MP/H Rules: Does the presence of metastases affect the application of the MP/H rules? See Discussion.
Single lung tumors presenting in each lung but the patient also presents with bone mets? Would rule M6 apply? Or do the bone mets represent additional tumors?
For cases diagnosed 2007 or later, the MP/H rules do not apply to metastases. Ignore metastases when applying the rules.
For the case above, use rule M6 and abstract as two primaries (right lung and left lung). The bone mets are ignored.
MP/H Rules--Breast: How many primaries should be abstracted when a patient has a mass at 6:00 that showed poorly differentiated ductal carcinoma and a hypoechoic nodule at 9:00 that was excised with no real tumor present there though path showed angiolymphatic invasion by carcinoma throughout the entire specimen? See Discussion.
Palpable mass in right breast at 6:00. Path stated 'poorly differentiated ductal carcinoma with extensive necrosis and extensive angiolymphatic invasion. Focal high grade comedocarcinoma (1%)'. Another hypoechoic nodule was seen at the 9:00 position. This mass was excised from surrounding tissue. This mass was more like an inflammatory mass; there was no real tumor present there. Path report stated "Breast mass 9:00 excisional biopsy - angiolymphatic invasion by mammary carcinoma throughout the entire specimen."
Is this two primaries because of the two different histology codes: 8500 and 8010?
For cases diagnosed 2007 or later, abstract as a single primary using rule M3 (a single tumor is always a single primary). There was one tumor present according to the information provided. The second specimen was not a separate tumor ("There was no real tumor present there").
MP/H Rules--Lung: Per rule M8, tumors of the same site (left lung), same histology (NSCC), greater than 3 yrs apart are separate primaries.
However, there was a recurrence to mediastinal LNs after 2 years. Would that make a difference as to whether the 2008 left lung carcinoma is reportable as a new primary or not? See Discussion.
Scenario: NSCC 2004 LLL with positive hilar/mediastinal LNs treated with LLL lobectomy, chemo and rad. 2006 per CT/PET recurrence in mediastinal LNs treated with chemoradiation. 2008 left lung nodule positive for NSCC stated by MD to be recurrence from 2004 (2008 path not compared to 2004 path).
For cases diagnosed 2007 or later:
The 2008 lung carcinoma is a separate primary according to rule M8. The 2006 diagnosis is metastases to the lymph nodes. Do not apply the MP/H rules to metastases.
MP/H Rules--Breast: Is inflammatory breast cancer always one primary per lifetime? Or is a subsequent inflammatory breast cancer a second primary if diagnosed more than five years later?
For cases diagnosed 2007 or later, a diagnosis of inflammatory breast cancer more than five years after a previous diagnosis of inflammatory breast cancer is a separate (new) primary. See rule M5 in the Breast Multiple Primary Rules.
Primary site: What is the correct primary site code for angiosarcoma of the spleen with mets to bone marrow C42.2 vs C49x? See Discussion.
Robbins Pathology states the following about liver angiosarcomas: Hepatic angiosarcomas are rare but of interest because they are associated with distinct carcinogens, including arsenic (exposure to arsenical pesticides), Thorocast (a radioactive contrast medium previously widely used in radiology), and polyvinyl chloride (PVC) (widely used in plastics). The increased frequency of angiosarcomas among works in the PVC industry is one of the truly well-documented instances of chemical carcinogenesis in humans. With all these agents, there is a very long latent period of many years between exposure and the development of tumors.
Could the same apply to the spleen?
Code C422 [Spleen] as the primary site for angiosarcoma of spleen with metastasis to bone marrow.
MP/H Rules--Brain and CNS: Are stigmata of neurofibromatosis in the brain reportable neurofibromatosis lesions? See Discussion.
