Papillary carcinoma is the most common type of thyroid cancer in both children and adults, comprising about 75-80% of thyroid cancer cases.
It is easily treatable since it is slow growing and susceptible to radioiodine therapy and surgical removal.
Papillary carcinoma has a tendency to spread to the nearby lymph nodes of the head and neck and may manifest with palpable lymphadenopathy on clinical exam.
This pathologic finding is named for a comic book character known as Little Orphan Annie who is drawn with egg-shaped, clear eyes but no pupils. It is used to describe how papillary carcinoma is made up of papillae that have cells with clear nuclei; these cells are surrounded with inclusions of the nuclear envelope called nuclear grooves.
This describes the “empty eyes” of Orphan Annie seen microscopically from a biopsy of this tumor. The nuclei appear empty because all of the heterochromatin has been pushed to the edges of the cell, creating a central clearing.
These distinct calcified structures are commonly described as concentric whorls and are found in about half of papillary carcinomas cases. Psammoma bodies are not unique to this disease; they may also be associated with serous cystadenocarcinoma of the ovary, meningioma, and mesothelioma.
Patients may be exposed to ionizing radiation from treatment for other head and neck cancer or environmental exposure from nuclear fallout from atomic weapons or power plants. Any such exposure to radiation raises lifetime risk of developing thyroid cancer.
Both of these proto-oncogenes are involved in the pathogenesis of several cancers. RET encodes a tyrosine kinase receptor while the BRAF gene codes for a serine/threonine kinase gene product that is involved in the MAP-kinase (MAPK) cell signaling pathway. Having a mutation in either of these genes increases one's lifetime risk of developing papillary thyroid cancer and has a greater chance of metastases and local tissue invasion if a cancer does form.
Also known as familial colorectal polyposis, Gardner syndrome is one of the hereditary causes of colon cancer. It is characterized by numerous polyps in the colon as well as other bone and soft tissue tumors like osteomas and epidermoid cysts. Inheritance of this syndrome will increase the risk of developing papillary thyroid cancer as well.
Commonly associated with PTEN tumor suppressor gene mutations, this inherited disease is one of several GI hamartomatous polyp syndromes that also manifests with breast, thyroid, and endometrial cancers, macrocephaly, and various skin tumors.
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