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Assign 8523/3.

The 2013 revision to ICD-O-3 has a new code for mixed acinar ductal carcinoma; however, this new code will not be implemented in the U.S. until 2018 or later. Page 7 of the Guidelines for ICD-O-3 Update Implementation document released by NAACCR 1/1/2014 instructs us to use 8523/3 in the meantime.

Clark's level can be used to assign in situ, localized or regional summary stage.

If there is a discrepancy between the Clark’s level and the pathologic description of extent, use the higher Summary Stage code.

The primary site is unclear. The lesion is intracranial, but this may not be the primary site. In the absence of any additional information, assign C390, 8020/3. According to WHO, sinonasal undifferentiated carcinoma can involve the nasal cavity, maxillary antrum, and/or ethmoid sinus.

SMARCB1 (INI-1) is a tumor-suppressor gene located on chromosome 22q11.2. Tumors that showed loss of expression were SMARCB1-deficient tumors which are characterized by nests, sheets, and cords of cells without any histologic evidence of specific (eg, squamous or glandular) differentiation.

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.                            

Code the histology to 8574/3 for Ductal carcinoma with neuroendocrine features.

Ductal carcinoma is also called "invasive breast carcinoma of no special type." WHO classifies Invasive breast carcinoma with neuroendocrine differentiation as 8574/3.

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.

Apply the Multiple Primaries/Histology, Breast Rule H3: DCIS and a more specific in situ are coded to the more specific histology term which in this case is solid. Code the histology to ductal carcinoma in situ, solid type (8230/2). Based on the information provided, there is no invasive component. The term "microca ++" means micro-calcifications are present, not micro carcinoma.

Code high grade urothelial carcinoma described as having "extensive sarcomatoid dedifferentiation" to sarcomatoid transitional cell carcinoma (8122/3).

Abstract a single primary. Based on the information provided, this is a single tumor, and therefore a single primary, Rule M3. Code the histology to malignant phyllodes tumor.

According to our expert pathologist consultant, "The presence of a phyllodes tumor component identifies the whole thing as such. Stromal overgrowth/sarcoma is the usual identifier of malignancy in a phyllodes tumor. (If there were no phyllodes component we would be left with undifferentiated sarcoma, but that is not the case here. The diagnosis of malignancy in phyllodes tumor may be difficult/problematic when there is no overt stromal/sarcoma overgrowth as in this case.) As an aside, the behaviors of pure sarcoma and a phyllodes tumor such as we have here are similar, but we would lose the primary diagnosis if we just called this sarcoma."

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.