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20100022 | Multiple primaries/Histology--Heme & Lymphoid Neoplasms: Is a 2010 diagnosis of ALK+ anaplastic T cell lymphoma following a 2008 diagnosis of follicular B cell lymphoma, grade 1 a new primary? If so, how is the histology coded? See Discussion. | A patient has a history of Stage 4 follicular B cell lymphoma, grade 1 [9695/3] diagnosed in 2008. The patient was treated with Adriamycin, Cytoxan, Rituxan, and Prednisone. In 2010, the medical oncologist states the patient has progression/recurrence of lymphoma with pathology that has changed to anaplastic T cell lymphoma ALK+. There was immunophenotyping, but there was no more specific diagnosis made. The patient died within 3 months. | Updated May 2026 Rule M15 applies to this case which instructs you to use the Multiple Primaries Calculator. The result for 9695/3 and 9714/3 is "New Primary."
Abstract the anaplastic T cell lymphoma as a new primary. Code the histology to 9714/3 [Anaplastic large cell lymphoma, ALK-positive].
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2010 |
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20130178 | Reportability--Heme & Lymphoid Neoplasms: Is refractory iron deficiency anemia reportable? | Updated May 2026 Refractory iron deficiency anemia is not reportable for any year of diagnosis. It is not a clonal disorder and, therefore, is not malignant. Refractory iron deficiency anemia is a condition that is unresponsive to oral iron treatment. |
2013 | |
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20100096 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a 9/30/10 biopsy diagnoses follicular lymphoma, grade 1 and the patient is subsequently diagnosed on a 10/11/10 biopsy with large B-cell lymphoma which is stated to be a transformation of the prior lymphoma? | Updated May 2026 Per Rule M11, this case is to be accessioned as two primaries: follicular lymphoma, grade 1 [9695/3] and diffuse large B-cell lymphoma (DLBCL) [9680/3]. The case represents a chronic neoplasm (follicular lymphoma, grade) and an acute neoplasm (diffuse large B-cell lymphoma) which was diagnosed after the initial clinical workup of the chronic disease was completed and there is documentation of two biopsies, one confirming the chronic disease and the other confirming the acute disease. |
2010 | |
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20100018 | Reportability/Heme & Lymphoid Neoplasms--Hematopoietic, NOS: Is light chain disease reportable if it is treated with chemotherapy agents? See Discussion. | A patient was diagnosed in 2010 with light chain disease based on SPEP and urine testing. Bone marrow aspiration and biopsy were done. Flow cytometry, cytogenetic studies and FISH for plasma cell disorders are all normal. Medical oncologist states diagnosis is light chain disease. Patient was started on Revlimid, dexamethasone and Velcade.
In reviewing the case reportability instructions, this seems to fall under Instruction 1, note 1. Immunoglobulin deposition disease, preferred term for light chain disease, is coded as 9769/1. This is normally a non-reportable diagnosis, but the patient was given cancer-directed treatment. Would this case be accessioned using the above morphology code and primary site of bone marrow [C42.1]? |
Updated May 2026 Light chain disease (9769/1) is not reportable for any year of diagnosis. See the Hematopoietic Database for further information.
Light chains are produced in neoplastic plasma cells (multiple myeloma) and are called Bence-Jones proteins. The physician did the cytogenetic studies and FISH to rule out plasma cell disease. 50-60% of people with Light-chain deposition disease (LCDD) have an associated lymphoproliferative disorder, most commonly multiple myeloma. The remaining patients develop LCDD in the setting of progression of monoclonal gammopathy of unknown significance (MGUS) with no evidence of neoplastic plasma cell proliferation. This patient falls in this category, MGUS, which is not reportable. |
2010 |
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20130195 | Laterality--Heme & Lymphoid Neoplasms: Is laterality coded to 0 [not paired] for all lymphoma cases including paired sites (e.g., breast, lung)? | Updated May 2026 Laterality coding for lymphomas is based on the primary site not histology. Laterality describes the side of a paired organ or side of the body on which the reportable tumor originated. Determine whether laterality should be coded for each primary.
Laterality coding instructions are located in the SEER Program Coding and Staging Manual. |
2013 | |
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20130118 | Primary site--Heme & Lymphoid Neoplasms: How is the primary site coded for a diagnosis of Langerhans cell histiocytosis with extensive bony metastatic disease and lymphadenopathy? See Discussion. | Patient was diagnosed with LCH on a biopsy of the right femur. Imaging showed extensive bony metastatic disease, extensive infiltrative perinephritis, encasement of both kidneys, renal hilar, retroperitoneal and periaortic lymphadenopathy. The right femur biopsy pathology report did not state this was metastatic. | Updated May 2026 In the Heme DB, there is a note in 9751/3 that states if primary site cannot be determined for LCH, code the primary site to C419 [bone, NOS].
