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SEER defines the apex as being the bottom-most portion of the gland. Apex means "narrowest part," which in the prostate would be the bottom-most portion of the gland.

For cases diagnosed 1998-2003:

When the prostate feels benign and the cancer is found incidentally at the time of the microscopic exam, code the EOD-Extension field to 10 [number of foci or % of involved tissue not specified]. Code as 13 (less than or equal to 5%) or 14 (greater than 5%) if percentage involved is given in the tissue resected. If the path report states "solitary focus of carcinoma" without mentioning the total amount of tissue resected, code extension to 13. If there is more than one focus, code extension to 10. Don't assign a code of 20 unless the tumor is clinically apparent.

Record the radiation as first course of treatment even though it was delayed for several months.

Radiation is highly effective when there is a small or microscopic amount of tissue left at the margin following prostatectomy. In most regions, radiation therapy is the standard of care for positive margins at prostatectomy.

Code the date of the biopsy as the date of diagnosis. This is the date the cancer was first identified by a medical practitioner.

Note: SEER collects the Month and Year of diagnosis. The "day" of diagnosis is not collected by SEER.

Apply 2007 MP/H rule H6 and assign the numerically higher ICD-O-3 code that reflects PNET (9364/3).

According to the WHO Tumors of Soft Tissue and Bone, though Ewing sarcoma ICD-O-3 code is 9260/3, Ewing sarcoma with a higher degree of neuroectodermal differentiation present is classically termed peripheral neuroectodermal tumors (PNET).  WHO does not offer guidance how to classify tumors stated to be Ewing sarcoma PNET.

Histology code 9364/3 is assigned for a Ewing/PNET that arises outside of the brain/CNS. Peripheral neuroectodermal tumor (PNET) and peripheral primitive neuroectodermal tumor (PPNET) are Ewing family tumors.

Histology code 9473/3 (PNET, primitive neuroectodermal tumor, central primitive neuroectodermal tumor, or supratentorial PNET) is only used for tumors arising inside the brain/CNS.

this is a single primary per Rule M1. According to our expert pathologist, "if multiple solid tissue tumors are present (sarcomas), then almost always there is one primary and the rest are metastases. There are infrequent occasions of multifocal liposarcoma or osteosarcoma occurring, but the patient would be treated as a patient with metastatic disease."

The steps used to arrive at this answer are:

Open the Multiple Primary and Histology Coding Rules manual. For a soft tissue primary, use one of the three formats (i.e., flowchart, matrix or text) under the Other Sites MP rules to determine the number of primaries because soft tissue primaries do not have site specific rules.

Go to the UNKNOWN IF SINGLE OR MULTIPLE TUMORS module, Rule M1.

Rule M1 states, "It is not possible to determine if there is a single tumor or multiple tumors, opt for a single tumor and abstract a single primary." Given the information from the expert pathologist, this case should be reported as a single primary applying this rule.

NTRK-rearranged spindle cell neoplasm is a newly identified variant of sarcoma; however, WHO has not yet proposed a specific ICD-O code for this rare neoplasm. Code to spindle cell sarcoma (8801/3).

WHO defines NTRK-rearranged spindle cell neoplasm as an emerging group of molecularly defined rare soft tissue tumors that span a wide group of morphologies and histological grades, and are most often characterized by a spindle cell phenotype among other characteristics.

Code the Primary Site field as follows:

1. C44.9 [Skin, NOS] as the default value when lesions develop simultaneously in skin and non-skin areas.

2. C07.9 [Parotid gland]

3. C44.9 [Skin, NOS] as the default value when there is no mention of lesions in the skin or other primary site.

Edward Klatt states in Practical AIDS Pathology, "...Visceral Kaposi (involving one or more internal organ sites) is also present in three-fourths of cases, but may not be diagnosed prior to autopsy. Visceral involvement frequently includes the lung, lymph nodes and gastro-intestinal tract."

For tumors diagnosed prior to 2007:

Code histology as 9260/3 [Ewing sarcoma]. ICD-O-3 does not have a code for extra-osseous Ewing sarcoma (EOE). Ignore the topography code listed in ICD-O and use the code for the primary site (vulva).

Site codes associated with morphology codes in the ICD-O are the most common sites and are not intended to limit coding only to those sites.

For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.