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Patient has a history of B-cell lymphoma with multimodality treatment in 2002. Lab work in 2015 showed multilineage dysplasia leading to a diagnosis of therapy-related myelodysplastic syndrome. Patient presents in 2020 for a bone marrow biopsy now showing low-grade MDS. The MDS appears to have the same multilineage dysplasia previously identified.

MDS, NOS is not listed in the Heme DB as a possible transformation of t-MDS, nor is it listed as a Same Primary for t-MDS. Likewise, t-MDS is not listed as a more specific myelodysplastic syndrome, a transformation of MDS NOS, or a Same Primary as MDS, NOS.

The first M rule that applies to this case is M15, and the Multiple Primaries Calculator indicates that the MDS, NOS should be a new primary.

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.

ICD-O-3.2 lists microscopic thymoma as benign (8580/0) and thymoma, type A as malignant (8581/3).

January 2021: Left central neck node dissection for thyroid carcinoma with thymic tissue showing an incidental type A microscopic thymoma, described as a small (<0.2 cm) focus. Diagnosis comments further indicate this is morphologically consistent with a microscopic thymoma (type A).

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.

We are preparing to send our hospitals a reminder that the behavior changes from 2 to 3 at the bottom of the basement membrane, and the T category changes from Tis to T1 at the bottom of the mucosa for colon and rectum carcinomas. We are confused by the wording of the Exception.

Distinguishing In Situ and Localized Tumors for the Digestive System

1.b. If the tumor has penetrated the basement membrane to invade the lamina propria, in which case it is localized and assigned Summary Stage 1 (localized) and for invasion of the lamina propria

Exception: Code 0 (behavior code 2) includes cancer cells confined within the glandular basement membrane (intraepithelial); includes in situ plus intramucosal carcinoma (involvement of the lamina propria and may involve but not penetrate through the muscularis mucosa) (penetration through the muscularis mucosa is behavior code 3.)

The text following (intraepithelial) is unclear. The question is: Does the text include in situ plus intramucosal carcinoma (involvement of the lamina propria and may involve but not penetrate through the muscularis mucosa) (penetration through the muscularis mucosa is behavior code 3.) mean the following:

Code 0 (behavior code 2) includes in situ plus intramucosal carcinoma. In situ plus intramucosal carcinoma is involvement of the lamina propria, which may involve (but not penetrate through) the muscularis mucosae. Penetration through the muscularis mucosa is behavior 3. If that is what the text above means, then it seems that the 2018 SEER Summary Stage Manual is saying colorectal tumors reported as: adenocarcinoma in situ, at least intramucosal adenocarcinoma in situ, high grade dysplasia/intramucosal adenocarcinoma in situ, focally intramucosal at the margin are to be coded behavior 2 and SEER Summary stage In situ (0) like the intraepithelial carcinoma tumors. However, it conflicts with the EOD Data for Colon and Rectum, Note 2, and SINQ 20210006. The text for both EOD Data for Colon and Rectum and SINQ 20210006 is clear. According to them, the above bulleted adenocarcarcinoma examples are coded SEER Summary Stage localized (1) and behavior 3. SINQ 20210006 states that: For purposes of Summary Stage, intramucosal carcinoma is a localized lesion So, intramucosal carcinoma is coded SEER Summary Stage 1 (localized) and (behavior code 3).

According to the text for EOD Primary Tumor, Colon and Rectum, Note 2 below, intramucosal, NOS involvement is invasive.

Note 2: Code 050 (behavior code 3) includes the following:

Intramucosal, NOS

Lamina propria

Mucosa, NOS

Confined to, but not through the muscularis mucosa

Thank you for your help clarifying the 2018 SEER Summary Manual Exception text above.

Patient was diagnosed with lambda myeloma based on the M spike found on serum protein electrophoresis. A bone marrow biopsy was performed, but it was an insufficient sample.

SPEP is not listed in the Hematopoietic Database as a lab test that can be used as a definitive diagnostic method. Since the physician did base the diagnosis on the SPEP result, would it be appropriate to assign code 5 (Positive laboratory test/marker study) since there was no histological confirmation?

Under code 5, the Hematopoietic Manual states: Laboratory tests are listed under Definitive Diagnostic Methods in the Hematopoietic Database.

Since the synoptic report and final diagnosis are equal in priority, and the Solid Tumor Rules tell us to code the more specific histology, would this be coded to signet ring cell adenocarcinoma, 8490/3, even though the pathologist used features in the final diagnosis? There is no histology adenocarcinoma with signet ring cell features on the CAP Protocol, so the pathologist may check off the next closest histology " signet ring cell carcinoma " which would not be truly representative of the actual histology. Final Diagnosis: Proximal colon, segmental resection: Invasive adenocarcinoma, poorly differentiated, with signet ring cell features. Synoptic Report A: Colon and Rectum - Resection Specimen Procedure: Right hemicolectomy, Tumor Site: Right (ascending) colon, Histologic Type: Signet-ring cell carcinoma, Histologic Grade: G3: Poorly differentiated.