MP/H/Histology--Sarcoma: How should Ewing Sarcoma/primitive neuroectodermal tumor (PNET) be coded for a 2012 case? See Discussion.
SEER SINQ 20031051 applies to cases diagnosed before 2007 and advises: Code histology as 9260/3, Ewing sarcoma. Ewing sarcoma is a specific histology on the continuum of primitive neuroectodermal tumors. Code Ewing sarcoma as it is more specific than PNET, NOS.
For tumors diagnosed 2007 or later, refer to the MP/H rules.
Apply 2007 MP/H rule H6 and assign the numerically higher ICD-O-3 code that reflects PNET (9364/3).
According to the WHO Tumors of Soft Tissue and Bone, though Ewing sarcoma ICD-O-3 code is 9260/3, Ewing sarcoma with a higher degree of neuroectodermal differentiation present is classically termed peripheral neuroectodermal tumors (PNET). WHO does not offer guidance how to classify tumors stated to be Ewing sarcoma PNET.
Histology code 9364/3 is assigned for a Ewing/PNET that arises outside of the brain/CNS. Peripheral neuroectodermal tumor (PNET) and peripheral primitive neuroectodermal tumor (PPNET) are Ewing family tumors.
Histology code 9473/3 (PNET, primitive neuroectodermal tumor, central primitive neuroectodermal tumor, or supratentorial PNET) is only used for tumors arising inside the brain/CNS.
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.
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.
MP/H Rules/Histology--Bladder: Should the term "dedifferentiation" be used to code sarcomatoid transitional cell carcinoma (8122/3)? Or is this typically referring to the grade, and not the histologic subtype? See Discussion.
Pathology report Final Diagnosis: TURBT : Urothelial carcinoma, high grade. Type/grade comment: Extensive sarcomatoid dedifferentiation is present (40-50% of tumor volume).
Assign 8122/3 for urothelial carcinoma, extensive sarcomatoid dedifferentiation. Sarcomatoid dedifferentiation refers to the histologic type. 8122/3 is also correct for the following diagnoses.
Urothelial carcinoma, sarcomatoid carcinoma or sarcomatoid variant 8122/3
Urothelial carcinoma with sarcomatoid features 8122/3
MP/H Rules/Histology--Brain and CNS: What is the code for Rosette-forming glioneural tumor from a pathology report of a brain tumor biopsy for a date of diagnosis in 2015? See Discussion.
Assign 9505/1 for Rosette-forming glioneuronal tumor. The new code, 9509/1, has not been implemented in the United States. 9505/1 is to be used until the new code is implemented. See page 7 of the NAACCR Guidelines for ICD-O-D Implementation, effective January 1, 2014, http://www.naaccr.org/LinkClick.aspx?fileticket=u7d3sB71t5w%3d&tabid=126&mid=466.
MP/H Rules/Histology--Breast: Which is the correct histology code to use and which MP/H rule applies in the case of a single lumpectomy specimen that demonstrates two separate tumors with the following histologies.
1) Invasive lobular carcinoma
2) Invasive ductal carcinoma with tubular features
See discussion.
Does ductal carcinoma with tubular features qualify for Breast MP/H Rule H28? Or, is it more appropriate to strictly follow Table 2 (not a type of ductal tumor) and apply Rule H29, thus losing the lobular component?
Abstract a single primary using Rule M13. Assign 8523/3 using rule H29. The code for invasive ductal carcinoma with tubular features (8523/3) is higher than the code for invasive lobular carcinoma (8520/3). H28 does not apply because 8523/3 is not included as a type of duct carcinoma on Table 2.
Reportability--Eye: Is conjunctival intraepithelial neoplasia (CIN III) from an excision of the left eye conjunctiva reportable?
Conjuctival intraepithelial neoplasia grade III (CIN III) is reportable. Intraepithelial neoplasia, grade III, is listed in ICD-O-3 as /2. It is reportable for sites other than skin.
Reportability--Carcinoid: Is a diagnosis of carcinoid heart disease, based solely on clinical information and no pathology, reportable?
Carcinoid heart disease is not reportable but this diagnosis indicates that the patient likely has a carcinoid tumor which may be reportable. Obtain further information.
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.
Primary Site--Stomach: How do I code the primary site when the operative report and pathology report state that the tumor site is incisura of the stomach?
Assign C163. Incisura, incisura angularis, gastric angular notch, angular incisure of stomach all refer to the sharp angular depression in the lesser curvature of the stomach at the junction of the body with the pyloric canal. See Gastric angular notch in #12 on page 76 in the SEER manual, http://seer.cancer.gov/manuals/2015/SPCSM_2015_maindoc.pdf. See also the SEER training website, #12 on the illustration corresponds to the angular notch, http://training.seer.cancer.gov/ugi/anatomy/stomach.html. We will correct the key for this illustration.
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