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For tumors diagnosed prior to 2007:

Code histology to 9084/3 [Dermoid cyst with malignant transformation] per the ICD-O-3. Dermoid cysts may contain a malignant component of a type typically encountered in other organs and tissues.

For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Code the primary site to C421 [bone marrow] per Rule PH30 which states to use the to determine the primary site and histology when rules PH1-PH29 do apply. Leukemia cutis is the term for a leukemic infiltration of the epidermis, the dermis or the subcutis. This infiltration is easily identified as cutaneous lesions, but the primary site is still bone marrow. This is a type of "metastasis" or spread of the leukemia cells. The "conventional" definition for leukemia cutis is the infiltration of skin from a bone marrow primary. See the Hematopoietic & Lymphoid Neoplasm Coding Manual Glossary.

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

For cases diagnosed 1998-2003:

Code EOD-extension to 30 [localized, NOS]. This is mucosal spread (since there is no muscle in the uvula). Soft palate and uvula are handled as a single site, and extension from uvula to soft palate is not addressed in EOD.

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.

Code CS Lymph Nodes 00 [clinically negative]. See note 3 for CS Lymph Nodes.

Code SSF 4 and 5 000 [Nodes clinically negative].

This is a correction to the SEER manual. Lymphomas originating in lymph nodes (C77) could have distant metastases to any site except lymph nodes. The following corrections to the manual apply now and will appear in the next version of the manual.

Remove C770-C779 from the instruction for assigning code 8 on the following pages.

Page 135 Mets at Dx--Bone

Page 137 Mets at Dx--Brain

Page 139 Mets at Dx--Liver

Page 141 Mets at Dx--Lung

Page 145 Mets at Dx--Other

Example

Biopsy of axillary lymph node: Diffuse Large B-Cell lymphoma. Lymph nodes involved above and below the diaphragm, multiple nodules seen in lung, lesions in liver. Bone marrow biopsy positive for DLBLC. Per Hematopoietic manual, primary site would be C778 for multiple lymph node regions involved.

Mets at Dx--Bone-0

Mets at Dx--Brain-0

Mets at Dx--Liver-1

Mets at Dx--Lung-1

Mets at Dx--Distant Lymph Nodes-8

Mets at Dx--Other-1

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.

Malignant perivascular epithelioid cell tumor (PEComa) is reportable because it is malignant. Assign 8005/3 to malignant PEComa.

We consulted an ICD-O-3 expert who explained that some PEComas such as angiomyolipoma and lymphangiomyomatosis have specific ICD-O codes and their malignant counterparts may be coded to 8860/3 and 9174/3 respectively. There are no separate ICD-O codes for other specific PEComas, e.g., clear cell “sugar” tumor of lung, clear cell myomelanocytic tumor of the falciform ligament and some “unusual” clear cell tumors occurring in other organs—or for PEComa, NOS. These PEComas may therefore be coded to 8005 as clear cell tumors NOS; in other words as clear cell tumors that are not clear cell variants of carcinomas, sarcomas, or other specific tumor type.

Please note, PEComa is non-specific as to behavior. Unless the pathologist states that it is malignant, (as was the case for this question), the default code is 8005/1 (non-reportable).

For cases diagnosed 1998-2002:

Code the Reason for No Cancer-Directed Surgery field to 1 [Cancer-directed surgery was not recommended] for patients who present with either a primary site or histology for which surgery is not a standard treatment. Also use code 1 for those patients who present with distant disease for a primary site that is typically treated surgically. Patients with distant metastasis typically do not have surgery performed as part of first course of treatment.

Code 2 [Contraindicated due to other conditions] is used when surgery would normally be recommended for the site (given the current stage of the tumor) but other medical conditions pose too much of a risk for the patient to undergo surgery.

Diffuse intrinsic pontine glioma is reportable. For cases diagnosed in 2018, assign 9385/3.