| Report | Question ID | Question | Discussion | Answer | Year |
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20110061 | Primary site/Histology--Heme & Lymphoid Neoplasms: Should the primary site and histology codes be updated when a patient with a history in 2005 of a bone marrow diagnosis of chronic lymphocytic leukemia later presents in 2010 with lymph node biopsy diagnosis of small B-cell lymphocytic leukemia? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. Per Rule M2, this is a single primary because there is a single histology. Code histology to 9823/3 [CLL/SLL]/ The distinction of CLL vs. SLL cannot be made on bone marrow biopsy in isolation. The pathologist cannot make a diagnosis of CLL vs SLL without having peripheral blood counts available for review. If the patient was treated for CLL in the past, that may alter the peripheral counts seen in 2010 (e.g., lymphocytosis). The distinguishing feature is peripheral lymphocytosis in CLL (not seen in SLL). The disease looks the same and both will often have bone marrow involvement and lymph node involvement. If the patient had true CLL in 2005, then any subsequent lymph node (or other) biopsy consistent with CLL/SLL remains consistent with the original diagnosis of CLL. I would not change the original CLL code. I agree with the previous response. We have to assume the 2005 diagnosis included a peripheral blood supporting that diagnosis. Otherwise, CLL and SLL look the same in nodes and marrow. The interplay between the two "diseases" is expected. This is why they are considered a single disease. 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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20100056 | Primary site/Histology--Heme & Lymphoid Neoplasms: How are these fields coded for a case with pathologic diagnosis of "anaplastic large cell lymphoma, ALK-negative" involving the brain and a clinical statement of involvement in the right inguinal lymph nodes and the right lower extremity by a cutaneous lymphoma? See Discussion. |
The final diagnosis on the pathology report for a brain biopsy is "Anaplastic large cell lymphoma, ALK-negative." Per a progress note: right inguinal lymphadenopathy. CT scan is consistent with multiple lymph node groups enlarged. Right lower extremity cutaneous nodular lesion; cutaneous lesions likely cutaneous lymphoma.
Should the histology be coded 9702/3 [anaplastic large cell lymphoma, ALK-negative], and the primary site C447 [skin of leg]? Or is the physician using "cutaneous lymphoma" as a general term indicating infiltration and the primary site is really C779 [lymph nodes, NOS]? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code to primary site to C447 [skin of leg]) per Rule PH25 and histology to 9702/3 [anaplastic large cell lymphoma, ALK-negative]. Per the Abstractor Notes section in Heme DB, these are the usual presentations for this disease. It also states this disease presents with peripheral node involvement and is often generalized with infiltrates in the bone marrow, liver, spleen, and extranodal tissue. Less frequently involved sites are lung, salivary gland and CNS.
Per PH25, code the primary site to the organ when the lymphoma is present in an organ (skin, right leg) and that organ's regional lymph nodes (inguinal). Distant lymph nodes or other organs may also be involved, but should be disregarded for coding primary site.
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. |
2010 |
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20100028 | Primary site/Histology--Head & Neck: How are these fields coded when the final diagnosis for a skull based mass is "neuroendocrine carcinoma" and the IHC studies are incompatible with a brain/spinal cord primary (ependymoma)? See Discussion. |
The pathology report final diagnosis is, "skull base mass, biopsy: neuroendocrine carcinoma, see note. NOTE: Ancillary IHC studies reveal ...the IHC signature is incompatible with ependymoma. The constellation of findings is diagnostic of well differentiated neuroendocrine carcinoma." The site/histology combination of C410 and 8246/3 is 'impossible' by SEER edits. There is no override. What is the correct primary site and histology? |
According to our subject matter expert physician, this unusual case is most likely a sino-nasal tumor (some variant of esthesioneuroblastoma [olfactory neuroblastoma]). Code to nasal cavity [C300] as indicated in ICD-O-3 by site-associated topography code attached to the morphology code for olfactory neuroblastoma [9522/3]. |
2010 |
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20031112 | Primary Site/Histology (Pre-2007)--Unknown & ill-defined site: How are these fields coded for a markedly atypical high grade malignant neoplasm diagnosed by a fine needle aspiration of a large iliac mass, right buttock area? See Description. |
