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Malignant Central Nervous System (CNS) Rule M5 instructs us to abstract a single primary (as malignant) when a single tumor is originally diagnosed as non-malignant, the “First course treatment was active surveillance (no tumor resection),” and the subsequent resection pathology is malignant.

This patient’s first course of treatment was not active surveillance. While the patient did not have first course tumor resection, the tumor was treated with Cabergoline.

Should Rule M5 apply because there was no tumor resection? If so, should Rule M5 clearly state no tumor resection is the criteria (not active surveillance)?

SINQ 20230023 does indicate a PitNET diagnosis following a diagnosis of pituitary adenoma does not fall into standard rules, but in the previous SINQ the first course treatment was a partial resection.

It is unclear whether other types of treatment could result in a new malignant PitNET, following a previously treated non-malignant pituitary tumor.

Rectal polyp excised in June, 2012, found to have adenocarcinoma in situ in a tubulovillous adenoma. Additional colorectal biopsies in November; all were negative. Another rectal polyp removed in December 2012 showing a tubulovillous adenoma with focal carcinoma in situ. Then, in February, 2013 another rectal polyp removed. This was diagnosed as mod. diff. adenocarcinoma with mucinous features, infiltrating into submucosa, seen in a background of tubulovillous adenoma. Surgical margins free (mucin %=40%). Finally, in May, 2013, a low anterior resection with no residual adenocarcinoma.

This appears to be adenocarcinoma in multiple adenomatous polyps (8221/3), although the final path from May 2013 described one benign polyp and said, 'no other masses, suspicious lesions or polyps are identified.' Going through the MP/H rules, both M13 and M14 result in this being a single primary, and come before the rule about an invasive tumor following an in situ tumor more than 60 days later is a new primary. The original abstract was coded C209 and 8263/2. If this is a single primary, should it be changed to 8221 with a behavior code of 3? Is this scenario another example of when to change the original diagnosis based on subsequent information?

Scenario: Patient had a transurethral resection of the prostate on 8-29-19, positive for basal cell carcinoma with focal squamous differentiation involving approximately 50% of tissue (determined not to be mets by consult). On 11-14-19, the patient had a prostatectomy positive for residual basal cell carcinoma and a small focus of infiltrating prostatic adenocarcinoma. According to AJCC, 8th edition, page 724, basal cell carcinoma of the prostate is 8147/3 and we ignored the small focus of adenocarcinoma. The above scenario was reported as two primaries (8090/3 and 8140/3), but we are thinking it is one.

Disease History

2018 - Lymphomatoid papulosis (non-reportable)

2020 - Transform to CTCL (and called Mycosis Fungoides specifically) (CTCL/MF same primary)

2021 - Transform to CTCL with large cell transformation

7/25/17

Part A: Left breast at 8:00, 5 CFN: Specimen type: Stereotactic biopsy. Tumor type: Ductal carcinoma in situ (DCIS), cribriform type. Tumor size: The largest focus of DCIS measures 1 mm in greatest dimension as measured on the slide. Nuclear grade: 2 (Intermediate grade). Microcalcifications: Present. Other findings: Stromal fibrosis, microcalcification and fat necrosis.

11/1/17

A. Sentinel lymph node, left: One lymph node, negative for metastatic tumor on three levels of routine H\T\E and pan cytokeratin immunohistochemical stains.

