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Report Produced: 02/24/2026 07:37 AM

Report Question ID Question Discussion Answer Year
20140010 Multiple primaries--Heme & Lymphoid Neoplasms: Is this one primary or two? Follicular lymphoma grade 1 (9695/3) on 8/23/12 from an abdominal lymph node. On 1/6/14 an abdominal lymph node biopsy showed diffuse large b cell lymphoma arising from high grade follicle center cell lymphoma. Patient has been on observation.

1st primary, 8/23/12: Follicular lymphoma, grade 1 2nd primary, 1/6/14: Diffuse Large B Cell Lymphoma

Apply the multiple primary rules twice for this case. The 2012 diagnosis is follicular lymphoma. There are two histologies in 2014: diffuse large b cell lymphoma and follicle center cell lymphoma diagnosed at the same time in the same location. This is one primary per rule M4.

Then compare the 2012 diagnosis to the 2014 diagnosis.

Per the Hematopoietic Database, follicular lymphoma (all types) transforms to DLBCL. Per Rule M10, the DLBCL would be a second primary.

2014
20140015 Primary site--Heme & Lymphoid Neoplasms: Is there an instruction missing under Rule PH22 of the 2014 Heme Manual that addresses when it might be appropriate to code primary site to C779 for a Stage II lymphoma? See discussion. It appears there is no instruction under PH22 that covers Example 5 (The patient has a history of Stage II lymphoma, no other information is available). All the bulleted instructions are for organ and lymph node combination involvement. Was the 2010 Heme Rule PH31 (Code the primary site to lymph nodes, NOS (C779) when lymph node(s) are involved but no primary site/particular lymph node region is identified) supposed to be listed under PH22? There does appear to be an empty bullet on the current web version.

The 5th bullet under Rule PH 22 was inadvertently omitted. A corrected version of the Heme manual will be posted soon. Thank you for identifying this omission.

In the meantime, please add the following to PH22:

Code the primary site to lymph nodes, NOS (C779) when lymph node(s) are involved but no primary site/particular lymph node region is identified.

 2014
20150045

MP/H/Histology--Thyroid:  What is the histology code for primary site of thyroid cancer with the histology of papillary thyroid carcinoma, classical and oncocytic type?

Code the histology to 8342/3, thyroid oncocytic (oxyphillic) papillary carcinoma.

2015
20150052

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.

2015
20150046

Reportability--Appendix:  Is the appendix the primary site for a low grade mucinous appendiceal neoplasm (LAMN) with diffuse peritoneal dissemination?  See discussion.

Patient had an appendectomy revealing a low grade mucinous appendiceal neoplasm (LAMN) with diffuse peritoneal dissemination. Patient now with cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC), which revealed metastatic disease in the abdomen, omentum, pelvic peritoneum, peri-cecal, and gallbladder.

For cases diagnosed prior to 1/1/2022

Low-grade appendiceal mucinous neoplasm (LAMN) is not reportable, even when it spreads within the peritoneal cavity, according to our expert pathologist consultant. Peritoneal spread of this /1 neoplasm does not indicate malignancy. It is still /1 when there is spread of LAMN in the peritoneal cavity.

 2015
20150047

Reportability--Bladder: Is a positive UroVysion test alone diagnostic of bladder cancer? See discussion.

The UroVysion website says that standard procedures, e.g., cytology, cystoscopy, take precedence over the UroVysion test. The Quest Diagnostics website says that "A positive result is consistent with a diagnosis of bladder cancer or bladder cancer recurrence, either in the bladder or in another site within the urinary system. A negative result is suggestive of the absence of bladder cancer but does not rule it out." Would we pick up the case if the UroVysion test was positive but the standard procedures were negative or non-diagnostic?

Do not report the case based on UroVysion test results alone. Report the case if there is a physician statement of malignancy and/or the patient was treated for cancer.

2015
20150021

MP/H Rules/Histology--Skin: How is histology coded for an "endocrine mucin-producing sweat gland carcinoma with transformation to mucinous carcinoma"? See Discussion.

Endocrine mucin-producing sweat gland carcinoma (EMPSCG) is a rare type of low-grade sweat gland carcinoma. Some journal articles indicate that most patients with EMPSCG have coexisting mucinous carcinomas, suggesting that EMPSCG is a precursor to invasive mucinous carcinoma of the skin. Sweat gland carcinoma has its own histology code per the ICD-O-3 (8400/3); should an endocrine mucin-producing sweat gland carcinoma also be coded as 8400/3? If so, would the correct histology for the skin case above be mucinous carcinoma (8480/3) per Rule H17? Conversely, if the terms "mucin-producing" are referring to mucin-producing carcinoma, and not referring to the sweat gland carcinoma, would the histology be coded 8481/3 (mucin-producing carcinoma)?

Assign 8480/3.

There is no mixed ICD-O-3 code for EMPSCG. Both histologies are in the mucinous family: mucinous adenocarcinoma (8480/3) and sweat gland carcinoma (8400/3). Apply Other sites rule H17 and code the numerically higher ICD-O-3 code (8480/3).

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare low-grade sweat gland carcinoma with a strong predilection to the eyelid region. It is histologically analogous to endocrine ductal carcinoma/solid papillary carcinoma of the breast and is characterized by a multinodular solid cystic mucinous tumor with immunoreactivity to neuroendocrine markers. Only 20 cases of this unusual tumor have been reported.

2015
20150015

Primary Site--Testis:  What is the prmary site for a 38 y/o male diagnosed with testicular cancer in a formerly undescended testis that was treated with orchiopexy at age 10-11?  See discussion.

Should it be coded to where the testis was physically at the time of diagnosis (C621), or should it be coded to C620 to reflect the increased risk for developing malignancy in an undescended testis?

Code the primary site C621 (descended testis). The primary site of this neoplasm is a scrotal (descended) testis. The history of orchiopexy can be noted in a text field, but does not change the primary site in this case.

2015
20150067

MP/H Rules/Histology--Kidney: What is the correct histology for this diagnosis? See discussion.

Procedure: Nephrectomy

Laterality: Left

Tumor type: SOLID VARIANT RENAL CELL CARCINOMA

Nuclear grade: High grade (3/4)

Histologic grade: Poorly differentiated

Pattern of growth: Solid

Tumor size: 5x4.5x4cm

Local invasion: Present

Renal vein invasion: None

Surgical margins: Negative

Non-neoplastic kidney: Unremarkable

Adrenal gland: Not submitted

Lymph nodes: Not present

Pathologic stage: T1b

There are solid sheets of tumor cells without papillary structure. The tumor stains positive for Pax-2, negative for Ecadherin, P63 and CK7, consistent with renal cell carcinoma, solid variant.

Assign histology code 8312, renal cell ca, NOS. There is no specific code for the solid variant of renal cell carcinoma.

2015
20150057

Reportability--Brain and CNS: Is this diagnosis reportable? If this neoplasm originated in the spinal cord, it is reportable, correct?

Specimen is described as a 'spinal cord mass.' The final diagnosis is 'fragments of adipose tissue demonstrating vascular proliferations consistent with angiolipoma. No histologic evidence of malignancy.' The microscopic description says: Sections of the spinal mass reveal bone, cartilage, fibrous tissue and adipose tissue. The adipose tissue demonstrates increased vascularity with thin walled blood vessels seen with islands of delicate fibrous stroma. The histologic findings are compatible with fragments of angiolipoma.

The neoplasm is reportable if it originated in the spinal cord or is intradural (within the spinal dura; spinal nerve roots are intradural). If there is not enough information to determine the exact site of origin, do not report the case.

2015