Eur Arch Otorhinolaryngol DOI 10.1007/s00405-014-3441-4

CASE REPORT

Adenoid cystic carcinoma sphenoid sinus with intracranial extension treated by radical radiotherapy: a rare case Prashanth Giridhar • Supriya Mallick M. A. Laviraj • Suman Bhasker



Received: 8 December 2014 / Accepted: 8 December 2014 Ó Springer-Verlag Berlin Heidelberg 2014

Keywords Sphenoid  Adenoid cystic carcinoma  Radiotherapy  Conformal  IMRT

Introduction The sino-nasal tract is an uncommon site for primary malignancy. Adenoid cystic carcinoma (ACC) is a rare tumour and may involve the sino-nasal tract. Sphenoid sinus is a very rare location for ACC. We present a case of ACC of sphenoid sinus with intracranial extension treated successfully with radical radiation.

Case report 38-year-old gentleman presented with frontal headache and double vision of 2 months duration. A non-contrast computed tomography of head revealed erosion of skull bone involving the sphenoid and left petrous apex with a soft tissue extending to bilateral cavernous sinus (CS) and left orbital apex. A contrast-enhanced MRI of the brain revealed a mass in left sphenoid sinus involving the left CS. The details of disease extension and post-treatment scan showing response to therapy have been depicted in Fig. 1. Biopsy from the left sphenoid sinus revealed a CD 117-positive tumour suggestive of ACC. Tumour was infiltrating the underlying bone. No perineural infiltration (PNI) or lymphovascular invasion was identified. Patient was deemed inoperable because of bilateral cavernous P. Giridhar  S. Mallick (&)  M. A. Laviraj  S. Bhasker Department of Radiation Oncology, All India Institute of Medical Sciences, New Delhi, India e-mail: [email protected]

sinus infiltration and proximity to the internal carotid artery and was referred for radiotherapy. The patient was evaluated in our multi-disciplinary head and neck cancer clinic. On examination, the patient had an ECOG performance status of 1 with manifest strabismus of the left eye. All eye movements were of normal range and both pupils were reactive. Cranial nerve examination revealed a sensory loss in the region of maxillary division of trigeminal nerve on the right side of face. No palpable pre-auricular, post-auricular or cervical nodes were present. A chest X-ray was normal. In view of the disease extension the patient was planned to be treated with radical radiation. Radiation was delivered by image-guided intensitymodulated radiotherapy with pinnacle (8.0) planning system. The gross tumour volume (GTV) was delineated with the help of pre-treatment MRI. An isotropic expansion of 5 mm was added to the GTV as high-risk Clinical Target Volume (CTV). Low risk CTV included the entire frontal, bilateral ethmoid and maxillary sinus and the nasal cavity. An isotropic 3 mm expansion of the CTV formed the planning target volume (PTV). The target delineation and dose volume histogram has been depicted in Fig. 1. A dose of 66 Gray in 33 fractions over 6.5 weeks was prescribed. A cone beam CT was used twice weekly as shown in Fig. 2. Table 1 summarizes the dose constraints given and achieved to the OAR. The patient tolerated the treatment well and developed grade II dermatitis and grade II conjunctivitis only. Three months post-treatment, a CECT of face and neck revealed a partial response. However, the patient was symptom free, strabismus disappeared and loss of sensation on right cheek improved. At 6 months post-treatment, a repeat CECT of face and neck revealed a non-enhancing plaque like soft tissue density in bilateral sphenoid sinus

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Fig. 1 a–c The target delineation (GTV, CTV, PTV), d dose colour wash for IGIMRT planning, e, f dose volume histogram of PTV

suggestive of post-treatment changes. There was no evidence of intracranial extension or orbital extension. Patient was asymptomatic and clinical examination revealed no abnormalities.

Discussion ACC of sino-nasal tract is a rare tumour constituting 10 % of malignancies in this region [1]. Sphenoid sinus ACC is even rarer. In a meta-analysis of 520 patients of ACC of sino-nasal tract, sphenoid sinus constituted 3 % (n = 16) of cases [2]. Surgery followed by post-operative radiation is considered optimum treatment. However, because of anatomically difficult location of the tumour achieving a R0 resection is challenging. Presence of positive/close margins and tumour epicentre in sphenoid is associated with poor prognosis [2]. Adjuvant radiation is considered to impart overall and disease-free survival benefit in sinonasal ACC [1]. ACC has a propensity for PNI. Interestingly, PNI is not found to affect outcomes in sino-nasal ACC [2]. ACC though considered a radiosensitive disease but usually cannot be cured by radiation alone [3, 4]. Dose of 70 Gray or more is required for local control of ACC [5]. Radiation has been shown to reduce perineural invasion by inhibiting paracrine mechanisms of PNI, affecting the nerve micro environment and also by direct effect on

