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20190081 | Race: How is race coded for a patient who self-reports as white? In the Family History portion of the genetics consult, it states the maternal family is of mixed European and Cherokee descent; the paternal side is of mixed German/mixed European descent. Is race coded as Race 1: 03-American Indian and Race 2: 01-White, or as 01-White according to self-report by the patient? |
Self-reported information is the highest priority for coding race. That is because the race information for the U.S. population comes from census data and that information is self-reported. For national cancer statistics, in order for the numerator (cancer cases) and the denominator (population) to be comparable, use self-reported race information whenever it is available. We will add this clarification to the SEER manual. |
2019 | |
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20190084 | Histology/Heme & Lymphoid Neoplasms: Should the histology be coded to chronic myeloid leukemia (CML), BCR-ABL1-positive (9875/3) regardless of the quantitative analysis percentage of BCR-ABL1 that was detected? See Discussion. |
Example: Bone marrow biopsy diagnosis is chronic myelogenous leukemia, chronic phase, and the RT-PCR test result proved, BCR-ABL1 p210 (Major Breakpoint) - Detected, 3.3659%. Even though the p210 fusion transcript was less than 5%, it was detected. The presence of BCR-ABL1 does define whether or not patients are treated with tyrosine kinase therapies. Therefore, it seems likely that the presence of any BCR-ABL1 would be captured using the more specific histology code 9875/3, instead of the non-specific CML, NOS histology code 9863/3. Are there minimum threshold requirements for these quantitative studies in order to code the histology to the more specific type of CML? |
Code chronic myeloid leukemia (CML) BCR-ABL1-positive as 9875/3. According to the WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues, 4th edition, CML BCR-ABL1-positive is characterized by the chromosomal translocation t(9;22) which results in the formation of the Philadelphia (Ph) chromosome containing the BCR-ABL1 fusion gene. The diagnosis requires detection of the Ph chromosome and/or BCR-ABL1. If the mutation is detected, regardless of percentage, it is positive. Quantitative levels of BCR-ABL are used to monitor response to tyrosine kinase inhibitor therapy. |
2019 |
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20190094 | Reportability/Heme & Lymphoid Neoplasms--Skin: Is elephantiasis nostras verrucosa (ENV) reportable as a lymphoma? See Discussion. |
The autopsy report indicated a diagnosis of: Skin: Hyperkeratosis and pseudoepitheliomatous hyperplasia as well as reactive angioendotheliomatosis indicating Elephantiasis Nostras Verrucosa. |
Elephantiasis nostras verrucosa (ENV) is not reportable. ENV is a rare form of chronic lymphedema caused by any number of conditions including neoplasms, trauma, radiation treatment, congestive heart failure, obesity, hypothyroidism, chronic venous stasis, and parasitic infection. |
2019 |
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20190032 | Summary Stage 2018--Lung: Are ground-glass lung nodules coded as distant for Summary Stage? See Discussion. |
Chest x-ray: Multifocal pneumonia in left lung; possibility of masses in left lung not excluded. Chest CT: 4 large ground-glass masses in LUL (largest 46mm); beginning of Tree-In-Bud appearance in LUL; 2 small ground-glass nodules in right lung. Lung LUL biopsy: Adenocarcinoma, Solid Predominant. No further information as patient did not want to discuss treatment options. Per the AJCC book and CAnswer Forum, multifocal classification should be applied equally whether the lesions are in the same lobe OR in different ipsilateral lobes OR contralateral lobes, cT2b(m), cN0, cM0. |
Do not assume that ground glass presentation is consistent with a neoplasm. There are numerous causes of a ground glass lung condition such as sarcoidosis or pulmonary fibrosis. A ground glass lung opacity may also be observed in conditions such as alveolar proteinosis, desquamative pneumonitis, hypersensitive pneumonitis, and drug-induced or radiation-induced lung disease. If an area of ground glass opacity persists in the lung, it is usually classified as an adenocarcinoma, a classification that ranges from premalignant lesions to invasive disease. This is in line with AJCC that states to stage based on the largest tumor determined to be positive for cancer. To Summary Stage the case example provided, ignore the lesions in the contralateral lung (do not assume that they are malignant). There are multiple lesions in the left lung, but once again, do not assume that those not biopsied are malignant. This leaves us with the lesion confirmed to be malignant, making this a Localized (code 1) tumor. |
2019 |
