Report | Question ID | Question | Discussion | Answer | Year |
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20110132 | Reportability/Histology--Heme & Lymphoid Neoplasms: Is a diagnosis of "small B-cell non-Hodgkin lymphoproliferative disorder" reportable? If so, how is the histology to be coded? See Discussion. | The final diagnosis of a bone marrow biopsy dated 10/99/2010 was "small B-cell non-Hodgkin lymphoproliferative disorder." The differential diagnosis includes atypical small lymphocytic lymphoma/chronic lymphocytic leukemia and marginal zone lymphoma. Mantle cell lymphoma is very unlikely based on BCL1 negativity. Lymphoplasmacytic lymphoma is also excluded due to the absence of a plasma cell component (CD138 negative). | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Yes. The term "small B-cell non-Hodgkin lymphoproliferative disorder" is reportable. Code the histology to 9591/3 [non-Hodgkin lymphoma, NOS] per Rule PH28. When there is a diagnosis of lymphoproliferative disorder and any lymphoma, code the lymphoma histology.
The information in the discussion is reflective of the difficulty in diagnosing hematopoietic and lymphoid neoplasms. The differential diagnosis indicates that a number of possible specific lymphoma/leukemia diagnoses that have been ruled out, which explains why the final diagnosis is non-Hodgkin, NOS.
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. |
2011 |
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20110017 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are reported if a patient originally diagnosed with CLL is subsequently diagnosed several months later on a bone marrow biopsy with Richter's syndrome that transformed into a large cell lymphoma? See Discussion. |
Per reviewed resources, the described condition is rare. Should the histology remain CLL or be changed to large cell lymphoma? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. This case is accessioned as two primaries per Rule M10 which states to abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm and there is a second diagnosis of an acute neoplasm more than 21 days after the chronic diagnosis. The first primary is CLL [9823/3] and it is a chronic neoplasm. The second primary is diffuse large B-cell lymphoma (DLBCL) [9680/3] and it is an acute neoplasm. Richter syndrome (RS) is a complication of B cell chronic lymphocytic leukemia (CLL) or hairy cell leukemia (HCL) in which the leukemia changes into DLBCL. There is also a less common variant in which the CLL changes into a Hodgkin lymphoma. Richter's transformation affects about 5% of CLL patients. Richter syndrome is listed under the Alternate Names section in the Heme DB for diffuse large B-cell lymphoma [9680/3]. 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. |
2011 |
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20110044 | MP/H Rules/Histology--Corpus uteri: What are the histologies for the primaries to be reported when the endometrium contains two separate tumors composed of adenocarcinoma with multiple differentiations as well as a separate small focus of clear cell carcinoma? See Discussion. |
The resected specimen showed, "Adenocarcinoma of endometrium with the following features: Histologic type: Endometrioid with squamous and focal clear cell differentiation. A second focus of endometrial adenocarcinoma is present in the fundus with admixed complex atypical hyperplasia in a polypoid, non-invasive mass. The second tumor is endometrioid with secretory differentiation. COMMENT: The tissue in between the two tumors is sampled, and contains foci of endometrial adenocarcinoma that is superficially present within the endometrium, as well as a small focus of clear cell carcinoma measuring 0.2 cm." Per MP/H rules M17, this is counted as multiple primaries because the histology codes differ at the third digit: 8323/3, 8382/3, 8310/3. The Multiple Primary rules make no reference to the histology tables. There is also no rule to ignore the in situ tumor. In addition, the histology table in the 2007 MP/H Rules Manual for Other Sites does not include "secretory differentiation" as a type of GYN malignancy. |
After consultation with our expert pathologist, the decision is report this case as a single primary. There was some confusion about how to apply the current MP/H rules to this pathology report given 1) the definition of M16 and M17 and 2) the likelihood for a single endometrial primary to present with several differentiations. According to our expert pathologist, "I would regard this case as a single endometrial primary with extensive endometrial involvement and several types of differentiation, all of which are seen in endometrial carcinomas." Next, the Multiple Primary and Histology Coding Rules Manual is the correct source for coding histology for cases diagnosed 2007 or later. The following steps are used to determine the histology code. Open the Multiple Primary and Histology Coding Rules manual. For an endometrial primary, use the Other Sites Histo rules to determine the histology code because endometrium does not have site specific rules. Start with the MULTIPLE TUMORS ABSTRACTED AS A SINGLE PRIMARY module, Rule H18. The rules are intended to be reviewed in consecutive order within the module from Rule H18 to Rule H31. You stop at the first rule that applies to the case you are processing. Code the appropriate combination/mixed code (Table 2) when there are multiple specific histologies. GYN malignancies with multiple types of adenocarcinoma have histology coded to 8323/3 [mixed cell adenocarcinoma] per rule H30. |
2011 |
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20110071 | Primary site: How is this field coded for an adenocarcinoma arising in a chronic perianal fistula without extension to the anal canal, but stated to arise in "ectopic rectal tissue"? See Discussion. | The patient underwent a resection of a perineal mass. Per review of slides it was stated to be "primary mucinous adenocarcinoma arising in a chronic perianal fistula." The adenocarcinoma was invasive into the dermal connective tissue and skeletal muscle, but there was no extension into the anal canal. The discharge diagnosis from the reporting facility called this adenocarcinoma of "ectopic rectal tissue in perianal area."
