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March 2022, left shoulder soft tissue mass excision shows a spindle cell tumor with outside consultation diagnosis of malignant neoplasm with neuroectodermal differentiation and TPR-NTRK1 gene rearrangement. Diagnosis comments indicate the findings most closely resemble the spectrum of kinase-rearranged mesenchymal neoplasms, such as lipofibromatosis-like neural tumor. However, the expression of SOX10 and mature melanocytic markers is unusual, and does not exclude melanocytic differentiation.

Should this be classified as a peripheral neuroectodermal tumor (9364) or as an "NTRK-rearranged spindle cell neoplasm (emerging)" (8990) if there is a NTRK gene rearrangement?

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)?

History provided by the oncologist

Right neck mass since 2019; 04/07/20, initial biopsy p16-negative SCC, delay of treatment due to patient preference, agreed to biopsy of tonsil and work-up August 2022; right tonsil biopsy: p16-positive, G2 SCC, nodal mass at that time >6 cm with extensive extranodal extension, Stage III (cT2, cN3, cM0, p16-positive); based on this history, was staged as a tonsil primary and p16-positive.

Patient details

1. March 2020, CT neck and chest revealed a 0.5 x 2.7 x 2.3 cm low-density necrotic nodal mass at right neck level 2 suspicious for metastatic disease.  There was a slight asymmetric increased size of the right palatine tonsil.  There are a few sub-4 mm pulmonary nodules which are nonspecific.

2.  April 7, 2020, FNA of right neck mass with pathology revealed p16-negative SCC   

3.  April 20, 2020, PET/CT revealed 3 x 2 cm right-sided level 2 node with FDG avidity  

4.  May 5, 2020, flexible laryngoscopy showed no obvious primary lesion   

5.  May 2020, after evaluation by a medical oncology, patient declined any treatment  

6.  June 17, 2022, return visit in medical oncology after PET/CT demonstrates significant progression in the neck; patient definitively declines chemo, but would like surgical opinion.  Now has more rapidly progressive disease with skin breakdown and weeping from malignant lesion right neck.  

7.  June 22, 2022, radiation oncology consultation   

8.  July 15, 2022, tonsil biopsy: Invasive squamous cell carcinoma, moderately differentiated with LVI, p16-positive  

9.  Patient now agreeing to treatment with radiation: Tooth extractions 8/30/2022, radiation planning 9/14/2022  

10.  Patient consulted with cancer specialist who explained surgery is not recommended given level of extranodal extension and risk of seventh cranial nerve paralysis and fistula formation with surgical excision and who recommended chemoradiation

11.  September 9, 2022, patient presented for radiation CT simulation/treatment planning and informs treatment team. Patient declined/refuses concurrent chemotherapy despite recommendations from two cancer institutions.

Laterality is often not noted for these sella turcica meningiomas; therefore, Laterality is often coded as 9 (Unknown). Because the sella turcica appears to be a midline structure in the base of the skull, is Laterality code 5 (midline) more appropriate when additional information is unavailable?

A common scenario is a patient has a positive CT of the abdomen/pelvis for liver mass only. Biopsy of the liver mass is positive for cholangiocarcinoma. The physician is also calling the liver tumor the primary site with histology of cholangiocarcinoma. There is no evidence of intrahepatic bile duct (C221) or gallbladder (C240) involvement which are sites specific to this histology. The hematology/oncology consult stages this as Stage IIIA, T3N0M0 intrahepatic cholangiocarcinoma.  Can we code cholangiocarcinoma with site code C220 (liver) or should we assume that C221 (intrahepatic bile ducts) would be a better code to reflect this histology?  

The patient underwent a resection of the transitional meningioma in 2012, but residual tumor was left behind. The patient was on surveillance until imaging showed growth of the residual tumor. The resection in 2022 proved atypical meningioma.

Rule M2, the first rule that applies, indicates this situation represents a single primary (a single tumor). However, Rule M4 states the transformation from a benign meningioma to a borderline meningioma would only be a single primary if the meningioma was a NOS.

This patient has microscopic confirmation of a meningioma showing different subtypes/variants (listed in Column 3, Table 6). Should this be accessioned as multiple primaries based on the transformation and distinctly different histologies?

Patient had a partial nephrectomy with final diagnosis of papillary renal neoplasm with reverse polarity.  Diagnosis comment states: Papillary renal neoplasm with reverse polarity is currently considered to be a histologic variant of papillary renal cell carcinoma; however, recent studies suggest that it has a very indolent clinical behavior.

Example:  Patient scheduled for left nephrectomy on 3/10/20 due to left renal papillary renal cell carcinoma diagnosed on 2/11/20 via needle core biopsy. Abstract indicated surgery was cancelled due to the pandemic. Abstract also indicated the surgery was not rescheduled.