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Patient has a resection labeled as: Spine, designated intramedullary lesion. The Final Diagnosis is: Melanocytic neoplasm with features most consistent with primary intramedullary melanocytoma. The Diagnosis Comment states: The overall immunophenotypic and morphologic impression is a primary central nervous system melanocytoma.

The ICD-O-3 lists melanocytoma, NOS histology code as 8726/0, but does not provide a site-associated code. If the ICD-O-3 is used, the histology would be 8726/0 and the primary site presumably would be C720 since the tumor was specifically described as being intramedullary (i.e., within the spinal cord medulla).

Table 6 (Solid Tumor Rules, Non-Malignant CNS Equivalent Terms and Definitions) does not list either an intramedullary melanocytoma or melanocytoma (NOS). However, Table 6 does include meningeal melanocytosis 8728/0 and meningeal melanocytoma 8728/1. If Table 6 is used and the histology is coded 8728/1, then the primary site would presumably be C701 per the ICD-O-3 site-associated listing for this histology (C709).

We think this should be a single primary because the Solid Tumor rules do not apply to metastases. However, we are not sure whether or not the instructions outlined for prostate (SINQ 20180088, 20130221), that indicate we are to accession a new metastatic tumor only with a small cell neuroendocrine histology after an adenocarcinoma, also applies to lung primaries.

We are aware of a phenomenon in which lung adenocarcinoma cases treated with Erlotinib can transform to small cell, but do not know whether it impacts the number of reportable primaries.

The Hematopoietic and Lymphoid Neoplasms Database (Heme DB) definition for systemic mastocytosis with an associated hematological neoplasm (SM-AHN, 9741/3) states SM-AHN is a variant of systemic mastocytosis that arises with a myeloid disease of non-mast cell lineage (e.g., MDS, MPN, etc.) and that,

However, SINQ 20130172 conflicts with the Heme DB stating the systemic mastocytosis and the associated hematological neoplasm are a single primary coded to a single histology (9741/3) per Rule M2.

The patient was clinically diagnosed with a pituitary adenoma on MRI in June 2009. The MRI noted an unusual contour involving the superior margin of the pituitary gland and the clinical interpretation was a small pituitary adenoma. The patient did not follow-up with the recommended repeat imaging and never received treatment for the pituitary adenoma.

The patient was eventually seen again in January 2020 and the MRI showed no adenoma in the pituitary gland. Since pituitary adenomas are known to spontaneously regress, should the 2009 diagnosis of pituitary adenoma be accessioned as a SEER reportable benign central nervous system (CNS) tumor?

Per Mayo consulting pathologist, the final diagnosis on this right lung biopsy is: SMARCA4-deficient malignant neoplasm (see Comment). Comment: Sections show a poorly-differentiated malignant neoplasm without any apparent glandular, squamous, or stromal differentiation. The tumor near totally replaces the underlying lung tissue without recognizable underlying alveolar parenchyma. Immunohistochemical stains performed at Mayo Clinic (Oscar keratin, INSM1, NUT, S100, desmin and BRG1 protein encoded by SMARCA4 gene) demonstrate that the malignant cells are positive for Oscar keratin (rare cells only), synaptophysin (weak/patchy) and p63 (focal) while negative for the remaining antibodies tested. Of note, SMARCA4 stain is negative in the tumor cells. Thus, this tumor can be categorized as a SMARCA4-deficient malignant neoplasm, which is known to be an aggressive malignancy, likely represent a SMARCA4-deficient thoracic sarcoma, a recently described entity. SMARCA4-deficient carcinomas in the lung have been reported to be mostly adenocarcinomas or squamous cell carcinomas, which would not fit for this case. Please refer to a paper published by our group (Sauter JL et al. Mod Pathol 2017;30:1422-32.

Per the 2018 Solid Tumor Rules instructions, Final Diagnosis and Staging Summary (synoptic report) have equal coding priority. However, it is unclear which takes priority, or if this should be a combination of components, when the histologies are two different specific histologic types per Table 3 of the Breast Solid Tumor Rules Manual.

11/18/2019 Left 1st Digit/Thumb Biopsy: Atypical Melanocytic Proliferation consistent with Early Acral Lentiginous Melanoma in situ. Margins Positive. (Not a reportable diagnosis for 2019.)

12/5/2019 Left 1st Digit Shave Biopsies: Malignant Melanoma in situ. Margins Positive.

1/15/2020 Started Aldara (treatment plan: use for ~3 months then Mohs/excision, but due to COVID could not get resection until 7/2020).

7/29/2020 Left Thumb Excision: Residual Melanoma in situ. Margins Positive. Treatment Plan: re-excision.

8/6/2020 Left Thumb Re-Excision: Atypical Lentiginous Melanocytic Proliferation at the 12-2 margin may represent the advancing edge of melanoma in situ. (8/19/2020 Plan to treat the 12-2 margin as positive with in situ; plan for re-excision).

8/20/2020 Left Thumb Re-Excision & Left Nail Plate Excision: Malignant Acral Lentiginous Melanoma with extensive melanoma in situ. Breslow 1.3mm. Margins Positive. Nail plate & bed epithelium with hemorrhage and a mild increase in melanocyte density likely represent melanoma in situ.

9/4/2020 Left thumb partial amputation & Left axillary Sentinel Lymph Node Excision: Residual Malignant Melanoma in situ. 0/3 sentinel nodes positive.

Per the site validation list: https://seer.cancer.gov/icd-o-3/sitetype.icdo3.20190618.pdf#search=site%20validation, ill-defined sites (ILL-DEFINED C760-C768) does not include 8070- Squamous cell carcinoma as a valid histology. Therefore when a Cervical Lymph Node and Unknown Primary Tumor of the Head and Neck is submitted with a C760 and 8070/3, it requires an override be set.