Should the primary site be coded to skin based on the dermal involvement and lack of anal or rectal involvement? Or, should the primary site be coded to rectum based on the physician's assessment that this adenocarcinoma arose in ectopic rectal tissue? |
For cases diagnosed 2007-2014: Code the Primary Site field to C210 [Anus, NOS]. This is an unusual and rare presentation. According to our expert pathologist, "There is no ideal site code [for] this case. I would code to C210. In this location it can at least be located by anyone who wants to get a look at such lesions. Because of the unusual location of this tumor, I would like to be able to code it to perineum, but it will be totally lost in those site codes as they represent extensive areas beyond perianal (skin of trunk, soft tissue of pelvis, and pelvis, respectively)... I would not code to rectum [because it would be] lost among too many primary rectal carcinomas." |
2011 |
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20110053 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned for a patient with a several month history of refractory anemia with excess blasts (RAEB), that may or may not have been treated, who now presents with a bone marrow biopsy that is compatible with acute myeloid leukemia? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Per Rule M10, abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm AND there is a second diagnosis of an acute neoplasm more than 21 days after the chronic diagnosis. Two primaries should be accessioned for this case: refractory anemia with excess blasts (RAEB) [9983/3] (a chronic neoplasm), and acute myeloid leukemia [9861/3] (an acute neoplasm).
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. |
2011 | |
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20110108 | Primary site--Heme & Lymphoid Neoplasms: What is the primary site for a bone marrow biopsy positive for systemic mastocytosis that also involves the spleen and lymph nodes with associated leukocytosis, mild anemia and thrombocytopenia? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Per Rule PH30, one is to use the to determine the primary site and histology when rules PH1-PH29 do apply. Code the primary site to C421 [bone marrow] because that is the only site listed under the Primary Site section of the Heme DB.
Under the Abstractor Notes section in the Heme DB, it indicates that the bone marrow is always involved, and the white and red pulp of the spleen may be involved with systemic mastocytosis. This is how this patient presented; therefore, the bone marrow is the primary site. The spleen is secondarily involved because the spleen cleanses the blood and the neoplastic cells have infiltrated the red and white pulp of the spleen. The same is true for the lymph nodes. Although the lymph nodes are rarely involved, they may be involved when the patient has systemic mastocytosis.
