|ADENOID CYSTIC CARCINOMA OF THE HARD PALATE
Adenoid cystic carcinoma(ACC) is a rare malignant tumor that affects the major and minor salivary glands. 30-40% of these carcinomas occur as head & neck tumors. It is well known for its prolonged clinical course and the tendency for delayed onset of distant metastasis.Here we are presenting a case report of hard palate adenoid cystic carcinoma.
A 52 year old male patient reported to the department of otorhinolaryngology, with complaints of ulcerative lesion over the hard palate which was progressive in nature since 2years and swelling over right side of neck since 1year which was slowly progressive. Personal history was negative for alcohol or tobacco consumption.
Examination revealed an ulcerative lesion measuring 2*2.5cm noted in the posterior aspect of hard palate towards rightside of the midline, covered with slough and surrounding area of induration with underlying bony defect.Figure 1
Right cervical level 2 lymphadenopathy measuring 2*2cm hard ,non tender and mobile was present.
FNAC of lymphnode revealed features suggestive of pleomorphic adenoma.
Biopsy from the lesion also revealed features suggestive of pleomorphic adenoma
Imaging- CECT neck revealed enlarged level 2 cervical lymph node on right side.
Wide excision was done with 1cm margin. Histopathology was reported as adenoid cystic carcinoma. Figure 2
Patient underwent radiotherapy and is on regular follow up since then with no evidence of recurrence.Figure 3
Adenoid Cystic Carcinoma(ACC) is a rare malignant tumor that affects both major and minor salivary glands. It is the commonest malignant tumor of minor salivary glands. Rarely, it may also present as a primary intraosseous tumor of the mandible and maxilla.The tumor affects men and women equally in the fifth decade of life as observed in the present case
Studies have found that smoking, alcohol consumption did not increase the risk of salivary gland cancer with possible increase in patients who received radiation treatment to the head and neck, ultraviolet light treatment or full mouth dental x-rays.In the present case also the patient does not give any history as shown by the previous studies.
The most frequent clinical presentation of Minor salivary gland tumor was in the form of a soft or slightly indurated lesion located in the hard or soft palate as opposed to the present case which presented as ulcer.
The diagnosis of Minor salivary gland tumors is based on a combination of clinical history and physical exploration, Magnetic Resonance Imaging, Computed Tomography alone or combined with sialography and Fine Needle Aspiration Biopsy.
CT scans are important to delineate the tumor, to plan extent of surgery and to look out for recurrences as a follow up postoperatively.Imaging for these patients must include axial views to define the anteriorposterior dimension, assess bone destruction, especially of erosion of pterygoid plates and skull base and coronal views to evaluate superior extension and paranasal sinus involvement. An MRI may be useful in evaluating cranial base and CNS.
After imaging, a biopsy needs to be performed.Three histological patterns of growth have been described.The typical Adenoid cystic carcinoma has a cribrifrom pattern nests and columns of cells of rather bland appearance are arranged concentrically around gland-like spaces which are filled with hyaline PAS positive material.Some have a predominantly tubular pattern while a few others have a solid pattern. If nodes are present, they require an FNA.
Treatment of ACC includes complete excision of the local disease followed by post-operative radiotherapy.The same treatment plan was followed in our case. Neutron radiotherapy has been studied in this disease with some authors suggesting a role for it.Small lesions may be excised transorally. Large tumors requiring partial maxillectomy can be approached through lateral rhinotomy incision or midface degloving. If cancer extends through the palate require total maxillectomy.Radiation is effective for both squamous cell tumors and salivary gland tumors, and that while surgery has a role in management of hard palate tumors, so does radiation therapy.Patients who receive only radiation are either poor surgical candidates with unresectable disease or have neoplasms so small they can be treated without surgery.Compared to mandible, the residual maxilla does not move, making a prosthesis a good option for functional recovery. Because of this, the Obturator was the “gold standard” for years. However, advancements in reconstructive surgery have lead to multiple publications in recent years advocating tissue reconstruction.In patients with severe trismus, a free flap is better.Soft tissue defects of the hard palate are essentially a non-issue, as the hard palate can be left to granulate with relatively little patient morbidity.
Chemotherapy using one or a combination of drugs (cyclophosphamide, 5- fluorouracil, mitomycin-C and cisplatin) has been used with some success and remission.
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