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cancer cells [6]. This may have played a role in the local control of disease in our case. In a case report by Muller et al., ACC involving the main-stem bronchi and trachea was successfully treated by radical radiation to a dose of 66 Gray in 2 Gray fractions over 7 weeks. The patient was reported to be in complete remission at 27 months posttreatment [7]. In our case this dose could not be reached as the dose to the surrounding neural structures became prohibitively high. So a dose of 66 Gray was prescribed. In the present case the tumour showed good response to radiation and patient has complete resolution of all symptoms. The CECT images corroborated with the clinical findings and there was no active residual disease. Chemotherapy has limited benefit in ACC and these tumours are considered chemo-resistant. Point should be made that ACC shows high EGFR expression. However, there is little consensus about the effectiveness of such therapy [8, 9].

Conclusion In sphenoid sinus ACC with intracranial extension, where surgery is associated with high morbidity and where there is no clear role of chemotherapy and targeted therapy, radical radiation seems to be a good option to achieve local control and relief of symptoms with minimal acute

Eur Arch Otorhinolaryngol

Fig. 2 a–c Axial, coronal and sagittal scan image showing the extension of the disease, d–f axial, coronal and sagittal scan image showing posttreatment response (6 months) Table 1 Dose constraints and dose limit achieved in IGIMRT planning OAR

Dmax (Gy)

Volume receiving dose

Constraints given (Gy)

Brain stem

62.71

\1 % more than 54 Gy

Dmax \ 54

Left cochlea PRV

36.91

Right cochlea PRV Left temporal lobe

36.16 70.94

\1 % more than 65 Gy

Right temporal lobe

70.31

\1 % more than 65 Gy

Conflict of interest

The authors have no conflict of interest.

Dmax \ 54 Dmax \ 54 Dmax \ 65 Dmax \ 65

6.76

Dmax \ 45

Left optic nerve

69.81

Dmax \ 54

Right optic nerve

69.05

Dmax \ 54

Optic chiasma

70.23

Dmax \ 54

Left eye

69.39

Dmax \ 45

Right eye

69.92

Dmax \ 45

Spinal PRV

in ACC of paranasal sinuses treated by radiation alone. A review of such cases is needed to better define the role of radiation alone.

morbidity. ACC is notorious for local relapse and distant metastases. A close follow-up with imaging is warranted. There is limited literature reviewing the local control rates

References 1. Lupintti D, Roberts B, Williams MD, Kupferman ME et al (2007) Sinonasal adenoid cystic carcinoma—the MDACC experience. Cancer 110:2726–2731 2. Amit M, Binenbaum Y, Sharma K, Ramer N et al (2013) Adenoid cystic carcinoma of paranasal sinuses and nasal cavity—a metaanalysis. J Neur Surgery B 74:118–125 3. Wiseman SM, Popat SR, Rigual NR et al (2002) Adenoid cystic carcinoma of the paranasal sinuses or nasal cavity: a 40-year review of 35 cases. Ear Nose Throat J 81:510–517 4. Leafstedt SW, Gaeta JF, Sako K, Marchetta FC, Shedd DP (1971) Adenoid cystic carcinoma of major and minor salivary glands. Am J Surg 122:756–762 5. Schulz-Ertner D, Didinger B, Nikoghosyan A, Ja¨kel O (2003) Optimization of radiation therapy for locally advanced adenoid

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Eur Arch Otorhinolaryngol cystic carcinomas with infiltration of the skull base using photon intensity-modulated radiation therapy (IMRT) and a carbon ion boost. Strahlenther Onkol 179(5):345–351 6. Bakst RL, Lee N, He S, Chernichenko N, Chen CH et al (2012) Radiation impairs perineural invasion by modulating the nerve microenvironment. PLoS One 7(6):e39925. doi:10.1371/journal. pone.0039925 (epub 2012 Jun 29) 7. Muller A, Stockamp B, Schnabel T (2000) Successful radiation therapy of adenoid cystic carcinoma of lung. Oncology 58(1):15–17

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8. Huang Y, Yu T, Fu X, Chen J et al (2013) EGFR inhibition prevents in vitro growth of salivary adenoid cystic carcinoma. BMC Cell Biol 14:13. doi:10.1186/1471-2121-14-13 9. Lee SK, Kwon MS, Lee YS, Choi SH (2012) Prognostic value of expression of molecular markers in ACC of salivary glands compared with lymph node metastases—a retrospective study. World J Surg Onc 10:266

Adenoid cystic carcinoma sphenoid sinus with intracranial extension treated by radical radiotherapy: a rare case.

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