MP/H Rules/Histology--Kidney: How is the histology coded and what rule(s) apply to the classification of succinate dehydrogenase-deficient renal cell carcinoma? See Discussion.
Partial nephrectomy showed carcinoma, histologic type: succinate dehydrogenase-deficient renal cell carcinoma. This is not a term in the ICD-O, and is not a histology covered in the Kidney MPH rules. However, a recent web search indicates this is a specific type of RCC that was added to the 2016 WHO classification of RCC (per abstract: https://www.ncbi.nlm.nih.gov/pubmed/27179267) and makes up 0.05-0.2% of RCC cases (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4229399/).
Code the histology to renal cell carcinoma, NOS (8312/3). While WHO lists succinate dehydrogenase-deficient renal cell carcinoma in the latest edition, no specific histology code is provided. MP/H Rule H10 applies since only one histology type is provided, though no code is listed.
First course treatment/Chemotherapy: Is metronomic chemotherapy coded as chemotherapy?
Code metronomic chemotherapy as chemotherapy. Metronomic chemotherapy, also referred to as low-dose metronomic (LDM) chemotherapy, is an emerging cancer treatment approach which administers relatively low doses of traditional chemotherapy drugs over a long period of time and without ‘breaks’ in treatment. By using lower doses this method of treatment minimizes the side effects of traditional chemotherapy.
First course treatment/Radiation Therapy--Prostate: How do you code fiducial markers for prostate cases?
Do not code fiducial markers as a form of radiation treatment; rather, code the radiation therapy in the radiation treatment section. Fiducial markers are small metal spheres, coils, or cylinders that are placed in or near a tumor to help guide the placement of radiation beams during treatment.
First course treatment/Immunotherapy--Prostate: Is XGEVA, given for bone mets from prostate cancer, abstracted as immunotherapy, or is it an ancillary drug and not recorded?
Do not record XGEVA when given for bone mets from prostate cancer. See SEER*Rx for more information.
MP/H Rules/Histology--Brain and CNS: What is the histology code for a tumor originating in the cerebellum and extending into the fourth venrticle described as a glioblastoma with primitive neuroectodermal tumor component (WHO Grade IV)?
The WHO Classification of CNS tumours lists glioblastoma with primitive neuroectodermal tumor component as a subtype of glioblastoma and assigns 9440/3. Also referred to as glioblastoma with a primitive neuronal component.
MP/H Rules/Histology--Breast: What histology code(s) and MP/H rule applies for a breast resection final diagnosis of "undifferentiated sarcoma associated with a malignant phyllodes tumor and a tumor size of approximately 7 x 6.5 x 4 cm"? (The tumor is primarily sarcoma, with the phyllodes tumor measuring 2.8 cm)? See Discussion.
Patient has a diagnosis of undifferentiated sarcoma with an associated malignant phyllodes tumor in a single mass. Should this be abstracted as two primaries, one for an undifferentiated sarcoma and the other for a malignant phyllodes tumor? Which MP/H rule applies?
Abstract a single primary. Based on the information provided, this is a single tumor, and therefore a single primary, Rule M3. Code the histology to malignant phyllodes tumor.
According to our expert pathologist consultant, "The presence of a phyllodes tumor component identifies the whole thing as such. Stromal overgrowth/sarcoma is the usual identifier of malignancy in a phyllodes tumor. (If there were no phyllodes component we would be left with undifferentiated sarcoma, but that is not the case here. The diagnosis of malignancy in phyllodes tumor may be difficult/problematic when there is no overt stromal/sarcoma overgrowth as in this case.) As an aside, the behaviors of pure sarcoma and a phyllodes tumor such as we have here are similar, but we would lose the primary diagnosis if we just called this sarcoma."
MP/H Rules/Histology--Breast: How should histology be coded for a breast primary with resection final diagnosis of "Ductal carcinoma with neuroendocrine features?" See Discussion.
Should the histology for "Ductal carcinoma with neuroendocrine features" be coded to 8500 (Ductal carcinoma, NOS) or 8574 (Adenocarcinoma with neuroendocrine differentiation)?
Code the histology to 8574/3 for Ductal carcinoma with neuroendocrine features.
Ductal carcinoma is also called "invasive breast carcinoma of no special type." WHO classifies Invasive breast carcinoma with neuroendocrine differentiation as 8574/3.
MP/H Rules/Multiple primaries/Histology: What histology and how many primaries are coded for a mixed germ cell tumor with a somatic type malignancy (rhabdomysarcoma) if the patient was diagnosed with seminoma of the testis in 2009 followed by a 2015 metastatic germ cell tumor in a retroperitoneal lymph node, stated to be a recurrence of the testicular cancer? See Discussion.
In September 2009 the patient was diagnosed with seminoma, classical type, following an orchiectomy. Testicular mass recurrence in 2014 was treated with chemotherapy.
Then in April 2015 a retroperitoneal dissection of a peri-aortic LN was positive for mixed germ cell tumor with somatic type malignancy (rhabdomyosarcoma) involving 1/11 nodes. Path Comment: major component of tumor is teratoma, rhabdomyosarcoma represents <5% of mass.
Now in October 2016, the patient has a retroperitoneal mass biopsy positive for spindle cell sarcoma with rhabdomyosarcomatous differentiation. The comment section of the pathology report states, "Given the history of a germ cell tumor w/ rhadbomosarcomatous component, the findings are consistent with a recurrence of rhabdomyosarcomatous component of germ cell tumor."
Can a seminoma transform to a mixed germ cell tumor with a somatic type malignancy (see SINQ 20140082 - testicular teratoma with somatic type malignancy)?
According to our expert pathologist consultant, yes, seminoma could transform to a mixed germ cell tumor with a somatic type malignancy. He advises us to code this case as 9061/3.
From our expert pathologist consultant: This occurs as "reprogramming" of the initial germ cell tumor/seminoma cell. The process is not understood, but genetic studies support this progression concept. Most often the next step is teratoma. It is out of the teratoma that the somatic malignancy usually comes. I do wonder about the possibility that this was really an embryonal carcinoma which resembles a seminoma - occasionally this can be a difficult separation. I wonder if they radiated the scrotum following the orchiectomy, also, given the scrotal recurrence.
SEER Summary Stage 2000--Melanoma: Can Clark's level classification still used to Summary Stage melanoma? It was previously used by AJCC TNM, but was not included in the 7th edition. I see it is still listed in the CAP protocols for melanoma.
Clark's level can be used to assign in situ, localized or regional summary stage.
If there is a discrepancy between the Clark’s level and the pathologic description of extent, use the higher Summary Stage code.
Primary site/MP/H Rules/Histology: What is the appropriate site and histology code for a tumor described as a "Large mass In suprasellar cistern encroaching into sphenoid & ethmoid sinuses", with the pathology described as "INI-1 deficient sinonasal undifferentiated carcinoma"? Of note, this patient has a history of a pituitary adenoma, resected overseas a few months prior to this diagnosis.
The primary site is unclear. The lesion is intracranial, but this may not be the primary site. In the absence of any additional information, assign C390, 8020/3. According to WHO, sinonasal undifferentiated carcinoma can involve the nasal cavity, maxillary antrum, and/or ethmoid sinus.
SMARCB1 (INI-1) is a tumor-suppressor gene located on chromosome 22q11.2. Tumors that showed loss of expression were SMARCB1-deficient tumors which are characterized by nests, sheets, and cords of cells without any histologic evidence of specific (eg, squamous or glandular) differentiation.
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