The diagnosis was made in Oct. 2002 by a CT guided fine needle aspiration of a large iliac mass, right buttock area. The cytology report says: a. positive for malignant cells, markedly atypical high grade malignant neoplasm. b. It is impossible to tell from this aspiration biopsy whether or not this represents a high grade sarcoma or a high grade carcinoma, but our consensus opinion is that this lesion is a high grade carcinoma. The combination of soft tissue topography and carcinoma morphology is Impossible by SEER edits. How should we code this? |
For tumors diagnosed prior to 2007: Code the site to C76.3 [Pelvis, NOS]. Code the histology to 8010/34 [Carcinoma, NOS, high grade]. Unless there is better information available regarding the site, assign C76.3. The information provided above does not indicate the exact site of the mass. Code the histology based on the consensus opinion stated above. 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. |
2003 |
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20230078 | Primary Site/Heme & Lymphoid Neoplasms--CLL/SLL: Should the primary site be coded C421 (bone marrow) for a diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) when the managing physician provides a Rai stage? See Discussion. |
The patient has adenopathy and a lymph node biopsy proved CLL/SLL. The patient underwent a peripheral blood smear, but the final diagnosis only indicated there is an abnormal CLL panel, positive for monoallelic or biallelic deletion of 13q. The pathologist noted a CLL related clone was detected, but there was no definitive diagnosis of CLL on the peripheral blood. No bone marrow biopsy was performed. However, the managing physician noted this was Rai Stage I CLL/SLL with adenopathy in the neck. The SSDI Manual notes, “Rai stage is only applicable for CLL, in which the bone marrow and/or peripheral blood are involved (primary site C421 for bone marrow, see Hematopoietic Manual, Module 3: PH 5, 6).” Should primary site default to C421 if the physician provides a Rai Stage in the absence of definitive peripheral blood or bone marrow involvement documented in the medical record? |
Assign primary site C421. The Site-Specific Data item (SSDI) Manual, Rai Classification section, states: Per confirmation from medical oncologists, Rai stage is only recorded for patients who have bone marrow and/or peripheral blood involvement. Per the Hematopoietic Rules, primary site would be C421 (See Hematopoietic Manual, Module 3: Rules PH 5, 6). A new code has been added to the 5 SSDIs (code 5) to use when primary site is not C421. |
2023 |
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20010122 | Primary Site/EOD-Extension/EOD-Lymph Nodes--All Sites: What codes are used to represent these fields for an "extramedullary myeloid tumor (granulocytic sarcoma)" of the colon with positive or negative lymph nodes? |
For cases diagnosed 1998-2003: If only the extramedullary site is involved, such as colon, code the Primary Site field to the site of origin. Granulocytic or myeloid sarcoma is an exception to the rule that all leukemias should be coded to bone marrow as the primary site. Granulocytic sarcoma is a deposit of malignant myeloid cells in a site other than bone marrow (extramedullary). For EOD staging, granulocytic sarcoma [9930/3] is included in the Hematopoietic, Reticuloendothelial, Immunoproliferative and Myeloproliferative Neoplasms scheme and the Extension field is coded to 10 when the lymph nodes are negative, since it (like solitary plasmacytoma) is a localized deposit of tumor. However, if the regional lymph nodes associated with the extramedullary primary site are involved, code the EOD-Extension field to 80 [Systemic disease] because the disease is no longer an isolated deposit of malignant granulocytes (in other words, it is not localized). The EOD-Lymph Nodes field is coded to 9 regardless of whether or not the lymph nodes are involved because that is the only allowable code for that field. |
2001 | |
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20091065 | Primary Site/CS Extension--Lymphoma: How are these fields coded for a non-Hodgkins lymphoma case with scans that show non-specific parenchymal lung nodules and a large mediastinal mass? See Discussion. |
Patient presented with large bulky mediastinal mass. CT showed no pleural effusion. Findings also show non-specific parenchymal lung nodules. Biopsy of mediastinal mass showed malignant B-cell lymphoma of follicle center cell origin. Abdomen /Pelvis CT showed borderline lymph nodes in bifurcation. Clinical diagnosis was probable stage 3 if not 4 lymphoma. Per lymphoma guidelines, if extra-nodal primary site is assigned to the extranodal site if an extra-nodal site and its regional lymph nodes are involved. Would the parenchymal lung nodules be indicative of pulmonary involvement? If so, would primary site be lung? Or, would the parenchymal nodules be stage 4 disease and primary site be assigned to lymph nodes? |
For cases diagnosed prior to 1/1/2010, 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 Primary Site to C779 [Lymph node, NOS]. In this case, there is no statement that lymphoma involves the lung. "Nonspecific parenchymal lung nodules" are not indicative of lymphoma involvement. Consequently, this cannot be assumed to be an extra-nodal lymphoma. Additionally, it is not clear whether or not the "borderline" pelvic lymph nodes are involved. If the physician cannot provide more information, follow instruction 4.e in the SEER manual on page 72. 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. |
