Case Report
A Case of Adenoid Cystic Carcinoma Parotid with Perineural Spread VS Jain*, KK Singh+, Y Sachdeva# MJAFI 2007; 63 :67-68 Key Words: Adenoid cystic carcinoma; Parotid; Perineural spread
Introduction denoid cystic carcinoma (ACC) of salivary glands is a slow growing malignant tumour, characterized by wide local infiltration, perineural spread, a propensity to local recurrence and late distant metastases [1,7]. ACC is a rare tumour and forms about 1% of all malignant tumours of the oral and maxillofacial region [2]. ACC constitutes about 21.9% of all salivary gland malignancies [3]. ACC has three histopathologic patterns viz. tubular, cribriform and a solid pattern. Solid pattern is associated with increased local recurrence, high metastic rate and higher mortality [4]. We present a case report of solid ACC of parotid salivary gland in a 42 year old female with intracranial extension. Despite good initial response to radiotherapy, patient developed recurrence in the eyelid, orbit, maxilla of the same side and pons. The patient expired within a year of diagnosis.
within normal limits. Computed tomography (CT) scan of parotid revealed enlarged parotid (6.6 x 4.1 cm) with intense enhancement without areas of necrosis or bone destruction (Fig. 1). CT scan of the brain revealed left temporo-parietal intensely enhancing lesion extending from base of skull, adjacent to dorsum sellae, with involvement of cavernous sinus and the left cerebello pontine angle (CP angle) with bone destruction. Histopathology of left parotid swelling revealed adenoid cystic carcinoma of solid variant. The patient was planned for external radiotherapy covering parotid swelling as well as intra-cranial lesion by anterior and posterior wedged paired fields by Telecobalt Unit to a total dose of 6600cGy in 33 fractions (field reduction was done after 25 fractions). The patient completed radiation treatment and was on regular follow up. The response with the radiation therapy was encouraging. However, two months after radiation, residual swelling (1.5 x 2 cm) was seen in the left parotid region. CT scan of brain revealed no residual lesion but radiation induced gliosis in left temporal region. Five months after completion of radiation treatment, the patient
Case Report A 42 year old female was referred to our centre as a case of carcinoma of left parotid with intracranial extension. The patient came with complaints of pain and swelling on left side of face since one year. The swelling gradually increased in size. Patient had forgetfulness, one episode of convulsion and inability to close left eye since one month. On clinical examination patient was conscious and oriented. Motor and sensory systems were normal. However personal history suggested that symptoms of forgetfulness were observed earlier as well. Left facial nerve palsy of lower motor neuron type (LMN), left lateral rectus palsy and left eye conjunctival congestion were present. Vision was normal in both eyes. A swelling of 6 x 7 cm was present in left parotid region. It was hard, fixed and non-tender. A healthy scar mark of biopsy was present over the swelling. The routine blood counts, biochemical tests and radiograph of the chest were within normal limits. Ultrasonography of abdomen and pelvis was
Fig. 1: CT scan at parotid level shows enlarged left parotid gland
A
*
Associate Professor, +Professor & Head, Department of Radiotherapy, #Assistant Professor, Department of Radiodiagnosis, Rural Medical College (RMC) of Pravara Institute of Medical Sciences (PIMS), (Deemed University), Loni. Received : 30.04.2006; Accepted : 21.09.2006
68
Fig. 2 : CT scan of orbit and maxillary region shows heterogeneously enhancing mass lesion in the left orbit and involving whole of the left maxillary sinus with destruction of roof, medial wall and anterolateral wall of left maxilla
noticed multiple small swellings over left lower eyelid. Fine needle aspiration cytology done from one of these swellings was positive for malignancy. The patient was given palliative chemotherapy of injection ifosfamide, cisplatin and etoposide in combination. After completion of two cycles of chemotherapy for left lower eyelid swellings, patient developed left maxillary recurrence. CT scan of orbit and maxillary region revealed heterogeneously enhancing mass lesion in the left orbit, involving whole of the left maxillary sinus with destruction of roof, medial wall and anterolateral wall of left maxilla (Fig. 2). As the response to chemotherapy was poor, the patient was again subjected to the radiation therapy of left maxilla and left eye. Patient responded partially to radiation therapy. Two months later the patient came with the complaints of weakness in both lower limbs and inability to walk. CT scan of brain showed peripherally enhancing lesion (2.6 x 2.04 cm) with central area of necrosis and perifocal oedema in region of pons. Patient was subjected to palliative radiation of the brain covering pons region, but patient did not respond and eventually expired one month after recurrence in pons region.
