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Malignant granulosa cell tumor is reportable. The case described above is malignant as proven by metastasis to the lymph node.

The histologies stated for this case are Ewing sarcoma (9260) and PNET, primitive neuroectodermal tumor,(9364)*. Use the Other Site Rules, starting with H8. Stop at H17 and assign the higher histology code -- 9364/3 [Peripheral neuroectodermal tumor].

*The term "PNET" is used for two different tumors. One is primitive neuroectodermal tumor (9473) and pertains to brain tumors per ICD-O-3 review. The other is peripheral primitive neuroectodermal tumor (pNET or PPNET 9364) and pertains to bone or soft tissue tumors. This case is stated to be soft tissue and Ewing sarcoma, so it is 9364 rather than 9473.

For tumors diagnosed prior to 2007:

Follow the rules in the 2004 manual for determining multiple primaries. When the sebaceous carcinomas are in different sites (topography code difference in the first two numeric digits after the C), they are separate primaries. When the sebaceous carcinomas are more than two months apart, they are separate primaries.

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.

For cases diagnosed 1998-2003: If 7mm is the measurement of the infiltrating duct portion of this cancer, assign extension code 13 [Invasive and in situ components present, size of invasive component stated and coded in Tumor Size].

If 7mm is the size of the whole malignancy and the size of the invasive portion cannot be determined, assign extension code 14 [Invasive and in situ components present, size of entire tumor coded in Tumor Size (size of invasive component not stated) and in situ described as minimal (less than 25%)]. "Focal areas of in situ carcinoma" qualifies as minimal.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Abstract the diffuse large B-cell lymphoma (Richter transformation) as a second primary per Rule M10. Rule M10 states to abstract as multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm (CLL) AND there is a second diagnosis of an acute neoplasm (the diffuse large B-cell lymphoma (Richter transformation)) more than 21 days after the chronic diagnosis.

"Richter transformation," also known as "Richter syndrome," is a term that indicates CLL has transformed to DLBCL. Richter syndrome is listed under the Alternate Names section in the Heme DB for DLBCL (9680/3).

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Per PH27, code the primary site to C809 [unknown]. Rule PH27 states one is to code the primary site to unknown [C809] when there is no evidence of lymphoma in lymph nodes AND the physician documents in the medical record that he/she suspects that the lymphoma originates in an organ(s) OR there is multiple organ involvement without any nodal involvement.

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

For cases diagnosed 1998-2002:

Code the Reason for No Cancer-Directed Surgery field to 1 [Cancer-directed surgery was not recommended] for patients who present with either a primary site or histology for which surgery is not a standard treatment. Also use code 1 for those patients who present with distant disease for a primary site that is typically treated surgically. Patients with distant metastasis typically do not have surgery performed as part of first course of treatment.

Code 2 [Contraindicated due to other conditions] is used when surgery would normally be recommended for the site (given the current stage of the tumor) but other medical conditions pose too much of a risk for the patient to undergo surgery.

For cases diagnosed 2007 or later, code the histology as 8213/2 [carcinoma in situ in a serrated adenoma].

The steps used to arrive at this decision are:

: Apply ICD-O-3 rule F (Matrix principle) and assign the behavior code /2 when the behavior assigned by the pathologist differs from the usual behavior as given in the ICD-O-3.

: Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text) and go to the Colon Histology rules.

: Start at the SINGLE TUMOR module, Rule H1. The rules are intended to be reviewed in consecutive order within a module. Stop at rule H4. Code the histology as 8213/2.

Note: The histology 8213 (adenocarcinoma in serrated adenoma) will be added to rule H4 in the next revision.

For tumors diagnosed 2001-2006:

This ovarian tumor is not SEER reportable if diagnosed between 2001-2006. The histology and behavior codes are 8442/1 [serous cystadenoma, borderline malignancy]. Sequence is coded appropriately from 60-88 [non-malignant tumor or central registry-defined neoplasm].

The behavior code could be changed to /3 only when the pathologist states that the disease is malignant. Approximately 20% of serous tumors of low malignant potential have lymph node involvement, according to the WHO Classification of Ovarian Tumours. In ovarian serous tumors of low malignant potential, lymph node involvement is not always equivalent to metastasis and does not signify malignancy in these tumors unless definitely stated as such by the pathologist.

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.

For tumors diagnosed prior to 2007:

Code histology to 8310/3 [Clear cell adenocarcinoma, NOS]. This is consistent with the WHO Classification of Tumours and reflects the current practice of placing less emphasis on "cyst-" prefix for ovarian malignancies.

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.