Primary Site/Sarcoma--Breast: How should the primary site and stage be coded for osteosarcoma of breast? Is C509 correct or should the code be a different primary site? When assigning C509, the Collaborative Stage (CS) still pertains to breast cancer and AJCC stages it as a breast cancer and not as a sarcoma.
Code primary osteosarcoma of the breast to breast, C500-C509. Not all site and histology combinations can be staged in CS or AJCC. 9180/3 of breast cannot be staged using the CS breast schema. Breast (C500-C509) cannot be staged using the CS soft tissue schema. The same is true for AJCC. You can stage this case using SEER Summary Stage.
Important: Do NOT change the primary site or histology code based on whether or not the case can be CS or AJCC staged. We need to know how many cases are unable to be staged because of their primary site and histology combinations.
MP/H Rules/Histology--Kidney: How is the histology coded and what rule(s) apply to the classification of succinate dehydrogenase-deficient renal cell carcinoma? See Discussion.
Partial nephrectomy showed carcinoma, histologic type: succinate dehydrogenase-deficient renal cell carcinoma. This is not a term in the ICD-O, and is not a histology covered in the Kidney MPH rules. However, a recent web search indicates this is a specific type of RCC that was added to the 2016 WHO classification of RCC (per abstract: https://www.ncbi.nlm.nih.gov/pubmed/27179267) and makes up 0.05-0.2% of RCC cases (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4229399/).
Code the histology to renal cell carcinoma, NOS (8312/3). While WHO lists succinate dehydrogenase-deficient renal cell carcinoma in the latest edition, no specific histology code is provided. MP/H Rule H10 applies since only one histology type is provided, though no code is listed.
Grade/Neuroblastoma: What grade is to be used when pathology states only differentiating retroperitoneal neuroblastoma?
For cases diagnosed prior to 2018
Assign grade code 2 for "differentiating" retroperitoneal neuroblastoma. The rationale of our expert pathologist advisor is that "it leaves the grade 1 category open (since a "well differentiated neuroblastoma" is actually called ganglioneuroblastoma), and it also avoids putting "differentiating" into what is usually a well differentiated category."
Additionally, assign grade code 3 to a poorly differentiated retroperitoneal neuroblastoma and grade code 4 to an undifferentiated retroperitoneal neuroblastoma.
For cases diagnosed 2018 and later
Follow the instructions for coding grade in SEER*RSA
MP/H Rules/Histology--Bladder: Is urothelial carcinoma, high-grade, predominantly solid type, coded as 8120/3 or 8230/3? See Discussion.
Urinary bladder: Invasive urothelial carcinoma, high-grade, 4.5cm, predominantly solid type, arising in background of carcinoma in-situ, carcinoma grossly extends into perivesical adipose tissue; lymph-vascular invasion is seen.
Assign histology code 8120/3, urothelial carcinoma, NOS. Solid type is not a recognized variant of urothelial tumors and likely represents the appearance of the urothelial cells within the tumor and not a specific histologic type.
Reportability--Heme & Lymphoid Neoplasms: Is a diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) with large cell transformation equivalent to a diagnosis of diffuse large B-cell lymphoma (DLBCL) without mention of Richter transformation or Richter Syndrome? See Discussion.
The patient has a history of CLL/SLL dating back to 2007, but has had progressive disease with development of a new left frontal brain tumor. The brain tumor resection proved CLL/SLL with large cell transformation, but neither the pathologist nor the managing physician called this a Richter transformation, Richter syndrome or provided a diagnosis of DLBCL. However, a large cell transformation of CLL/SLL is a Richter transformation. Can this be accessioned as a new acute neoplasm per Rule M10?
Accession as multiple primaries according to Hematopoietic and Lymphoid Neoplasm Coding Manual Rule M10. Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) followed by CLL/SLL with large cell transformation is multiple primaries because it is a chronic neoplasm followed by an acute neoplasm, more than 21 days in this case.
MP/H Rules/Histology--Brain and CNS: What is the code for an embryonal tumor with multilayered rosettes. WHO shows the code as 9478/3, but this code is not available for use in the United States.
Assign ICD-O-3 code 9392/3 until code 9478/3 is implemented in 2018. Per our expert neuropathologist, embryonal tumor with multilayered rosettes was previously called ependymoblastoma.
Race, ethnicity: How do you code race for someone from New Zealand?
I recently did a presentation on coding the data item Race. In my presentation I discussed understanding geography help code race in some circumstances. One of the slides demonstrates how large Polynesia is and what Pacific islands are found in Polynesia, such as, Tahiti, Samoa, and even Hawaii, all of which have their own codes. Someone in the audience asked "How do you code New Zealand? Upon some research, New Zealand is not listed in Appendix D of the SEER coding manual. We could code them 01-White. But research shows there is a very large indigenous population. Technically, New Zealand is located within the boundaries of Polynesia - Code 25 (Polynesian).
If the only information you have on race is that the person is from New Zealand, code race as white. This is based on the instructions for Australia, the closest neighbor to New Zealand as no other guidance was found.
Reportability/Behavior--Ovary: Is adult granulosa cell tumor a reportable malignant tumor if the primary ovarian tumor ruptured intraoperatively, the peritoneum was contaminated, and the patient underwent adjuvant treatment with chemotherapy given the increased risk of recurrence due to intraoperative tumor spill? See Discussion.
Per SINQ 20130176 and 20140034, adult granulosa cell tumors of the ovary are reportable malignant tumors when there are peritoneal implants or metastases. The SINQ responses describe how these adult granulosa cell tumors are different from low malignant potential (LMP) epithelial ovarian tumors. Would these SINQ scenarios apply to a case with intraoperative tumor rupture that resulted in peritoneal tumor?
In this case, the pathologist indicated these excised peritoneal specimens were favored to be intraoperative contamination with adult granulosa cell tumor. However, the oncologist went on to treat this patient as high risk with chemotherapy. The oncologist only described one of the pelvic peritoneal implants as possibly contamination due to the rupture. The oncologist never indicated the tumors were definitely peritoneal implants. Should the behavior of this tumor be /1 because the peritoneal tumor appears to be contamination, or /3 because the oncologist treated this patient as high risk?
If the "implants" were due to intraoperative contamination and were not present prior to surgery, do not interpret them as indicative of malignancy. The behavior of this tumor is /1.