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The patient has adenopathy in multiple lymph node regions above and below the diaphragm and a lymph node biopsy pathology proved CLL/small lymphocytic lymphoma (SLL). Further work-up with peripheral blood proved an abnormal CD5-positive B-cell population comprising only a small percentage of the white blood cells (WBCs). The pathologist noted this neoplastic B-cell population comprises “3.5% of white blood cells and has an immunophenotype characteristic of CLL/SLL and is similar to the recent lymph node biopsy in this patient.” The managing physician indicated this was a Lugano Stage III SLL. The registrar coded the peripheral blood involvement in EOD Primary Tumor.

If this small percentage of WBCs with an abnormal B-cell population is included in EOD Primary Tumor as peripheral blood involvement, then this would indicate peripheral blood/bone marrow involvement and primary site would need to be coded to C421 per Rule PH5. Rules PH5 and PH6 confirm primary site must be coded C421 if peripheral blood or bone marrow are involved.

Is there a cutoff value for these abnormal B-cell populations in the peripheral blood? Or should these abnormal B-cell populations be ignored unless the pathologist states the abnormal B-cell population is consistent with CLL/SLL (not just immunophenotypically characteristic of CLL/SLL)?

The patient has adenopathy and a lymph node biopsy proved CLL/SLL. The patient underwent a peripheral blood smear, but the final diagnosis only indicated there is an abnormal CLL panel, positive for monoallelic or biallelic deletion of 13q. The pathologist noted a CLL related clone was detected, but there was no definitive diagnosis of CLL on the peripheral blood. No bone marrow biopsy was performed. However, the managing physician noted this was Rai Stage I CLL/SLL with adenopathy in the neck.

The SSDI Manual notes, “Rai stage is only applicable for CLL, in which the bone marrow and/or peripheral blood are involved (primary site C421 for bone marrow, see Hematopoietic Manual, Module 3: PH 5, 6).” Should primary site default to C421 if the physician provides a Rai Stage in the absence of definitive peripheral blood or bone marrow involvement documented in the medical record?

The patient has a history of refractory anemia with excess blasts diagnosed in 2008. A vocal cord biopsy performed on 6/2/2010 stated, "in view of a previous history of myelodysplastic syndrome this is indicative of transformation to acute leukemia" and consistent with granulocytic sarcoma. A bone marrow biopsy done on 7/19/2010 stated this was compatible with refractory anemia with excess blasts in transformation.

Granulocytic sarcoma is a solid manifestation of AML. When these diagnoses occur more than 21 days apart, are they separate primaries?

According to the WHO definition, this is acute myeloid leukemia complicating myelodysplasia. Which rule applies for this case?

The patient has a history of prostate adenocarcinoma with lymph node metastases, status post prostatectomy and treatment by Lupron in 2014. The most recent prostate serum antigen measurement (April 2018) was normal. CT scan of the abdomen and pelvis revealed new hypodense liver lesions, a slightly enlarging lung right lower lobe nodule, and enlarging lobular mass in the prostatectomy bed. The core liver biopsy contains areas of metastatic tumor with a differential diagnosis on pathology of high-grade neuroendocrine carcinoma of the prostate (small cell type), which may have been seen in association with prostate adenocarcinoma, or metastatic small cell carcinoma of a different site.

Clinically, the physician impression is that this represents metastatic castration-resistant prostate cancer. The Solid Tumor Rules note that the Multiple Primary Rules are not used for tumor(s) described as metastases. However, SINQ 20130221 indicates that, at least historically, these would have been accessioned as multiple primaries (histology 8140 & 8041 per Rule M10). Does the previous SINQ note still apply to these types of cases, and if so how would one know to move beyond the initial note indicating metastases are not new primaries?

The patient has a history of ganglioglioma, WHO grade I, involving the deep right parietal lobe diagnosed on resection in 07/2012. Tumor recurrence in 2017 was treated with radiation.

The patient then had right temporal tumor biopsy and resection 06/2021 with final diagnosis of anaplastic glioneuronal tumor, BRAF p.V600E mutant, WHO Grade III. Pathologist notes that the tumor demonstrates a ganglioglioma with frequent mitoses and possible vascular proliferation. Subsequent consult findings support an anaplastic glioneuronal tumor, compatible with progression of the patient's ganglioglioma that is post-irradiation. However, the pleomorphic and epithelioid areas are also reminiscent of pleomorphic xanthoastrocytoma, which may occur in combination with ganglion cell components. There is no related SINQ to code this histology.

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 patient has a history of CLL/SLL dating back to 2007, but has had progressive disease with development of a new left frontal brain tumor. The brain tumor resection proved CLL/SLL with large cell transformation, but neither the pathologist nor the managing physician called this a Richter transformation, Richter syndrome or provided a diagnosis of DLBCL. However, a large cell transformation of CLL/SLL is a Richter transformation. Can this be accessioned as a new acute neoplasm per Rule M10?

The patient has a 6 cm tumor arising in posterior-lateral bladder extending to prostate, obliterates seminal vesicle, and invades pelvic wall.

TURB final diagnosis: Small cell neuroendocrine carcinoma. High grade urothelial carcinoma involves 10% of tumor.

Following the current MP/H single tumor rules, it appears Rule H8 applies. Per Rule H8, code the numerically higher code of 8120. By following this rule, it does not seem the histology code fairly represents this tumor.

The patient had residual tumor following the 2018 transsphenoidal resection and underwent an additional surgery after the residual tumor increased in size. Since pituitary adenoma/pituitary neuroendocrine tumor (PitNET) is a new malignant neoplasm for cases diagnosed 2023 and later, should this be a new primary per M5? Or do we disregard the change in behavior and apply rule M2 (single tumor is a single primary) for this scenario?

The patient had neoadjuvant chemotherapy for rectal carcinoma. An AP resection revealed an incidental second primary intramucosal carcinoma in adenomatous polyp in the descending colon. Is the chemotherapy coded as therapy for the intramucosal carcinoma of the descending colon?