ADENOID CYSTIC CARCINOMA

INTRODUCTION

Also known as cylindroma,adenocystic basal cell carcinoma,pseudoadematous basal cell carcinoma,basaloid mixed tumor. ACC is a clinically and pathologically well defined entity and occurs primarily in the major SG and relatively frequently in the oral accessory SGs particularly the palate
Rarely also occurs in some head and neck areas like ceruminal glands of ears and lacrimal glands of eyes

HISTORICAL REVIEW

Foote and Frazell proposed the current name Adenoid cystic carcinoma During 48 yrs study of AFIP proposed it is 4th most common malignant tumor following MCC, Acinic cell carcinoma, Adeno carcinoma NOS

CLINICAL FINDINGS
Age & sex incidence Occur in 5,6 & 7 decades of life Although rare in young people occasionally occur in first 2 decades Show equal sex predilection

ANATOMIC LOCATION

In major SG mostly occur in parotid and sub mandibular gland, rarely in sub lingual gland In minor SG palate is most common followed by tongue, cheek, upper lip, floor of mouth, oro pharynx and lower lip In tongue 3rd most common tumor following SCC and MEC

CLINICAL FINDINGS
Clinical complains Slow growing tumor Pain and tenderness occur during tumor growth Fixation to skin and surrounding structures develop in later stages Cause paralysis of facial nerve

GROSS FINDINGS

Present as a fairly well defined mass within the substance of involved gland On cut section tumor lacks encapsulation but can be demarcated from surrounding SG tissue

Firm in consistency and white or grayish white in color

Grossly observable cystic zones are uncommon

Close Inspection shows infiltration into surrounding parenchymal tissue

MICROSCOPIC FINDINGS

ACC has variety of microscopic pattern The cells that form these patterns are uniform in size, shape and staining qualities called isomorphic cells

Various microscopic patterns Cribriform Tubular solid

Cribriform

It is most important and most recognized pattern Tumor cells contain dark basophilic nuclei and scanty cytoplasm. Nucleoli is rarely observed These tumor cells arranged in nests of variable size and shape that contain many circular or ovoid spaces give rise to Swiss Cheese pattern .

These spaces are filled with eosinophilic hyaline material (PAS positive)&/or lightly basophilic myxoid ground substance (alcian blue positive)
These spaces occur due to accumulation of extracellular substance like GAGs & duplicated basal lamina.

Tubular type

It is second major microscopic pattern Tumor cells are identical to those observed in cribriform pattern Here the arrangement of cells is different and they form ductal structures

In longitudinal sections they viewed as tubules

Lumen of duct may contain mucous substance and stains positive for PAS Some times cribriform may coexist with tubular pattern

The tubules have an inner layer of cells with eosinophilic cytoplasm & an outer layer of basaloid cells.

Rarely the tubules are apparently coiled upon themselves,producing a NECKLACE APPEARANCE.

Solid

It is third microscopic pattern

Here tumor cells arranged in nests of variable size and shape without ovoid spaces and tubular structures Occasionally areas of necrosis may be found within the solid nests
In addition to necrosis cellular pleomorphism and mitosis are also observed

MICROSCOPIC FINDINGS

A major microscopic feature in most ACC is the propensity for the tumor to involve peripheral nerves. It invades into perineural space and sometimes into nerves targetoid appearance. Neurovascular structures rarely involved. Although perineural invasion is characteristic of ACC but it is not unique to the tumor, can also seen in PLGA and salivary duct adenocarcinoma.

FNAC FINDINGS

Aspiration biopsies consist of round or ovoid basophilic cells arranged in branching structures. Amorphous, hyaline globoid structures with tumor cells is characteristic feature of ACC.

IHC

It reveals the presence of 2 cell population Ductal cells Myoepithelial cells Ductal cells express Carcino embryonic antigen Epithelial membrane antigen Low molecular weight cytokeratin S-100 protein

IHC

Myoepithelial cells express

Muscle specific antigen Low molecular weight cytokeratin S-100 protein

Laminin and collagen type IV were

demonstrated in cribriform tubular pattern that line the pseudo cysts and outer surfaces of tubular areas

TREATMENT AND PROGNOSIS

Surgical excision is the treatment of choice. Because it is very much prone to local recurrence and eventual distant metastasis adjunct radiotherapy will improve patient survival.

Regional lymph node metastasis is uncommon.

Prognosis is poor because of local recurrence and distant metastasis. Solid pattern has worst prognosis.

TREATMENT AND PROGNOSIS

Tumors arising in maxillary sinus and submandibular gland has poor prognosis. Perineural invasion has little effect on prognosis.

Metastatic spread most commonly occurs to lungs and bones. Lesions in the palate and maxillary sinus may invade upward to the brain.