General
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• First described in 1995 by Eichhorn and Scully (Mod Pathol 1995;8:731)
• Very rare ovarian tumors
• Postulated to be of surface epithelial origin
• Adenoid cystic carcinomas are more aggressive than basaloid carcinomas, present at older age

Pathophysiology
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• Postulated origin is through metaplasia of coelomic / surface epithelium
• Rarely may be teratomatous origin

Clinical features
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• Abdominal pain, unilateral abdominal mass
• Adenoid cystic carcinoma: mean age 67 years, range 60 to 78 years
• Basaloid carcinoma: mean age 49 years, range 19 to 65 years

Case reports
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• 23 year old woman (Arch Pathol Lab Med 2000;124:1529)
• 45 year old woman (Mod Pathol 1996;9:413)

Treatment
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• Unilateral salpingo-oophorectomy (Mod Pathol 1996;9:413) with or without adjuvant radiotherapy (Int J Radiat Oncol Biol Phys 2000;46:551)
• Chemotherapy for metastatic disease

Gross description
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• Unilateral
• Encapsulated
• Solid with small cystic spaces (Mod Pathol 1995;8:731)

Micro description
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• Small uniform and basaloid cells in tubular pattern with cribriform and focal solid areas
• Multiple round hyaline stromal cylinders
• Minimal pleomorphism or mitotic activity, no necrosis
• With myoepithelial differentiation, tumor cells are ovoid to spindled (Arch Pathol Lab Med 2000;124:1529, Mod Pathol 1996;9:413)

Micro images
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Adenoid cystic carcinoma

Basaloid and ameloblastoma-like features

S100

GFAP

Positive stains
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• Cytokeratins CAM 5.2 and AE1/3, CEA
• Peripheral cells positive for muscle-specific actin, S100, GFAP

Negative stains
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• Alcain blue, alpha inhibin, ER, PR, desmin, AFP

Electron microscopy description
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• Myoepithelial component shows convoluted nuclei and filamentous structures associated with dense bodies

Differential diagnosis
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• Metastasis of ACC from another site (Mod Pathol 1995;8:731)
• Primary or metastatic ACC-like carcinoma (Am J Surg Pathol 1988;12:134)
• Reticular variant of a yolk-sac tumor (Blaustein's Pathology of the Female Genital Tract)

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