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Do not record WHO/ISUP grade in the grade/differentiation field.

Designated fields for this grade system are being proposed for future implementation.

Lymphangioma of the brain or CNS is not reportable. Lymphangioma is a malformation of the lymphatic system. Even though it has an ICD-O-3 code, do not report it.

Hematopoietic neoplasms that transform generally don't have 'chronic' or 'acute' as part of their preferred name. The 'chronic' and 'acute' designations are determined by the usual course of the neoplasm. Chronic neoplasms are generally slow growing while acute neoplasms grow fast and are more widespread. Not all Hematopoietic neoplasms transform. Each neoplasm that has the ability to transform has the transformations listed under the 'Transformations to' and/or 'Transformation from' sections in the Hematopoietic database.

For example, Diffuse large B-cell lymphoma (histology code 9680/3) has no histologies/neoplasms listed under 'transformations to.' This means that this neoplasm does not transform to any other neoplasm. There are multiple histologies/neoplasms listed under 'Transformations from' indicating the neoplasms listed under the Transformations from are the chronic neoplasms, and DLBCL is the acute neoplasm. If DLBCL (9680/3) occurs at the same time, within 21 days, or greater than 21 days of any of the histologies listed under 'Transformations From,' rules M8-M13 apply. If DLBCL (9680/3) occurred at the same time as a neoplasm not listed in the Transformations sections, the acute and chronic rules do not apply.

Code to metaplastic carcinoma, 8575/3. According to our expert pathologist consultant, "The term 'metaplastic stroma' implies that at least a portion of the carcinoma has undergone a 'metaplastic' change from epithelial in appearance to 'stromal' in appearance. I assume this is what CAP means by 'Invasive mammary carcinoma with matrix production,' which the WHO equates to metaplastic carcinoma."

You may code grade as follows.

Grade 1 – NET G1 (M8240/3)

Grade 2 – NET G2 (M8249/3)

Grade 1 – neuroendocrine carcinoma, low grade (M8240/3) or neuroendocrine carcinoma, well differentiation (M8240/3)

Grade 2 – neuroendocrine carcinoma, moderate differentiation (M8249/3)

According to our expert pathologist consultant, yes, seminoma could transform to a mixed germ cell tumor with a somatic type malignancy. He advises us to code this case as 9061/3.

From our expert pathologist consultant: This occurs as "reprogramming" of the initial germ cell tumor/seminoma cell. The process is not understood, but genetic studies support this progression concept. Most often the next step is teratoma. It is out of the teratoma that the somatic malignancy usually comes. I do wonder about the possibility that this was really an embryonal carcinoma which resembles a seminoma - occasionally this can be a difficult separation. I wonder if they radiated the scrotum following the orchiectomy, also, given the scrotal recurrence.

Conjuctival intraepithelial neoplasia grade III (CIN III) is reportable. Intraepithelial neoplasia, grade III, is listed in ICD-O-3 as /2. It is reportable for sites other than skin.

Mammary fibromatosis is not reportable. The WHO classification for breast tumors assigns mammary fibromatosis a behavior code of /1. According to WHO, mammary fibromatosis "is a locally infiltrative lesion without metastatic potential…"

Assign 9085/3. Code this combination of teratoma, yolk sac tumor, and embryonal tumor in the testis to mixed germ cell tumor (9085/3) based on the WHO Classification of Tumors of the Male Genital Organs.                            

Assign 9505/1 for Rosette-forming glioneuronal tumor. The new code, 9509/1, has not been implemented in the United States. 9505/1 is to be used until the new code is implemented. See page 7 of the NAACCR Guidelines for ICD-O-D Implementation, effective January 1, 2014, http://www.naaccr.org/LinkClick.aspx?fileticket=u7d3sB71t5w%3d&tabid=126&mid=466.