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Rule PH28 confirms the more specific histologies are ignored if this is truly a low grade B-cell lymphoma (i.e., non-Hodgkin lymphoma, NOS) since both MALT lymphoma and LPL are more specific types of low grade B-cell lymphomas. This leaves only a diagnosis of low grade B-cell lymphoma with plasmacytic differentiation to consider.

SINQ 20130033 states a low grade B-cell lymphoma with plasmacytic differentiation should be coded as 9680/3 (diffuse large B-cell lymphoma (DLBCL)). However, DLBCL is a high grade B-cell lymphoma, not a low grade B-cell lymphoma.

If the pathologist classifies this as a non-specific low grade B-cell lymphoma, and clarifies that this may represent a more specific type of low grade B-cell lymphoma (MALT lymphoma or LPL), should the histology be coded to a high-grade lymphoma (DLBCL) or non-Hodgkin lymphoma, NOS?

Example: Left breast total mastectomy final diagnosis is incidental microscopic findings suspicious for early Paget disease of the nipple. The diagnosis comment states: The left breast mastectomy shows mildly atypical cells within the nipple epidermis which are suspicious for early Paget disease of the nipple. Additional sampling of the left breast was performed, and no evidence of atypical hyperplasia, in situ carcinoma, or invasive carcinoma within the left breast tissue was identified.

Would this case be non-reportable using rationale similar to an early/evolving melanoma per SINQ 20180029?

IMPRESSION/PLAN: Patient is a previously healthy middle aged woman with a diagnosis of adenocarcinoma of the rectum, clinical stage II (T3N0M0). We will proceed with a neoadjuvant course of radiation and concurrent chemotherapy (5-FU) to maximize local regional control and survival, and hopefully facilitate a sphincter-sparing resection in the future. The primary tumor and the pelvic nodes at risk will receive 4500 cGy delivered over 25 treatments. The primary tumor will subsequently receive an additional 1080 cGy delivered over 5 treatments, for a cumulative dose of 580 cGy.

PATHOLOGY: Adenocarcinoma of the rectum, clinical stage II (T3N0M0). The patient is referred by (dr) for a neoadjuvant course of chemoradiotherapy.

HPI: Patient presented recently with rectal bleeding and a change in bowel habits. Colonoscopy revealed an ulcerated mass located 4.0 cm above the anal verge. A biopsy was positive for invasive well-differentiated adenocarcinoma that arose from a tubular adenoma. A staging work-up demonstrated no evidence of metastatic disease.

Example: Right breast core biopsy found ductal carcinoma in situ in the upper outer quadrant. Subsequent resection has a final diagnosis of invasive mucinous carcinoma, grade 1, measuring approximately 7 mm, with close margins. See staging summary. Gross description mentions only the primary tumor with associated marker clip from previous biopsy.

Breast Cancer Staging Summary lists (testing and margins removed for brevity):

Procedure type: Lumpectomy.

Specimen laterality: Right.

Tumor size: 7mm.

Histologic type: Invasive mucinous carcinoma.

Histologic grade (Nottingham histologic score): Grade 1, (score 5/9).

Tumor focality: Single focus.

Lymph-vascular invasion: Not identified.

Treatment effect: No known therapy.

Ductal carcinoma in situ (DCIS): Present.

Architectural pattern: Cribriform.

Nuclear grade: Grade 1.

Necrosis: Not identified.

Calcifications: Not identified.

Estimated size/extent of DCIS: Spanning an area measuring 15mm.

Pathologic stage: pT1b, pNx. (AJCC 8th ed).

Distant metastasis: Not applicable.

Additional findings: Background lobular carcinoma in situ (LCIS), flat epithelial atypia (FEA), and atypical ductal hyperplasia (ADH).

Table 3 (Specific Histologies, NOS, and Subtypes/Variants) lists Ewing sarcoma as a synonym for Peripheral primitive neuroectodermal tumor 9364. Presumably, this is to be used for the reportable malignant peripheral nerve tumors when diagnosed as pPNET or Ewing sarcoma. However, this patient has a type of central (or CNS) primitive neuroectodermal tumor (histology 9473). Table 3 does not list central primitive neuroectodermal tumor (PNET or CPNET) as a valid histology for CNS tumors.

While Table 3 does not list all the possible histologies for the CNS, it currently is not clear how one would arrive at the histology code for a CNS Ewing sarcoma family tumor with CIC alteration, as this is recognized as a new entity for primitive neuroectodermal tumors of the CNS (i.e., PNET, histology 9473) per multiple journal articles. Ewing sarcoma family tumors include both peripheral PNET and central PNET tumors, but to code this histology as a peripheral PNET (9364) in this case seems incorrect when the primary tumor is stated to be of central nervous system origin, not peripheral nervous system origin.

Example: Mastectomy specimen final diagnosis shows two foci of invasive ductal carcinoma including: Invasive ductal carcinoma, no special type, in association with solid papillary carcinoma (tumor #1, 1 cm, slices 6 and 7) and invasive ductal carcinoma, no special type (tumor #2, 1.2 cm, slices 9 and 10).

Summary Staging outlines, Tumor #1: Histologic Type: Invasive ductal carcinoma, no special type, in association with solid papillary carcinoma. As well as, Tumor #2: Histologic type: Invasive ductal carcinoma, no special type. Additional findings include ductal carcinoma in situ (DCIS): presently approximately 3.3 cm, spanning slices 10-13.

The behavior of the solid papillary carcinoma component will affect the provisional histology of the first tumor (8523/3) per Rule H17 vs. 8500/3 per Rule H7). Based on the response, we can determine whether this represents a single or multiple primaries (single primary per M13 vs. multiple primaries per M14).

The patient is a 34 year old who presented with testicular pain radiating into the abdomen approximately 1 month before orchiectomy in 2018. CT abdomen/pelvis: Multiple focal sclerotic bone lesions. Given the lack of change from July 2014, these are likely benign bone islands. No adenopathy mentioned. He has no prior history of germ cell tumor nor any surgery for any tumor/cancer before this. Pathology: Testis, left, radical orchiectomy: - Demarcated scar tissue (1.3 cm), with atrophic seminiferous tubules and cortical bone consistent with burnt-out germ cell tumor. No evidence of germ cell neoplasia in situ (GCNIS). - Margins are unremarkable.

Core biopsy of left breast at 2:00: Invasive ductal carcinoma, Nottingham score 6/9.

Core biopsy of left breast at 4:00: Invasive mucinous carcinoma (variant of ductal carcinoma), Nottingham score 5/9.

Post neo-adjuvant mastectomy: Main (largest tumor): Invasive ductal carcinoma, upper outer quadrant grade 2. Secondary tumor: mucinous carcinoma, grade 1 at 4:00.