                 Malignant
             ethmoid sinuses           (brain metastasis)
14       Superior nasal cavity,              Benign
              ethmoid sinus
15          Ethmoid sinus and          Malignant (cervical
         bilateral frontal fossa     lymph nodes metastasis)
16           Maxillary sinus                 Benign
17            Ethmoid sinus                 Malignant
                                        (bone metastasis)
18          Middle turbinate                 Benign
19      Frontal sinus (primary);            Malignant
           orbit, optic nerve,            (recurrence,
            cavernous sinus,              intracranial
          maxillary and ethmoid             invasion)
           sinuses (recurrent)
20            Nasal cavity                   Benign
21            Ethmoid sinus                 Malignant
                                       (brain metastasis)
22            Ethmoid sinus                  Benign
23            Nasal cavity,                  Benign
              ethmoid sinus
24      Ethmoid sinus (primary);     Malignant (recurrence,
       maxillary sinus (recurrent)     submandibular lymph
                                        nodes metastasis)
25            Nasal cavity                   Benign
26            Ethmoid sinus                  Benign
27             Frontal and               Benign; ectopic
             ethmoid sinuses             ACTH production
28          Middle turbinate                 Benign
29            Nasal cavity                   Benign
30       Sphenoid sinus, sella,              Benign
             cavernous sinus
31            Sphenoid and                   Benign
             ethmoid sinuses
32         Superior turbinate        Malignant (questionable
                                         transformation
                                       simulating Ewing's
                                        sarcoma/primitive
                                     neuroectodermal tumor)
33         Lateral nasal wall                Benign
34           Maxillary sinus                 Benign
35          Middle turbinate                 Benign
36            Nasal cavity                   Benign
37      Ethmoid sinuses, anterior     Malignant (recurrent,
        skull base, frontal lobe     intradural metastases)
        dura (primary); ethmoid,
           sphenoid, cavernous
        sinus, orbit (recurrent)
38            Ethmoid sinus                 Malignant
                                       (brain metastasis)
39            Nasal cavity,           Malignant (recurrent,
         maxilloethmoidal angle,     intracranial invasion)
         maxillary, ethmoid, and
            sphenoid sinuses
        (primary); nasal cavity,
          anterior skull base,
        frontal lobe (recurrent)
40            Ethmoid sinus                  Benign
41            Nasal cavity                   Benign
42            Nasal cavity                   Benign
43           Maxillary sinus           Malignant (cervical
                                     lymph node metastasis)
44        Cribriform plate and               Benign
            fovea ethmoidalis
       presenting as nasal polyps
45         Lateral nasal wall                Benign
46            Ethmoid sinus                  Benign
47     Nasal cavity, ethmoid sinus        Benign (local
                                         recurrence x2)
48        Anterior nasal septum              Benign

Case        Therapy             Follow-up

1       Exc, RT, lig ECA      NED at 1 year
2       Exc, ligation of     NED at 2 years
        external carotid
             artery
3       Exc, ligation of     NED at 11 years
        external carotid        following
             artery             final exc
4             Exc                No f/u
5             Exc            NED at 7 years
6           Exc, RT          NED at 2 years
       (incomplete cycle)
7      Exc, RT, hormonal     NED at 6 years
        pharmacotherapy
8              RT             No follow-up
                                available
9             Exc             NED at 1 year
10            Exc             No follow-up
                                available
11            Exc           NED at 2.5 years
12          Exc, RT          NED at 3 years
13        Exc, RT, CT        DOD at 3 years
14            Exc             NED at 1 year
15          Exc, RT          NED at 2 years
16      Embolization of      NED at 3 months
       internal maxillary
          artery, exc
17          Exc, RT          DOD at 15 years

18            Exc            NED at 1 month
19        CT, RT, exc;      DOD at 19 months
             vessel
       embolization, exc
         8 months later
         for recurrence
20            Exc            NED at 3 years
21          Exc, RT         DOD at 28 months

22          Exc, RT          NED at 2 years
23            Exc           NED at 15 months
24            Exc             No follow-up
                                available
25            Exc            NED at 8 months
26            Exc            NED at 4 years
27            Exc            NED at 2 years
28            Exc            NED at 2 years
29            Exc            NED at 5 months
30      Partial exc, RT      Residual tumor
                            stable at 2 years
31       No information       No follow-up
           available            available
32            Exc            NED at 3 years
33            Exc             NED at 1 year
34            Exc             NED at 1 year
35            Exc            NED at 2 years
36            Exc            NED at 15 years
37       2 exc, RT, CT      DOD at 60 months
38          Exc, RT         DOD at 28 months
39            Exc            DNOD 17 months
                            after 2nd surgery
40            Exc            NED at 2 years
41            Exc            NED at 3 years
42            Exc            NED at 2 years
43          Exc, RT           NED at 1 year
44            Exc            NED at 3 years
45            Exc            NED at 2 years
46            Exc             NED at 1 year
47            Exc              NED 5 years
                             after final exc
48            Exc           NED at 18 months

RT, radiation therapy; CT, chemotherapy; Exc, surgical
excision; NED, no evidence of disease; DOD, died of disease.
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Title Annotation:Case Report
Author:Kim, James H.; Tu, Nathan; Wrobel, Bozena Barbara
Publication:Case Reports in Otolaryngology
Article Type:Report
Date:Jan 1, 2018
Words:4145
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