Multiple Primaries--Brain and CNS: How many primaries should be recorded in a patient with von Hippel Lindau disease that has a hemangioblastoma of the cerebellum in 2003 and a hemangioblastoma of the brainstem in 2007?
A tumor of the cerebellum (C716) and a tumor of the brainstem (C717) are multiple primaries because the topography codes are different at the fourth character of site.
MP/H Rules/Histology--Sarcoma: What would be the morphology code for a low grade myofibroblastic sarcoma of the left distal forearm? I tried several different combinations but the closest I could come up with is myosarcoma.
Assign code 8825/3. Apply the ICD-O-3 Matrix Concept, Rule F, page 29 of the hardcover ICD-O-3. The WHO Classification of Soft tissue and Bone, page 85, lists low grade myofibroblastic sarcoma, also called myofibrosarcoma, 8825/3.
MP/H/Histology--Breast: What MP/H Rule, histology, and behavior code for a breast primary apply to the following?
2 foci DCIS, solid, high grade (Grade 3) w/microca++
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.
MP/H Rules/Histology: What is the correct histology code for a NUT midline carcinoma?
Code histology to 8010/3.
NUT carcinoma is identified by the NUTM1 gene rearrangement.
NUT midline carcinomas (NMC) are lethal and morphologically indistinguishable from other poorly diff carcinomas. They are epithelial tumors which can range from undifferentiated carcinomas to carcinomas with prominent squamous differentiation.
A new proposed ICD-O-3 code has been suggested for NUT tumors but it is not yet approved for implementation. Do not use the new code until it is approved for use in the United States.
Reportability--Brain: Is benign lymphangioma of the brain (9170/0) reportable? It is not on the list of non-malignant blood vessel tumors in the National Program of Cancer Registries Clarifications for Central Nervous System (CNS) tumors.
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.
Scope of Regional Lymph Node Surgery/EOD Fields: When a patient has two simultaneously diagnosed primaries, and a regional lymph node dissection intended for one of the primaries removes nodes that are also regional for the other primary, is the information from the lymph node dissection coded for both primaries?
For cases diagnosed 1998-2003:
If the lymph nodes are negative, the status of nodes that are regional for both sites would be used to code the EOD and Site-Specific Surgery fields for both sites.
If any of the lymph nodes are positive use the histology from the lymph nodes to determine how the EOD and Site-Specific Surgery will be coded. For example: If prostate cancer is an incidental finding when a cystoprostatectomy and pelvic lymph node dissection are done to treat a bladder cancer, and all of the positive lymph nodes reflect the histology of the prostate primary (adenocarcinoma), code the nodes as positive for the prostate primary and negative for the bladder primary.
Reportability/Histology--Pituitary Gland: How are Rathke cleft cyst and Rathke pouch tumor distinguished and are they both reportable?
Rathke cleft cyst is not reportable. Cysts are not neoplastic. However, Rathke pouch tumor (C751, 9350/1) is a reportable neoplasm for cases diagnosed 2004 and later. The Rathke pouch is coded to the pituitary gland. Benign and borderline pituitary tumors have been reportable since 2004.
First course treatment/Chemotherapy: Is metronomic chemotherapy coded as chemotherapy?
Code metronomic chemotherapy as chemotherapy. Metronomic chemotherapy, also referred to as low-dose metronomic (LDM) chemotherapy, is an emerging cancer treatment approach which administers relatively low doses of traditional chemotherapy drugs over a long period of time and without ‘breaks’ in treatment. By using lower doses this method of treatment minimizes the side effects of traditional chemotherapy.
Grade/Sarcoma--Breast: Is the correct grade for high grade angiosarcoma of the breast a code 3 or 4? The breast usually uses a three grade system but sarcoma is not a typical histologic type of the breast.
Assign grade code 4 using the sarcoma table. Nottingham or Bloom-Richardson (BR) Score/Grade does not apply to angiosarcomas. This is a good question and points out needed clarification of the grade rules.
MP/H Rules/Histology--Prostate: What is the histology code for a prostate case whose histology reads “adenoca with mixed ductal and acinar variants?
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.
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