This patient has widely metastatic disease. Per the Abstractor Notes section, Langerhans cell histiocytosis arises in the bone and many times can involve multiple bones, along with other organs and lymph nodes. Although the right femur was biopsied, this does not prove that the primary site is the femur [C402] because the patient has what was described as extensive bony metastatic disease. |
2013 |
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20130215 | Reportability--Heme & Lymphoid Neoplasms: Is hemophagocytic lymphohistiocytosis synonymous with an EBV-associated lymphoproliferative disorder in children reportable? See Discussion. |
Pathology report states: Prominent T-cell infiltrate with frequent immunoblast-like cells. COMMENT: Findings consistent with an acute EBV-associated hemophagocytic process. In addition, there is a prominent CD8 + T-cell infiltrate with many large, activated forms. This T-cell process may represent an EBV-associated lymphoproliferative disorder in children. EBV-associated lymphoproliferative disorder in children is listed in the Heme database. However, throughout multiple admissions, the oncologist states the diagnosis as "hemophagocytic lymphohistiocytosis". Are the two the same condition? The patient is being treated with Etoposide. |
Updated May 2026 Hemophagocytic lymphohistiocytosis is not reportable for any year. The oncologist likely used the pathology report and clinical factors to determine the diagnosis of hemophagocytic lymphohistiocytosis, which is not reportable. Hemophagocytic lymphohistiocytosis is caused by an over stimulated immune system (infection, etc.). This clinical syndrome is associated with a variety of underlying conditions. To be reportable, it must state "fulminant hemophagocytic syndrome" (in a child) to be reportable (9724/3). The pathology report for this case is not definitive. It states that the process "may" represent the EBV-associated lymphoproliferative disorder in children. Follow back on this case to confirm reportability if possible. |
2013 |
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20170014 | Reportability/Histology--Heme & Lymphoid Neoplasms: Is a physician statement that a patient has a malignant histiocytic disorder best described as Erdheim-Chester disease reportable? If reportable, should histology be coded to 9751/3? See Discussion. |
The patient had a mediastinal mass biopsy showing fibrosclerotic tissue with patchy lymphohistiocytic foci and scattered plasma cells, followed by a retroperitoneal mass biopsy showing fibrohistiocytic infiltrate. Erdheim-Chester disease is not reportable per the Heme Database. However, the physician specifically states this is a malignant disorder. |
Updated May 2026 For years prior to 2021, Erdheim-Chester disease is not reportable. Starting in 2021, Erdheim-Chester disease is reportable and coded 9749/3. See the Hematopoietic database. |
2017 |
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20091048 | Surgery of Primary Site--Lymphoma/Soft Tissue: How is this field coded for an excision of a neck mass that found lymphoma in soft tissue (C49.0)? See Discussion. | CT scan showed soft tissue mass in the retropharynx. 9/23/2008 Laryngoscopy with biopsy taken of left tonsil and left base of tongue and random biopsies of nasopharynx; FNA of left neck. Path stated left tonsil, squamous papilloma. Left base of tongue, no significant histopathology. Nasopharynx biopsies, compatible with tonsillar tissue. Pretracheal lymph node biopsies, mild reactive lymphoid hyperplasia. 9/30/2008 Excision of left neck mass with limited deep jugular chain lymph node dissection. Path stated lymph node left jugular biopsy, no tumor seen. Soft tissue, left neck biopsy, malignant B cell lymphoma with plasmacytoid differentiation. Addendum from consult: favor a diagnosis of a marginal zone lymphoma. Per the gross description, the specimen was fibrofatty connective tissue in which there is a tumor infiltrate. | Updated May 2026 For cases diagnosed prior to 2023: Assign code 26 [partial resection]. Use the surgery codes that apply to the primary site, C490-C499. For cases diagnosed 2023+, see code A260. |
2009 |
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20160052 | Summary Stage 2000--Lymphoma: How is SEER SS2000 coded for an ocular adnexal lymphoma when it extends from the primary site to adjacent sites that are still orbital structures? See Discussion. |
In this case, the lymphoma arose in the posterior orbit and the primary site was coded as C696 (orbit, NOS). The mass directly extended to at least one "adjacent" site, the lacrimal gland. Should SS2000 be coded to 1 (localized) or 5 (regional, NOS) when an ocular adnexal lymphoma arises in the posterior orbit and extends to involve the lacrimal gland? Although both the posterior orbit and the lacrimal gland are parts of the orbit, they have separate ICD-O-3 topography codes. Should extension to multiple sites within the orbit be classified as localized disease?
The issue is what constitutes "adjacent" structures for a tumor that arises in the orbit. In an article published by the Indian Journal of Opthamology it states, "According to the Ann-Arbor staging system, lymphoma confined to the orbit is designated as Stage I, involvement of adjacent structures (sinuses, tonsil and nose) makes it Stage II." Does SEER agree with this definition of "adjacent" structures? Or are the lacrimal gland, ciliary body, retina, conjunctiva and/or choroid "adjacent" structures for a lymphoma stated to arise in the posterior orbit? |
Updated May 2026 For cases diagnosed prior to 2018, assign SEER SS2000 code 5 (Regional, NOS) in the Lymphoma chapter for a lymphoma of orbit extending to lacrimal gland. In SEER SS2000, this is Stage IIE: Direct extension to adjacent organs or tissues. For cases diagnosed 2018+, see Summary Stage 2018, Lymphoma Ocular Adnexa chapter, Code 2 (Regional Extension)
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2016 |
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