B. Left breast: Procedure: Total mastectomy with skin and nipple. Specimen Laterality: Left. Lymph Node Sampling: Yes, portion A. Specimen Integrity: Intact. Histologic Type: Extensive ductal carcinoma in situ and one focus of Invasive ductal carcinoma with mucinous features. Histologic Grade (Nottingham Histologic Score): Glandular Differentiation: Score 3 Nuclear Grade: Score 2. Mitotic Count: Score 1. Total Nottingham score 6 (grade 2, moderately differentiated). Tumor Size: 3.3 x 2 mm (0.33 x 0.2 cm) measured on slide (B3). Tumor Site: Lower inner quadrant of left breast. Tumor Focality: Unifocal. Ductal Carcinoma In Situ (DCIS): Present, cribriform, solid and micropapillary types with focal necrosis and calcifications. Size of DCIS: Number of blocks examined: Thirty (30). Number of blocks with DCIS: Thirteen (13). Lobular Carcinoma In Situ (LCIS): Not identified, Lymphovascular Invasion: Present. Perineural Invasion: Not identified. Other Findings: Changes consistent with previous biopsy site. Cysts, foci of atypical ductal hyperplasia, focal ductal hyperplasia, adenosis, stromal fibrosis and microcalcifications. Skin (epidermis): Uninvolved. Nipple: Uninvolved. Margins: 1 mm from DCIS to the closest deep margin (slide B12). At least 10 mm (1 cm) from invasive carcinoma to deep margin. Estrogen receptor (ER, clone 1D5) by immunohistochemistry performed on this material: Positive (invasive and in situ carcinoma), high intensity, in greater than 95% of carcinoma cells. Progesterone receptor (PR, clone 16) by immunohistochemistry performed on this material: Positive (invasive and in situ carcinoma), moderate intensity in about 80% of the carcinoma cells. Her 2 by FISH performed on this material: Pending, an addendum to follow. Pathologic staging: pT1aN0(sn)MX (AJCC 7th edition). Dictated by: (Pathologist), MD Intradepartmental review.

The patient has a history of a minimally invasive endometrial adenocarcinoma that was low grade and confined to an endometrial polyp in 2001. The patient underwent a total abdominal hysterectomy/bilateral salpingo-oophorectomy (TAH/BSO) that entirely removed the tumor at that time.

Almost 18 years later, the patient had a left inguinal mass excision that was, Carcinoma of gynecologic origin, consistent with clear cell carcinoma. No other disease was found, the physician never indicated whether this was felt to be metastatic from the previous, low grade adenocarcinoma or not. It was only noted as, an unusual malignancy of the left lower quadrant and inguinal region of gynecologic origin. No further information was available in the medical record or from the physician on follow-up.

Although neither the Solid Tumor Rules nor the MPH Rules (still in use for the Other Sites schema) apply to metastasis, given the differences in histology and behavior of these two tumors (i.e., minimally invasive, low grade disease diagnosed in 2001 vs. higher grade, more aggressive tumor in 2019) should the current clear cell carcinoma of GYN origin really be the same primary as the 2001 endometrial adenocarcinoma?

The 2007 SEER Manual (page 3) states that cases diagnosed clinically are reportable. Exception 2 states if enough time has passed that it is reasonable to assume the physician has seen the negative pathology report, but the clinician continues to call this a reportable disease, accession the case.

SEER reporting guidelines state that severe dysplasia is not reportable, however, many clinicians regard it to be equivalent to carcinoma in situ.

Example 1: In 09-2007 the pathology report for excisional biopsy of right floor of mouth states the final diagnosis is severe dysplasia. At the time, the case is not accessioned based on non-reportable pathology. Patient is subsequently admitted in 3-09. According to the clinical history the patient was diagnosed with squamous cell carcinoma in 2007 and treated with laser. Is this reportable? If yes, how is behavior to be coded? How is "Ambiguous Terminology at Diagnosis" to be coded?

Example 2: In 2-08, the pathology report for a punch biopsy of a skin lesion states the final diagnosis is atypical melanocytic hyperplasia. In 3-08, patient is admitted for re-excision. The clinical diagnosis states re-excision being done for melanoma in situ.

Reference: SINQ 20061123

Polycythemia, NOS is not reportable. However, there is a statement in the Heme Manual Glossary for JAK2 that states, "When JAK2 is positive, the MPN is definitely reportable." Does a positive JAK 2 always mean there is a reportable myeloproliferative disorder or must there also be an associated statement of a reportable neoplasm (e.g., myeloproliferative disorder, polycythemia vera, or essential thrombocythemia)?