2009 |
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20190076 | Primary Site/Brain and CNS: How is primary site coded when the ICD-O-3 provides a sub-site-associated morphology code and the only information available to code primary site for a particular diagnosis indicates a non-specific/not otherwise specified (NOS) site code? See Discussion. |
ICD-O-3 Rule H states to use the topography code provided when a topographic site is not stated in the diagnosis. This topography code should be ignored if the tumor arose in another site. For the following brain and central nervous system (CNS) examples, should the suggested sub-site codes be assigned based on the histology, or should the primary sites be coded as C719 (posterior fossa or suprasellar brain) since the only information available was a tumor in these non-specific sites? Example 1: Resection of a posterior fossa tumor proved medulloblastoma, WNT-activated. Although medulloblastoma has a site-associated code in the ICD-O-3 (C716, cerebellum), the only information available is that this was a posterior fossa tumor (C719). Example 2: Resection of a suprasellar brain tumor proved pineoblastoma. The pathologist labeled this as a brain tumor, suprasellar. Although pineoblastoma has a site-associated code in the ICD-O-3 (C753, pineal gland), the only information available is that this was a suprasellar brain tumor (C719). |
If possilbe, ask the physician(s) about the exact site of origin. If it is not possible to obtain more information, the information in the medical documentation takes priority over ICD-O-3 Rule H, even when that results in a less specific topography code. |
2019 |
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20210051 | Primary site/Biliary tract--Ampulla of Vater: What is the correct primary site code for intra-ampullary and periampullary adenocarcinoma, C241 (8144/3) or C249? See Discussion. |
Ampulla, biopsy: High grade dysplasia with focal intramucosal carcinoma in a background of ulceration with acute and chronic inflammation. Surgery pathology: Head of pancreas, duodenum, and distal stomach, pancreaticoduodenectomy-Ampulla, Adenocarcinoma, intestinal type, intra-ampullary and peri-ampullary (mixed type). Grade moderately differentiated, 1.5cm. Tumor invades into duodenal submucosa. Lymphovascular Invasion: Foci suspicious for lymphovascular invasion identified. Perineural Invasion: Present. Synoptic report: Tumor Site Intra-ampullary and peri-ampullary (mixed type). Histologic Type Adenocarcinoma, intestinal type. There is not enough information regarding site in radiology reports or operative report. CT-A/P/C:The patient's known ampullary mass is not well visualized on this exam. No significant intrahepatic or extrahepatic biliary ductal dilation is identified. The pancreatic duct is normal caliber. |
Assign C241. Ampulla (C241) includes both periampullary and intra-ampullary. |
2021 |
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20210024 | Primary Site--Vulva: What is the primary site of patient with an excision of a left vulvar cystic mass showing focal mammary-type ductal carcinoma in situ (DCIS) on 11/06/2020? See Discussion. |
Final Pathologic Diagnosis: Vulvar cyst, excision: Focal mammary-type ductal carcinoma in situ, intermediate grade, arising within cystically dilated duct (See Comment) Size of DCIS: 0.7 CM. Margins: Negative. Comment Sections demonstrate a cystically dilated duct. Focally, at the periphery of the duct, there is a neoplastic monomorphic proliferation of ductal cells with intermediate grade nuclei. No associated necrosis is identified. Immunostains for GATA-3 and estrogen receptor are strongly positive within the neoplastic cells, supporting origin from mammary-like epithelium. Immunostain for p63 demonstrates preservation of a basal layer around the dilated duct, including the region involved by DCIS. Immunostain for cytokeratin 5/6 shows loss of expression within the DCIS. No stromal invasion is identified. The cyst appears to be completely excised. 12/01/2020 post op visit with surgeon: Ductal carcinoma in situ (DCIS) of the left vulva in an excised cystic lesion. PLAN: I reviewed the pathologic findings from the excision of the left vulvar cyst. This appears to be a cystic lesion in the mammary line with focal DCIS. It was excised completely with negative margins. It would not warrant any additional treatment except expectant management. |
Code the primary site to vulva. Use text fields to record the details. According to the WHO classification, several types of primary vulvar mammary-like carcinoma have been reported. It is rare and is thought to arise from specialized anogenital mammary-like glands within the vulva. It does not arise from ectopic breast tissue and is does not represent metastatic breast carcinoma. |
2021 |
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