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20190029 | Reportability--Testis: Is demarcated scar tissue with atrophic seminiferous tubules and cortical bone consistent with burnt-out germ cell tumor and no evidence of germ cell neoplasia in situ (GCNIS) reportable? See Discussion. |
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. |
Burnt-out germ cell tumor (9080/1) is not reportable. According to WHO Classification of Urinary System and Male Genital Organ, regressed germ cell tumors are germ cell tumors that have undergone partial or complete regression leaving a generally well-delineated nodular focus of scar or fibrosis in the testis. |
2019 |
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20190037 | Solid Tumor Rules/Multiple Primaries--Breast: How many primaries should be abstracted for simultaneously diagnosed non-contiguous invasive duct carcinoma and mucinous carcinoma? Does rule M12 apply since the two histologies are on different rows of Table 3 of the Breast Solid Tumor Rules? See Discussion. |
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. |
Abstract multiple primaries when separate, non-contiguous tumors are on different rows in Table 3 of the Breast Solid Tumor Rules. Use Rule M14 as each row in the table reflects a distinctly different histology, in this case, invasive ductal carcinoma (8500) and mucinous carcinoma (8480). |
2019 |
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20190005 | Primary Site--Bladder: Does instruction #4 in the Urinary Sites Solid Tumor Rules Instructions for Coding Primary Site apply to a mix of in situ and invasive urothelial tumors? Instruction #4: Code Urinary System NOS C689 when there are multiple non-contiguous tumors in multiple organs within the urinary system. See Discussion. |
Example: Patient has multiple biopsies with final diagnosis of in situ papillary urothelial carcinoma in the prostatic urethra and invasive papillary urothelial carcinoma in the bladder. How should primary site be coded in this type of mixed in situ and invasive situation? |
Code Urinary System NOS C689 for this case since there are two separate urinary sites involved. Apply instruction #4 when there is a mix of in situ and invasive urothelial tumors. |
2019 |
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20190011 | Reportability--Skin: Is an atypical smooth muscle cell proliferation of the skin reportable? See Discussion. |
Example: Patient has left thigh skin excision with final diagnosis of atypical smooth muscle cell proliferation, inked peripheral margin is involved and inked deep margin is free of disease in the sections examined. See Comment. Diagnosis comment states: The terminology regarding this lesion is controversial. Lesions with identical features are designated as leiomyosarcoma in the dermatopathology literature, whereas, the preferred classification in the soft tissue pathology is atypical intradermal smooth muscle neoplasm. Although the lesion appears predominantly dermal based, since the margin is involved, the lesion cannot be entirely evaluated, and therefore the final designation is deferred to the findings in the excisional specimen. (This slide was read by bone and soft tissue pathologist.) There has been no excision of this tumor and, as a central registry, we have no access to the pathologist for clarification. Is this skin case reportable based on the dermatopathology interpretation when further documentation is not available? |
Since you do not have the option of checking with the pathologist and no further information is available, do not report this case. The diagnosis is atypical smooth muscle cell proliferation of the skin, which is not reportable. Registrars with access to the pathologist should querry the pathologist for clarification in this situation. |
2019 |
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20190053 | Solid Tumor Rules (2018)/Histology--Brain and CNS: What is the histology code for a central nervous system (CNS) Ewing sarcoma family tumor with CIC alteration of the right parietal lobe? See Discussion. |
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. |
Code as 9364/3. WHO Classification of Tumors of the CNS, 4th edition, refers to Ewing sarcoma/peripheral primitive neuroectodermal tumor as a tumor of neuroectodermal origin involving the CNS either as a primary dural neoplasm or by direct extension from contiguous bone or soft tissues (such as skull, vertebra, or paraspinal soft tissue). |
2019 |
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20190054 | Update to current manual/Solid Tumor Rules (2018)/Histology--Brain and CNS: Table 6 (Non-Malignant CNS Equivalent Terms and Definitions) lists as a subtype/variant of craniopharyngioma 9350/1. This is not a valid histology per the ICD-O-3 or the 2018 ICD-O-3 Update Table. Is this actually supposed to read, ? |
Adamantinomatous craniopharyngioma (9351/1) is a subtype of craniopharygioma. We will correct the Non-Malignant CNS Solid Tumor Rules in the next update. |
2019 |
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