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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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20110042 | MP/H Rules/Histology--Testis: How is histology coded when the initial biopsies of retroperitoneal mass demonstrated non-seminomatous germ cell tumor, but after neoadjuvant chemotherapy the final diagnosis on the radical orchiectomy specimen demonstrated mature teratoma, NOS (not stated to be malignant)? See Discussion. | A large retroperitoneal mass was found on CT scan. A biopsy demonstrated non-seminomatous germ cell tumor. The biopsy was done at an outside facility. Neither the CT scan nor biopsy pathology report is available for review. Following neoadjuvant chemotherapy, the retroperitoneal mass decreased to 12 cm. Subsequently, the patient had a right radical orchiectomy. The final diagnosis per the pathology reports was a 3.5 cm mature teratoma (NOS, not stated to be "malignant") of right testicle. The patient then had resection of the retroperitoneal mass and biopsies. Pathology showed the "excision" specimen contained 6 benign lymph nodes and two of the "biopsy" specimens showed non-seminomatous germ cell neoplasm with IHC findings suggestive of a mix of embryonal carcinoma and a lesser component of yolk sac tumor. | This is a reportable case. Even though the pathology from the orchiectomy stated mature teratoma, NOS, the presence of lymph node metastases proves that this tumor is malignant. Code the histology as 9065/3 [germ cell tumor non-seminomatous].
The majority of germ cell tumors show the presence of multiple histologies. While the original tumor showed only mature teratoma, there were obviously yolk sac cells that were not detected on the sections taken from the primary tumor. Both teratoma and yolk sac are germ cell tumors. This explains why the pathologist gave you the diagnosis of germ cell tumor. The classification of "non-seminomatous" simply means that there was no seminomas present in the mixture of germ cell histologies. |
2011 |
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20110007 | MP/H Rules/Multiple primaries--Bladder: How many primaries are to be abstracted and how are the histologies coded when a bladder resection demonstrates tumor with invasive small cell neuroendocrine carcinoma [8041/3], high grade papillary urothelial carcinoma in situ [8130/2], adenocarcinoma in situ [8140/2], and multifocal flat urothelial carcinoma in situ? See Discussion. | Are the areas of in situ tumor to be ignored or would MP/H Rule M9 apply? |
Ignore the in situ histologies. This is a single primary. Code the histology to invasive small cell neuroendocrine carcinoma [8041/3]. | 2011 |
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20110012 | Reportability--Sarcoma: Is "atypical lipomatous tumor/well-differentiated liposarcoma" reportable? See Discussion. | The final diagnosis for a soft tissue excision is, "atypical lipomatous tumor/well-differentiated liposarcoma". The Comment section states, "Atypical lipomatous tumor/well differentiated liposarcoma has a significant risk for local recurrence, but no metastatic potential."
Per the 2010 SEER Manual, page 3, example 4: The pathologist makes the final decision about the behavior for a particular case. In this case, the pathologist uses both a reportable and a non-reportable term in the final diagnosis and in the comment section of the pathology report. Does the pathologist's comment impact the behavior and reportability of this tumor? |
For cases diagnosed 1/1/2014 and later: Atypical lipomatous tumor (8850/1) is not reportable. If the pathologist uses the term "well-differentiated liposarcoma" (8851/3) report the case. Use of this terminology indicates a less favorable prognosis. | 2011 |
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20110030 | Reportability--Heme & Lymphoid Neoplasms: If and when did Langerhans cell histiocytosis (LCH) become a reportable neoplasm? See Discussion. | Per the Histiocytosis Association of America, "Over the years, cancer treatments have been used in patients with histiocytosis. Consequently, hematologists and oncologists, who treat cancer, also treat children with Langerhans cell histiocytosis. However, the disease is not cancer." | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Langerhans cell histiocytosis (LCH) [9751/3] is reportable to all agencies starting for cases diagnosed 1/1/2010 and later. See Appendix D: New Histology Terms and Codes.
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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20110124 | MP/H Rules/Histology--Lung: How is the histology coded for a single tumor of the left lower lobe that is stated to be a sarcomatoid carcinoma with features of carcinosarcoma, spindle cell carcinoma, poorly differentiated squamous cell carcinoma and giant cell carcinoma? | Histology is sarcomatoid carcinoma [8033/3]. This case was sent to the lung physician experts because of the difficulty in trying to apply the current MP/H rules. Their rationale for the coding decision follows:
"This pathologist has diagnosed a sarcomatoid carcinoma, and then listed all of the subtypes associated with that diagnosis. I would go with the primary diagnosis, sarcomatoid carcinoma. The inclusion of squamous cell differentiation would exclude spindle cell and giant cell as diagnoses, so the pathologist is using them descriptively. We have no basis for picking one of the subtypes and sarcomatoid carcinoma covers all of the diagnoses given."