Reference: SINQ 20051108; SINQ 20061018 Three year old patient with history of neurofibromatosis 1. 3/05 MRI of the brain showed right optic nerve glioma. It also showed heterogeneous high t2 signal in the middle cerebellar peduncles and near the genu of the internal capsules bilaterally are stigmata of neurofibromatosis type I. 3/08 MRI showed new mass suspicious for glioma in the hypothalamus. Clinical diagnosis is benign glioma secondary to diagnosis of neurofibromatosis. How many primaries are to be accessioned for this patient? Should the matrix principle be invoked for the second glioma? Should the behavior code for the glioma be 0?
For cases diagnosed 2007 through 2017
Accession NF (9540/1) when there is CNS tumor -- a glioma or some other intracranial/intraspinal tumor. Stigmata of NF are reportable when the stigmata themselves are reportable tumors. For example, glioma, or another intracranial/intraspinal tumor. Do not report sitgmata that are only termed "stigmata seen on MRI," for example, without other reportable terminology.
Do NOT accession NF (9540/1) when there is only peripheral nerve/nervous system involvement.
Accession the neurofibromatosis itself only once per patient. Accession any initial neoplasm in the CNS separately. Abstract and code any subsequent CNS neoplasms according to the multiple primary brain rules.
Accession three primaries for the case described above.
Neurofibromatosis (C729 9540/1)
Optic nerve glioma (C723 9421/3)--> see below.
Hypothalamus glioma (C710 9380/0)
--> Optic nerve gliomas associated with NF are pilocytic astrocytomas. Code pilocytic astrocytoma as 9421/3 in North America.
For cases diagnosed 2018 or later
See the 2018 Solid Tumor Rules for Non-Malignant CNS tumors.
Surgery of Primary Site--Melanoma: Which surgery codes should be used for cases that have a 1 cm margin? See Discussion.
For a melanoma case the surgery codes in the 30's are to be used when margins are stated to be less than 1 cm. The codes in the 40's are to be used for cases where the margins are greater than 1 cm.
If the margin is exactly 1 cm, assign a surgery code from the 20-36 range. Use a code in the 40's only when the margin is greater than 1 cm.
MP/H Rules--Corpus uteri: How is histology coded for an endometrial tumor described as an "endometrioid adenocarcinoma with prominent squamous metaplasia"?
For cases diagnosed 2007 or later:
Endometrioid adenocarcinoma with squamous metaplasia is coded 8570 [Adenocarcinoma with squamous metaplasia]. This falls under the Histology Coding Rules for Other Sites, rule H17. The code for Endometroid adenocarcinoma is 8380. The code for Adenocarcinoma with squamous metaplasia is 8570. The histology with the numerically higher ICD-O-3 code is Adenocarcinoma with squamous metaplasia -- 8570.
Histology/Behavior--Brain and CNS: How are these fields coded for an "anaplastic glioneuronal neoplasm with spongioblastic architecture"? See Discussion.
Scenario: Addendum from Mayo Clinic review, IHC and consultation made dx of "anaplastic glioneuronal neoplasm with spongioblastic architecture". The original micro states 'high grade glial neoplasm w/o characteristic features of glioblastoma multiforme in that it lacks areas of significant necrosis, no nuclear palisading nor endothelial vascular proliferation...."
The best code available according to our pathologist consultant is 9505/3 [Ganglioglioma, anaplastic]. According to our consultant, while ganglioglioma is traditionally a benign tumor, anaplastic ganglioglioma is classified as malignant by WHO (page 103), and comes as close to fitting the description of this tumor as any other term.
Laterality--Brain and CNS: When a meningioma extends to both right and left sides, is laterality coded 4 for bilateral or 9 for midline? See Discussion.
Operative Findings: Bilateral frontal craniotomies for excision of giant meningioma which extended onto optic chiasm.
Path: Bifrontal tumor, 6.5 cm meningotheliomatous meningioma.
If it is not possible to determine whether the meningioma originated on the left or the right, assign code 4 [Bilateral involvement, lateral origin unknown; stated to be single primary].
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