Discussion ACC is a rare cancer that arises in major and minor salivary glands with an affinity for nerve invasion [4, 5]. The most common malignancies associated with head and neck and perineural spread (PNS) are tumours of salivary, mucosal and cutaneous origin. For salivary gland origin, typical primary sites include the parotid gland and minor salivary glands mainly in the palate. Any
Jain, Singh and Sachdeva
histological sub type can spread perineurally but ACC is most notorious [6]. PNS is often present at the time of diagnosis and rarely presents prior to the detection of a head and neck primary cancer. It is also common for PNS to constitute, or occur simultaneously to disease recurrence. A typical history of a new cranial neuropathy in a patient who had previously undergone resection of a lesion is available, PNS being the cause of the neuropathy [6]. Imaging of PNS with magnetic resonance imaging (MRI) is analogous to imaging with CT but is far more sensitive. The MRI facility is not available at our centre, and the patient could not afford consultation at a higher centre. Available CT images could not explain the perineural spread of the disease but patient’s clinical presentation during successive follow-ups explained the antegrade and retrograde perineural spread. Majority of the cases of ACC are slow growing with prolonged survival. In the presence of initial bad prognostic factors of solid pattern on histopathology and perineural spread, despite a good response of the primary and intracranial lesion to radiation therapy, there is a high risk of local recurrence and metastases with the disease following a rapid and fulminating course within a period of just one year. Conflicts of Interest None identified References 1. Avery CME, Moody AB, McKinna FE, Taylor J, et al. Combined treatment of adenoid cystic carcinoma of the salivary glands. Int J Oral Maxillofac Surg 2000; 29: 277-9. 2. Kokemueller H, Eckardt A, Brachvogel P, Hausamen E. Adenoid cystic carcinoma of the head and neck – a 20 years experience. Int J Oral Maxillofac Surg 2004; 33: 25-31. 3. Luo XL, Sun MY, Lu CT, Zhou ZH. The role of Schwann cell differentiation in perineural invasion of adenoid cystic and mucoepidermoid carcinoma of the salivary glands. Int J Oral Maxillofac Surg 2006; 35: 733-9. 4. Huang MX, Ma DQ, Sun KH, Yu GY, Guo CB, Gao F. Factors influencing survival rate in adenoid cystic carcinoma of the salivary glands. Int J Oral Maxillofac Surg 1997; 26: 435-9. 5. Eshleman JS, Brown PD, Foote RL, Strome SE. Does adjuvant radiotherapy for primary adenoid cystic carcinoma of the parotid gland reduce the risk for locoregional recurrence and associated morbidity? Int J radiation oncology biology physics 2000; 48: 325. 6. Lawrence EG. MR imaging of perineural tumour spread. Magnetic resonance imaging clinics 2002; 10: 511-25. 7. Umeda M, Komatsubara H, Ojima Y, Minamikawa T, et al. Establishment and characterization of metastasizing cell lines from heterotransplanted human adenoid cystic carcinoma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;98:211-6.
MJAFI, Vol. 63, No. 1, 2007
A Case of Adenoid Cystic Carcinoma Parotid with Perineural Spread VS Jain*, KK Singh+, Y Sachdeva# MJAFI 2007; 63 :67-68 Key Words: Adenoid cystic carcinoma; Parotid; Perineural spread
Introduction denoid cystic carcinoma (ACC) of salivary glands is a slow growing malignant tumour, characterized by wide local infiltration, perineural spread, a propensity to local recurrence and late distant metastases [1,7]. ACC is a rare tumour and forms about 1% of all malignant tumours of the oral and maxillofacial region [2]. ACC constitutes about 21.9% of all salivary gland malignancies [3]. ACC has three histopathologic patterns viz. tubular, cribriform and a solid pattern. Solid pattern is associated with increased local recurrence, high metastic rate and higher mortality [4]. We present a case report of solid ACC of parotid salivary gland in a 42 year old female with intracranial extension. Despite good initial response to radiotherapy, patient developed recurrence in the eyelid, orbit, maxilla of the same side and pons. The patient expired within a year of diagnosis.