See the glossary in the Lung Equivalent Terms and Definitions for Sarcomatoid carcinoma: A group of tumors that are non-small cell in type and contain spindle cells and/or giant cells. Depending on the histologic features the tumor may be designated: pleomorphic carcinoma [8022/3]; spindle cell carcinoma [8032/3]; giant cell carcinoma [8031/3], carcinosarcoma [8980/3]; or pulmonary blastoma [8972/3]. |
2011 | |
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20110019 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are to be abstracted when bilateral testes are involved with lymphoma? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
This is a single primary per Rule M2 which indicates to abstract a single primary when there is a single histology. Code the histology to 9590/3 [lymphoma] and the primary site to C629 [testes. Unless your software has edits that prevent coding laterality for lymphomas, code the laterality as bilateral. Up to half of extranodal lymphomas occur in multiple sites, particularly in paired sites.
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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20110010 | Multiple primaries--Heme & Lymphoid Neoplasms: Is a recently diagnosed granulocytic sarcoma followed by a diagnosis of AML two primaries? See Discussion. |
6/10/10 Axillary lymph node biopsy was compatible with AML. The physician noted that the patient was diagnosed with granulocytic sarcoma [9930/3] in the axillary node. 6/15/10 Bone marrow biopsy compatible with AML FAB M1 [9873/3]. After induction, a second bone marrow biopsy on 6/30/10 shows persistent/refractory AML. The physician noted that the second biopsy is compatible with AML FAB M7 [9910/3]. Is the granulocytic sarcoma a chronic form of the disease? If so, do we have one primary diagnosed 6/10/10 with primary site coded to C42.1 and histology coded to 9873/3? Does the second biopsy on 6/30/10 represent the same primary even though the persistent disease is now FAB M7? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. Granulocytic sarcoma does not transform into AML. Per the Abstractor Notes section in the Heme DB under the term "granulocytic sarcoma," it indicates that "Myeloid sarcoma (also known as granulocytic sarcoma) may occur de novo; it may precede or coincide with AML, or represent an acute blastic transformation of myelodysplastic syndromes." This means that when granulocytic/myeloid sarcoma is seen with AML, it represents a solid manifestation of the systemically involved AML. In other words, it is all the same disease process (coded to AML) if it occurs simultaneously (i.e., at the same time or within 21 days of on another). Apply Rule M3 to this case which states to abstract a single primary when a sarcoma is diagnosed simultaneously or after a leukemia of the same lineage. Code the primary site to C421 [bone marrow] with histology coded to 9873/3 [acute myeloid leukemia, M1]. The FAB category is an older classification that is seldom used. Changes from FAB 1 to FAB 7 do not constitute a new primary. 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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20110032 | Primary site--Heme & Lymphoid Neoplasms: What primary site is coded for Langerhans cell histiocytosis (LCH) [9751/3] when it is limited to the skin? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Per Rule PH30, use the Heme DB to determine the primary site and histology when PH1-29 do not apply, In this case, code the primary site to C449 [Skin]. According to the Abstractor Notes section in the Heme DB, the solitary form of Langerhans cell histiocytosis (LCH) [9751/3] occurs less commonly than the multisystem form of the disease; but can appear in nodes, skin and lung. This is a solitary form of LCH. Code the primary site to skin [C449].
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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20110143 | Multiple primaries--Heme & Lymphoid Neoplasms: How many and what primary site(s) are to be accessioned when biopsies of clavicular and neck skin lesions are both consistent with mycosis fungoides? See Discussion. |
Per the Heme DB and Manual, this is a single primary; however, per the MP/H Rules, this would be multiple primaries. Which rules apply to this case? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. When there is a question of whether the SEER MP/H Rules or Hematopoietic and Lymphoid Neoplasm Rules apply, check the histology and refer to the Case Reportability Instructions in the Hematopoietic and Lymphoid Neoplasm Manual. All ICD-O-3 morphology codes in the range 9590 - 9992 are included in the Hematopoietic Rules. Mycosis Fungoides [9700/3] is included in this range. Therefore, the SEER MP/H Rules do not apply to mycosis fungoides. This case should be accessioned as a single primary: mycosis fungoides [9700/3] of the skin, NOS [C449]. Per Rule M2 abstract a single primary when there is a single histology. Note that in the Primary Site(s) section of the Heme DB, it states the primary site must always be coded to skin (C440 - C449) for mycosis fungoides. Because the primary site is stated in this section of the Heme DB, it is not necessary to use the Primary Site Rules to determine the primary site. Code the primary site to C449 [skin, NOS] because the patient has multiple sites of skin involvement and there is no documentation indicating which subsite of skin was the origin of the mycosis fungoides. 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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