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. |
2011 | |
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20110057 | MP/H Rules/Behavior--Appendix: How do you code mucinous cancers of the appendix? Is a "low grade mucinous appendix tumor/neoplasm" with peritoneal spread reportable? See Discussion. |
Low grade mucinous neoplasms can spread to the peritoneal cavity and in that sense are metastatic but histologically have bland/benign features (may be a benign cystadenoma that ruptured and spread by rupturing) are not a carcinoma. Thus, some have termed this group as DPAM (diseminated peritoneal adenomucinous) and not a true carcinoma. Others indicate that if you have metastasis the tumor is a carcinoma. |
For cases diagnosed 2007 or later, low-grade mucinous tumors of the appendix are a /1, borderline/uncertain behavior, and not reportable. These tumors do spread to the peritoneal cavity (pseudomyxoma peritonei). This spread, or deposits, or implants are also borderline/uncertain behavior and do not make the appendiceal tumor reportable. By contrast, a high-grade mucinous tumor of the appendix may produce malignant/invasive pseudomyxoma peritonei. When the pseudomyxoma peritonei are diagnosed as invasive or malignant, the mucinous tumor in the appendix is reportable as a /3. |
2011 |
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20110045 | Reportability--Ovary: Is immature teratoma of the ovary reportable if a subsequent comment states that "the teratoma shows immature neuroepithelium, but no malignant elements"? | There is conflicting information for this case. The final diagnosis conflicts with the comment. Go back and check with the physician to clarify his/her intent. If no further information can be obtained, the final diagnosis is preferred over the comment. This case is reportable based on the final diagnosis: "immature teratoma." | 2011 | |
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20110046 | MP/H Rules/Multiple primaries--Stomach: If there is no statement of recurrence, how many primaries are to be abstracted when a patient is diagnosed with multiple gastric carcinoid tumors between 12/2003 and 3/2009? See Discussion. |
Are the multiple primary rules applicable when a patient has multiple gastric carcinoid tumors? The patient was diagnosed with carcinoid tumors starting in 12/2003 through 3/2009. According to the 2004 SEER Manual, rule 5, if a tumor with the same histology is identified in the same site at least two months after the original diagnosis, this is a separate primary. The physician does not indicate that the pre-2007 carcinoid tumors were recurrent. The patient does not have familial polyposis syndrome. Should each of the following tumors be a separate primary? 12/2003 - Gastric Polyp Removal - Path: Gastric carcinoid tumor 05/2004 - Stomach body polyp removal - Path: Carcinoid Tumor (endocrine cell tumor) 09/2004 - Single polyp in body removal - Path: Gastric carcinoid 03/2005 - Multiple gastric body polyps removed - Path: Carcinoid tumor 07/2005 - 3 small polyps in fundus removal - Path: Carcinoid tumor 02/2007 - Localized nodularity in lesser curvature - Path: Carcinoid (neuroendocrine) tumor 03/2009 - Stomach body polypectomy - Path: Carcinoid tumor |
Code as a single primary. The histology is carcinoid. Our expert pathology consultant replied as follows: "This patient clearly has a condition driving the proliferation of neuroendocrine cells. Possibilities include hypergastrinemia from a gastrinoma or from response of antral gastrin cells due to achlorhydria from long standing chronic atrophic gastritis, or multiple endocrine neoplasia (MEN1) syndrome (genetically driven). How should these cases be coded given we do not have a way to code the inciting situation. (I suspect the gastroenterologist knows what it is, but we haven't obtained that information.) We do not have an ICD-O-3 code for the underlying condition, MEN1 or hypergastrinemia. Therefore, the only choice is to code the resulting tumor, carcinoid [8240/3]." |
2011 |
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20110077 | MP/H Rules/Multiple primaries--Breast: How many primaries are to be reported if different recurrence scores are found on the Oncotype Dx studies performed for multiple tumors in the same breast if the clinician states the patient has two primaries but the pathologist does not address the issue? See Discussion. | A patient has two separate lesions in the same quadrant with the same histology. According to the MP/H rules this is a single primary. However, Oncotype Dx studies were performed on both tumors and the DX recurrence was different for each tumor. The medical oncologist states the patient has two primaries. The pathologist does not indicate the number of primaries. | This is a single primary. The only rules used to determine the number of primaries are the MP/H rules for cases diagnosed 2007 or later. Do not use other information such as Oncotype Dx to determine the number of primaries for a patient. Oncotype is used to determine whether the cancer is likely to recur AND whether the cancer would benefit from chemotherapy.
The steps used to arrive at this decision are:
Open the Multiple Primary and Histology Coding Rules manual. Once in the manual, locate the Breast MP rules under one of the three formats (i.e., flowchart, matrix or text).
Start with the MULTIPLE TUMORS module, Rule M4. The rules are intended to be reviewed in consecutive order within the module from Rule M4 to Rule M13. You stop at the first rule that applies to the case you are processing.
The patient has two tumors in the same breast with the same histology. Abstract a single primary for this patient. |
2011 |