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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.

The best code available for this situation is 8153/3 [Gastrinoma, malignant].

Many pancreatic endocrine tumors produce more than one peptide, such as gastrin and somatostatin in this case. ICD-O-3 does not provide a code for pancreatic endocrine tumors which produce more than one peptide. According to the WHO Classification of Tumours of Endocrine Organs, there is a distinct hormonal syndrome associated with gastrin producing tumors, and not with many of the somatostatin producing tumors. Therefore, our pathologist consultant advises us to code to gastrinoma in this case.

The histology code is 8720/3 [malignant melanoma, NOS].

There is no specific code for melanoma arising in melanocytic nevus. According to our pathologist consultant, this is likely because nevi are so common, melanoma arising in association with them is common and appears to have no bearing on prognosis or treatment. Most pathologists do not include the nevus in the diagnosis of melanoma, even when they see it.

Code melanomas arising in melanocytic nevi to the appropriate melanoma code, probably 8720, 8721, or 8743 in most cases.

For cases diagnosed prior to 1/1/2010:

Code the histology as 9699 [marginal zone B-cell lymphoma]. Code the histology from the original diagnosis.

For cases diagnosed 2010 forward, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ.

For cases diagnosed prior to 1/1/2010:Assign code 9690 [Follicular lymphoma, NOS]. According to the WHO Classification of Lymphoid tumors, follicular lymphoma is a neoplasm of follicle center B cells which has at least a partially follicular pattern.

Assign code 9695 for follicular lymphoma grade 1, 9691 for follicular lymphoma grade 2, and 9698 for follicular lymphoma grade 3.

For cases diagnosed 1/1/10 and later, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ.

For cases diagnosed prior to 1/1/2010:Assign code 9755 [Histiocytic sarcoma; True histiocytic lymphoma]. "Histiomonocytic" is not standard terminology, according to our expert consultant. However, 9755 is the best code to assign.

For cases diagnosed 2010 forward, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ.

For cases diagnosed prior to 1/1/2010:

The best code currently available for this entity is 9727/3 [precursor cell lymphoblastic leukemia].

The WHO classification refers to this as "Blastic NK-cell lymphoma." The 2005 WHO-EORTC classification for cutaneous lymphomas states that blastic NK-cell lymphoma may be derived from a plasmacytoid dendritic cell precursor. They suggest more appropriate terms for this condition may be "CD4+/CD56+ hematodermic neoplasm," and "early plasmacytoid dendritic cell leukemia/lymphoma." According to WHO, this is a rare form of lymphoma.

Willemze, et al. WHO-EORTC classification for cutaneous lymphomas. Blood, 15 May 2005. Volume 105, Number 10.

For cases diagnosed 2010 forward, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ.

For cases diagnosed prior to 1/1/2010:

9834/3 [Prolymphocytic leukemia, T-cell type]. According to the WHO Classification of Haematopeietic and Lymphoid Tissue Tumours, cerebriform or Sezary cell-like is a variant form of T-cell prolymphocytic leukemia.

For cases diagnosed 2010 forward, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ.

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

There has been a change in coding practice based on a change in clinical classification of leukemia/lymphomas. In the past, we did, indeed, default to lymphoma when both tissue and bone marrow were involved. The problem was that when only bone marrow was involved, the case was coded to leukemia with a primary site of bone marrow. When lymphoma symptoms developed later, there was a lot of inconsistency in how registries handled these cases. Some coded a new primary "lymphoma;" while others ignored the lymphoma calling it progression.

The clinical world, including the hematopoietic experts in the World Health Organization and the Inter-Lymph Consortium, agreed that for certain neoplasms (CLL/SLL being one of them) it was not useful or practical to code the leukemia and lymphoma separately OR to capture only one of the neoplasms (because these neoplasms almost always progress to lymphoma); so new codes for the leukemia/lymphoma were developed. According to the experts, 9823/3 most accurately portrays the neoplastic process for the neoplasms assigned to a lymphoma/leukemia code.

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.