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.
Histology--Brain and CNS: How is histology to be coded for a pituicytoma WHO grade I, of the pituitary?
Assign code 9380/1 [glioma, borderline].
According to our pathologist consultant, the term pituicytoma is restricted to low-grade glial neoplasms of the neurohypophysis or infundibulum. The best category currently available for these is glioma.
First course treatment: Is subsequent treatment with R-ICE first course or second course therapy if the patient underwent ABVD x2 cycles and subsequent imaging showed no response to treatment and evidence of progression [new adenopathy] for a lymphoma case? See Discussion.
Patient was initially diagnosed with Hodgkin Lymphoma, Nodular Sclerosing on 3/3/06.
Patient received ABVD x 2 cycles. Had disease reassessed in May, 2006, no response to treatment, showed evidence of progression (new adenopathy). Patient's pathology from 3/06 was sent for consult: Diagnosis was Hodgkin with some overlapping features of B-cell Non Hodgkin Lymphoma. Treated 5/18/06 with R-ICE FOR NHL.
The R-ICE treatment in this case is not part of the first course. Documentation of treatment failure and/or disease progression signifies the end of the first course of treatment.
Histology--Pancreas: What is the correct code for "non-secretory pancreatic endocrine tumor" with positive lymph nodes on excision indicating a malignant tumor? Pathologist indicated it was not an exocrine tumor.
Code as islet cell carcinoma [8150/3].
There are several cell types in the islets, and each produces a different hormone. The custom has been to name the tumors by their hormone production e.g. insulinoma, glucagonoma, etc. Occasional tumors do not produce any hormone (at least one that can be determined or measured). These tumors are called non-functioning endocrine tumors. Most of the endocrine tumors in the pancreas are islet cell tumors.
CS Extension--Brain and CNS: How is this field coded for a malignant brain tumor that presents as a lesion with significant pressure on the left frontal ventricle and dilation of the right ventricles? See Discussion.
CS Extension code 30 includes tumor that invades or encroaches upon the ventricular system. Does significant pressure mean the same thing as encroach?
This answer was provided in the context of CSv1 coding guidelines. The response may not be used after your registry database has been converted to CSv2.Do not assign extension code 30 in this case unless there is evidence elsewhere of ventricular system involvement.
"Significant pressure" is not synonymous with encroachment or involvement. See the list of ambiguous terms for CS staging on page 121 of the 2007 SEER manual.
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--Breast: Patient has 2 existing primaries, both of left breast and both were pure lobular carcinoma, one was diagnosed in 1994 and the other in 2005. Now a biopsy in 2008 of a supraclavicular lymph node (laterality unknown) and subcutaneous scalp tissue show metastatic DUCTAL carcinoma. Per path report, breast is the primary site. Slides from prior tumors were not reviewed. Should this be made a new primary or assumed to be metastasis from the prior breast tumors? See Discussion.
A modified radical mastectomy was performed on 10/6/94.
The 2007 MP/H rules tell us that multiple ductal and lobular tumors of breast are a single primary; however, the rules do not apply to metastatic tumors.
For cases diagnosed 2007 or later:
Abstract the 2008 diagnosis as a new primary.
Since the primary site is unproven but stated to be breast, and since the laterality is unknown, we cannot determine that the 2008 diagnosis is the same as the 2005 or the 1994 diagnosis.
Revise this case accordingly if more information becomes available.
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.
MP/H Rules--Breast: Is a 2008 invasive ductal carcinoma counted as a new primary when it follows a 2005 invasive lobular carcinoma diagnosed in the same breast? See Discussion.
The patient has invasive lobular breast carcinoma excised in 2005. She returns in 2008 with a new invasive ductal carcinoma tumor same breast. Following MP/H rules, M10 seems to apply, which states this is still a single primary. Does this mean that this invasive ductal carcinoma is ignored and the patient remains in the registry with only a lobular carcinoma primary?
For cases diagnosed 2007 or later:
Rule M10 applies. The 2008 diagnosis is not a new primary.
The abstract for the 2005 diagnosis should be annotated to include the new information.
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