SEER Inquiry System - Search

2022 case: Surgical Pathology Report-spinal cord tumor, biopsies: ALK-positive neoplasm most consistent with ALK-positive histiocytosis.

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.

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.

Is a physician’s statement of progressive disease adequate to determine disease progression in coding first vs. second course treatment? Can an increase in tumor burden (i.e., a change in overall stage) be used by the registrar to determine disease progression?

Often, determining disease progression is difficult as there are no guidelines in the SEER Manual related to this topic. It seems a physician’s statement of progressive disease should always be accepted. However, that statement is not always available. While it seems an increase in tumor size alone would not be “progressive disease” as tumors will continue to grow, can registrars use an increase in tumor burden to make this determination?

The instructions for coding first vs. second course treatment are clear when a treatment plan is changed, but determining whether there has been disease progression, recurrence, or treatment failure can be difficult without a physician’s assessment.

For example, a patient was diagnosed with a newly diagnosed resectable pancreatic cancer; the documented treatment plan was for upfront chemotherapy, followed by repeat staging, followed by pancreatectomy. The patient completed 3 cycles of FOLFIRINOX, but the physician noted that the CT scan shows progressive disease, and the plan was to start a new treatment regimen with Abraxane, Gemzar, and stereotactic body radiation (SBRT) (Cyberknife). The patient completed the additional chemotherapy, radiation, and proceeded to the initially planned surgery. The pathologist staged this as yp disease, but the surgery appears to be second course treatment, and we would not code the surgery, or collect the staging (yp staging) since the physician stated this was progressive disease. The classification as yp staging can be misleading, since the resection is technically after neoadjuvant treatment, but is not collected per our guidelines. In this case, is it correct to code first course treatment as FOLFIRINOX only?

Examples:

Bladder TURB:  Invasive high grade urothelial carcinoma with poorly differentiated (40%), lipoid (5%), and sarcomatoid (55%) components.

Bladder tumor base TURB:  Invasive high grade urothelial carcinoma with poorly differentiated (65%) and sarcomatoid (30%) components.

The Urinary Sites Solid Tumor Rules, histology coding rules, say to code the most specific histology or subtype/variant, regardless of whether it is described as majority, minority, or component.  Poorly differentiated (8020) and sarcomatoid (8122) are both urothelial subtypes, but there is no rule to instruct how to code a tumor/tumors with multiple urothelial subtypes.

11/16/20:  Patient diagnosed with endometrial cancer on by MRI of the pelvis; 11.5 cm uterine mass consistent with cancer with no lymphadenopathy.

1/6/21:  Patient had a total abdominal hysterectomy/bilateral salpingo-oophorectomy and pelvic lymph node dissection. Operative report stated patient had mildly enlarged bilateral pelvic nodes.

Path report:  Endometrioid adenocarcinoma with invasion of the serosa. Five bilateral pelvic nodes were sampled and negative. Originally, staging had patient as node negative.

1/22/21:  Patient had post op imaging done that showed metastatic retroperitoneal, aortocaval, and possibly left iliac lymph nodes. Physician changed staging to include the lymph node involvement.

The pathology report states this is a rare entity described in case reports and not incorporated into the WHO classification of tumors. A subsequent endocrinology note stated “papillary tumor, benign by path; tumor was not an adenoma; based on one Mayo study, the recurrence risk is low.”