within normal limits. Computed tomography (CT) scan of parotid revealed enlarged parotid (6.6 x 4.1 cm) with intense enhancement without areas of necrosis or bone destruction (Fig. 1). CT scan of the brain revealed left temporo-parietal intensely enhancing lesion extending from base of skull, adjacent to dorsum sellae, with involvement of cavernous sinus and the left cerebello pontine angle (CP angle) with bone destruction. Histopathology of left parotid swelling revealed adenoid cystic carcinoma of solid variant. The patient was planned for external radiotherapy covering parotid swelling as well as intra-cranial lesion by anterior and posterior wedged paired fields by Telecobalt Unit to a total dose of 6600cGy in 33 fractions (field reduction was done after 25 fractions). The patient completed radiation treatment and was on regular follow up. The response with the radiation therapy was encouraging. However, two months after radiation, residual swelling (1.5 x 2 cm) was seen in the left parotid region. CT scan of brain revealed no residual lesion but radiation induced gliosis in left temporal region. Five months after completion of radiation treatment, the patient
Case Report A 42 year old female was referred to our centre as a case of carcinoma of left parotid with intracranial extension. The patient came with complaints of pain and swelling on left side of face since one year. The swelling gradually increased in size. Patient had forgetfulness, one episode of convulsion and inability to close left eye since one month. On clinical examination patient was conscious and oriented. Motor and sensory systems were normal. However personal history suggested that symptoms of forgetfulness were observed earlier as well. Left facial nerve palsy of lower motor neuron type (LMN), left lateral rectus palsy and left eye conjunctival congestion were present. Vision was normal in both eyes. A swelling of 6 x 7 cm was present in left parotid region. It was hard, fixed and non-tender. A healthy scar mark of biopsy was present over the swelling. The routine blood counts, biochemical tests and radiograph of the chest were within normal limits. Ultrasonography of abdomen and pelvis was
Fig. 1: CT scan at parotid level shows enlarged left parotid gland
A
*
Associate Professor, +Professor & Head, Department of Radiotherapy, #Assistant Professor, Department of Radiodiagnosis, Rural Medical College (RMC) of Pravara Institute of Medical Sciences (PIMS), (Deemed University), Loni. Received : 30.04.2006; Accepted : 21.09.2006
68
Fig. 2 : CT scan of orbit and maxillary region shows heterogeneously enhancing mass lesion in the left orbit and involving whole of the left maxillary sinus with destruction of roof, medial wall and anterolateral wall of left maxilla
noticed multiple small swellings over left lower eyelid. Fine needle aspiration cytology done from one of these swellings was positive for malignancy. The patient was given palliative chemotherapy of injection ifosfamide, cisplatin and etoposide in combination. After completion of two cycles of chemotherapy for left lower eyelid swellings, patient developed left maxillary recurrence. CT scan of orbit and maxillary region revealed heterogeneously enhancing mass lesion in the left orbit, involving whole of the left maxillary sinus with destruction of roof, medial wall and anterolateral wall of left maxilla (Fig. 2). As the response to chemotherapy was poor, the patient was again subjected to the radiation therapy of left maxilla and left eye. Patient responded partially to radiation therapy. Two months later the patient came with the complaints of weakness in both lower limbs and inability to walk. CT scan of brain showed peripherally enhancing lesion (2.6 x 2.04 cm) with central area of necrosis and perifocal oedema in region of pons. Patient was subjected to palliative radiation of the brain covering pons region, but patient did not respond and eventually expired one month after recurrence in pons region.
Discussion ACC is a rare cancer that arises in major and minor salivary glands with an affinity for nerve invasion [4, 5]. The most common malignancies associated with head and neck and perineural spread (PNS) are tumours of salivary, mucosal and cutaneous origin. For salivary gland origin, typical primary sites include the parotid gland and minor salivary glands mainly in the palate. Any
Jain, Singh and Sachdeva
histological sub type can spread perineurally but ACC is most notorious [6]. PNS is often present at the time of diagnosis and rarely presents prior to the detection of a head and neck primary cancer. It is also common for PNS to constitute, or occur simultaneously to disease recurrence. A typical history of a new cranial neuropathy in a patient who had previously undergone resection of a lesion is available, PNS being the cause of the neuropathy [6]. Imaging of PNS with magnetic resonance imaging (MRI) is analogous to imaging with CT but is far more sensitive. The MRI facility is not available at our centre, and the patient could not afford consultation at a higher centre. Available CT images could not explain the perineural spread of the disease but patient’s clinical presentation during successive follow-ups explained the antegrade and retrograde perineural spread. Majority of the cases of ACC are slow growing with prolonged survival. In the presence of initial bad prognostic factors of solid pattern on histopathology and perineural spread, despite a good response of the primary and intracranial lesion to radiation therapy, there is a high risk of local recurrence and metastases with the disease following a rapid and fulminating course within a period of just one year. Conflicts of Interest None identified References 1. Avery CME, Moody AB, McKinna FE, Taylor J, et al. Combined treatment of adenoid cystic carcinoma of the salivary glands. Int J Oral Maxillofac Surg 2000; 29: 277-9. 2. Kokemueller H, Eckardt A, Brachvogel P, Hausamen E. Adenoid cystic carcinoma of the head and neck – a 20 years experience. Int J Oral Maxillofac Surg 2004; 33: 25-31. 3. Luo XL, Sun MY, Lu CT, Zhou ZH. The role of Schwann cell differentiation in perineural invasion of adenoid cystic and mucoepidermoid carcinoma of the salivary glands. Int J Oral Maxillofac Surg 2006; 35: 733-9. 4. Huang MX, Ma DQ, Sun KH, Yu GY, Guo CB, Gao F. Factors influencing survival rate in adenoid cystic carcinoma of the salivary glands. Int J Oral Maxillofac Surg 1997; 26: 435-9. 5. Eshleman JS, Brown PD, Foote RL, Strome SE. Does adjuvant radiotherapy for primary adenoid cystic carcinoma of the parotid gland reduce the risk for locoregional recurrence and associated morbidity? Int J radiation oncology biology physics 2000; 48: 325. 6. Lawrence EG. MR imaging of perineural tumour spread. Magnetic resonance imaging clinics 2002; 10: 511-25. 7. Umeda M, Komatsubara H, Ojima Y, Minamikawa T, et al. Establishment and characterization of metastasizing cell lines from heterotransplanted human adenoid cystic carcinoma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;98:211-6.
MJAFI, Vol. 63, No. 1, 2007
