Primary site--Heme & Lymphoid Neoplasms: What primary site and heme rule applies when a PET scan shows bilateral renal masses, hypermetabolic liver lesions and retroperitoneal lymphadenopathy, a right kidney biopsy was positive for diffuse large B-cell lymphoma, and the bone marrow biopsy was negative? See Discussion.
Patient has a history of chronic lymphocytic leukemia (CLL). February 2011 abdomen/pelvis x-ray showed development of bilateral renal masses. April 2011 PET scan showed intense areas of hypermetabolic activity corresponding to known bilateral renal masses, new hypermetabolic liver lesions, as well as left upper retroperitoneal lymphadenopathy. All findings are worrisome for malignancy. March 2011 right kidney mass biopsy was positive for diffuse large B-cell lymphoma (DLBCL). Bone marrow biopsy was negative for lymphoma.
Per Rule PH25, code the primary site of the diffuse large B-cell lymphoma to C649 (kidneys) and laterality to 4 (bilateral). Per PH25, code the primary site to the organ when a lymphoma is present in an and that . This patient had involvement of an organ (bilateral kidneys) as well as regional lymph nodes for that organ. The retroperitoneal lymph nodes are regional for the kidney.
The diffuse large B-cell lymphoma is an acute transformation of the chronic lymphocytic leukemia. Because the DLBCL occurred more than 21 days after the CLL, it is a new primary per Rule M10.
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.
Primary Site--Heme & Lymphoid Neoplasms: What primary site code should be assigned and what rule justifies that code?
Scenario: Pleural effusion, underwent thoracentesis. Pleural fluid unexpectedly showed Large B-Cell Lymphoma. Extensive workup including CT & PET was done and all findings were within normal limits. No evidence of lymphoma was seen and no palpable adenopathy was found. The only indication of lymphoma was the malignant pleural effusion.
Code to pleura, C384.
Per the Hematopoietic database, Diffuse Large B-Cell Lymphoma can originate in the pleural cavity.
Primary site--Heme & Lymphoid Neoplasms: When only pathology reports are available, how should the primary site be coded when a both a bone marrow biopsy and colon biopsy demonstrate "mantle cell lymphoma"?
For this case, code primary site to C189 [colon, NOS] per Rule PH24.
Mantle cell lymphoma usually begins with lymph node involvement and spreads to other tissue. However, it can begin in a lymphocyte such as those in the GI tract. Per the Abstractor Notes section in the Heme DB, patients usually present with advanced disease. About half will have some combination of B symptoms. Swelling of lymph nodes and spleen are usually present. Bone marrow, liver and GI tract involvement occurs in a very high percentage
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.
Primary site--Heme &Lymphoid Neoplasms: What is the primary site of two extraosseous plasmacytomas, with positive pathology of right orbit and left lung. The patient's bone marrow biopsy, flow, and peripheral blood smear were negative. Is this coded as 9732/3, multiple myeloma (Primary Site and Histology Rule PH2) with the primary site as C809 (PH27)? Or is the primary site C421 since code 9732 says primary site is always C421, though bone marrow came back as negative?
Assign the primary site to C421 since that is the only allowable primary site for plasma cell myeloma, even though the bone marrow was negative. According to the revised criteria from the WHO Blue Book for Hematopoietic and Lymphoid Neoplasms (2017), the presence of multiple plasmacytomas is plasma cell myeloma (9732/3).
Primary Site--Liver: What is the topography code for combined hepatocellular carcinoma/cholangiocarcinoma (M-8180/3) especially when there is no documentation that intrahepatic bile duct is the tumor site? Reports usually just indicate a liver mass(es) but since the intrahepatic ducts are within the liver, is the code C221 due to the cholangiocarcinoma component, thus making the case stageable?
If there is no further information about where the cancer originated, assign C220. Use ICD-O-3 as the source for coding topography. The topography code associated with combined hepatocellular and cholangiocarcinoma (8180/3) is C220 when there is no other information available, according to ICD-O-3.
Primary site--Lung: What primary site code is used for bronchus intermedius?
Assign code C340 [main bronchus].
The bronchus intermedius is the lower part of the main bronchus on the right side. The bronchus intermedius begins just below the point where the upper lobe bronchus branches off from the main bronchus. The bronchus intermedius branches into the middle lobe bronchus and the lower lobe bronchus.
Primary Site--Sarcoma: What is the best primary site code for an undifferentiated sarcoma of the pulmonary artery? See discussion.
Consolidation of the case: The operating hospital stated: SOFT TISSUE: Resection: Procedure: Radical resection Other: Pneumonectomy Tumor Site: Right pulmonary artery - They used code C383 (mediastinum NOS). The consulting hospital stated: Lung, right, pneumonectomy: High grade sarcoma consistent with intimal sarcoma; tumor involves pulmonary artery. They used code C449 (other soft tissue NOS). Would C493 (soft tissue thorax) be correct?
Code the primary site to pulmonary artery, C493. According to the WHO classification of tumors, intimal sarcomas are malignant mesenchymal tumors arising in large blood vessels. They show mostly intraluminal growth with obstruction of the vessel. They may occur in the pulmonary vessels or, less often, in the aorta.
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