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Rule H6 states: Code 8742/3 (Lentigo maligna melanoma) when the diagnosis is lentigo maligna melanoma with no other histologic types. However, if the diagnosis was strictly lentigo maligna or lentigo maligna melanoma, the first rule that applies is Rule H1 because lentigo maligna melanoma is a single, specific histologic type and Rule H1 states, Code the histology when only one histologic type is identified. Following the current rules, one would never arrive at Rule H6. Should the H Rules be reordered? Or should an example of when one would use Rule H6 be added to clarify when to use this rule?

For example, total thyroidectomy specimen shows right lobe papillary thyroid carcinoma, 4.2 cm, unencapsulated, with numerous psammoma bodies in non-tumoral thyroid parenchyma, without angioinvasion; left lobe with papillary thyroid carcinoma, 0.6 cm, encapsulated, with capsular invasion, with intralymphatic psammoma bodies in non-tumoral thyroid parenchyma, without angioinvasion. The synoptic summary documents vascular invasion present (psammoma bodies only).

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.

The following cases were submitted to the central registry as separate primaries. First case submitted as C772 with histology coded 9591/3 (Lymphoma, NOS). Second case submitted as C162 with histology coded 9699/3 (MALT Lymphoma).

Sequence 01 - 5/2016, Excisional biopsy pancreatic tail lymph node: suspicious for malignant B-cell lymphoma. No treatment recommended or administered.

Sequence 02 - 2/2019, Stomach biopsy: MALT Lymphoma. Unknown if treatment was recommended or administered. Biopsy was only at this facility.

Using the Hematopoietic and Lymphoid Neoplasm Multiple Primaries/Histology rules, Rule M7 makes this a single primary. Note 4 instructs to change the histology of the initial abstract to the more specific histology (9699/3). If this is done, they would be multiple primaries per the exception within Rule M2. Should the histology on sequence 01 be changed to the MALT lymphoma and the cases would be multiple primaries or is this a single primary?

4/28/21 PET: There is extensive widespread/multifocal hypermetabolic uptake within lymph nodes, skeleton, and spleen, compatible with malignancy. Differential diagnosis includes lymphoma and metastatic disease of indeterminate primary, with lymphoma favored.

4/28/21 Right retroperitoneal lymph node, needle core biopsy: Large B-cell lymphoma. See comment. Comment: The differential includes T-cell/histiocyte-rich large B-cell lymphoma and diffuse variant of nodular lymphocyte predominant Hodgkin lymphoma. It is challenging to distinguish these two on the needle core biopsy. An excisional biopsy is recommended for a definite diagnosis if clinically appropriate. ADDENDUM: B-Cell Lymphoma, FISH: negative. No rearrangement of MYC, BCL2 and BCL6 and no fusion of MYC and IGH.

5/14/21 Gastrohepatic lymph node, biopsy: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) with T-cell/histiocyte rich diffuse large B-cell lymphoma-like transformation. Focal in situ follicular neoplasia.

6/3/21 Medical Oncologist: Biopsy confirms that patient has a nodular lymphocytic Hodgkin lymphoma which has transformed into a T-cell rich DLBCL. This variant of Hodgkin disease is a good prognostic histology which generally behaves indolently, like a low grade lymphoma.

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.

The Solid Tumor histology coding guideline #3 for Lung states that an ambiguous histology can only be coded over an NOS when a physician clinically confirms it or the patient receives treatment based on the ambiguous histology; similar instructions exist in rules H3 and H12.  We are in a central registry and don't typically have access to physician notes or treatment plans; unfortunately our hospital abstracts rarely document physician confirmation of ambiguous histology and we are uncertain if we should accept their coding of the more specific histology, assuming they did find clinical confirmation that was not documented.  If not, our understanding of the Solid Tumor rules is that the histology in such a case would have to be coded as malignancy NOS (8000/3) per the non-ambiguous final diagnosis, and that we cannot use the more specific but ambiguous squamous cell carcinoma since we don't have definite clinical confirmation.  We also have a fair number of cytology-only lung cases without any hospital information to clinically confirm an ambiguous histology.