Reportability--Heme & Lymphoid Neoplasms: Is a diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) with large cell transformation equivalent to a diagnosis of diffuse large B-cell lymphoma (DLBCL) without mention of Richter transformation or Richter Syndrome? See Discussion.
The patient has a history of CLL/SLL dating back to 2007, but has had progressive disease with development of a new left frontal brain tumor. The brain tumor resection proved CLL/SLL with large cell transformation, but neither the pathologist nor the managing physician called this a Richter transformation, Richter syndrome or provided a diagnosis of DLBCL. However, a large cell transformation of CLL/SLL is a Richter transformation. Can this be accessioned as a new acute neoplasm per Rule M10?
Accession as multiple primaries according to Hematopoietic and Lymphoid Neoplasm Coding Manual Rule M10. Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) followed by CLL/SLL with large cell transformation is multiple primaries because it is a chronic neoplasm followed by an acute neoplasm, more than 21 days in this case.
Reportability--Brain and CNS: Is meningioangiomatosis reportable as meningiomatosis (9530/1) or angiomatous meningioma (9534/0)? See Discussion.
Pathology report: Brain tumor, left side: Gliotic cortex and subcortical white matter with meningioangiomatosis (see Comment). Comment This specimen represents a meningioangiomatous lesion located in the leptomeninges that projects along the Virchow-Robin spaces into the underlying cortex. The surrounding brain parenchyma demonstrates reactive changes with astrogliosis and microgliosis. An intraparenchymal neoplasm is not seen. Meningioangiomatosis is a rare benign meningovascular hamartomatous condition and usually appears in young patients.
Meningioangiomatosis is not reportable. It is a cortical lesion which may occur sporadically or in NF2 (neurofibromatosis type 2). It is not listed in ICD-O-3.
MP/H Rules/Histology--Brain and CNS: What is the histology code for a patient with a pathology report Final Diagnosis indicating, mucin-rich neuroepithelial neoplasm, favor low-grade? See Discussion.
The pathologist noted this was a challenging brain neoplasm that did not easily fit into a specific WHO diagnostic classification. Multiple differential diagnoses were given including pilomyxoid astrocytoma, ganglioglioma and dysembryoplastic neuroepithelial tumor (DNET), but there were no definitive features characteristic of any of these tumors. In the Comment section following the Final Diagnosis, it further states: "In summary, the tumor appears to be a difficult to classify non-infiltrating glial/glioneuronal neoplasm without definitive high-grade features."
Code as 9505/1, Ganglioglioma, NOS. The Multiple Primaries/Histology Rules for Benign and Borderline Intracranial and CNS Tumors Chart 1 lists several histology codes for neuronal and mixed neuronal-glial tumors. Ganglioglioma, formerly Glioneuroma that is now obstolete in ICD-O-3, is the most applicable in this situation.
MP/H Rules/Histology--Pancreas: What is the histology code of invasive adenocarcinoma, non-mucinous with intraductal tubulopapillary features, moderately differentiated, from the pathology report final diagnosis of the pancreas? Does 'intraductal" refer to a non-invasive/in-situ component or describe the pattern of growth?
Assign 8503/3, intraductal papillary adenocarcinoma with invasion, to capture the more specific features of the adenocarcinoma. Histology Rule H13 for Other Sites states to code the most specific histologic term. Examples include Adenocarcinoma and a more specific adenocarcinoma. Note: The specific histology may be identified as type, subtype, predominantly, with features of, major, or with ___ differentiation.
Reportability--Liver: Is intraductal papillary mucinous neoplasm (IPMN) of the liver a reportable diagnosis? See Discussion.
Pathology shows: Right liver lobe, partial hepatectomy " intraductal papillary neoplasm with high grade dysplasia.
Intraductal papillary mucinous neoplasm (IPMN) of the liver with high grade dysplasia is reportable. While most IPMNs arise from the pancreas, there exists a subset of IPMN of the biliary tract (BT-IPMN). Code as 8453/2.
MP/H Rules/Histology--Bladder: Is urothelial carcinoma, high-grade, predominantly solid type, coded as 8120/3 or 8230/3? See Discussion.
Urinary bladder: Invasive urothelial carcinoma, high-grade, 4.5cm, predominantly solid type, arising in background of carcinoma in-situ, carcinoma grossly extends into perivesical adipose tissue; lymph-vascular invasion is seen.
Assign histology code 8120/3, urothelial carcinoma, NOS. Solid type is not a recognized variant of urothelial tumors and likely represents the appearance of the urothelial cells within the tumor and not a specific histologic type.
MP/H Rules/Multiple primaries--Brain and CNS: How many primaries should be abstracted for a patient with a 2011 diagnosis of oligodendroglioma followed by biopsy of tumor which demonstrated progression in 2016 with pathology report Final Diagnosis indicating WHO grade III anaplastic astrocytoma? See Discussion.
The clinical documentation clearly identifies residual tumor after the 2011 craniotomy. Scans demonstrated slow enlargement of the tumor over the years, which resulted in a repeat craniotomy. The pathologist noted in the diagnosis comment section of the pathology report that
Is this a single primary per MP/H Rule M3 (A single tumor is always a single primary), or an additional brain malignancy per MP/H Rule M8 (Tumors with ICD-O-3 histology codes on different branches in Chart 1 or Chart 2 are multiple primaries)?
Based on the information provided, this is a single primary. The 2011 tumor was not completely removed and progressed over the years. MP/H Rule M3 for malignant brain cancer applies. Do not change the original histology code. Use text fields to document the later histologic type of anaplastic astrocytoma, WHO grade III.
First Course of Treatment/Surgery of Primary Site--Corpus uteri: Do you code total hysterectomy or radical hysterectomy when a specimen indicates the uterus, cervix, ovaries, fallopian tubes, and right and left parametrium were resected, but shows no portion of the vagina. See Discussion.
AFS1-AFS2-frozen section control, endomyometrium; AFS3-frozen section control, subserosal intramural mass; A4-anterior cervix; A5-posterior cervix; A6-anterior cervical endometrial junction; A7-posterior cervical endometrial junction; A8-A10-anterior endomyometrium, including tumor; A11-A13-posterior endomyometrium, including tumor and adjacent mass; A14-random section subserosal mass; A15-left parametrium at margin of resection; A16-right parametrium at margin of resection; A17-A18-left ovary and fallopian tube; A19-A20-right ovary and fallopian tube. The final diagnosis includes Endometrial adenocarcinoma, favor serous carcinoma, with papillary and solid areas. Tumor involves: Cervix present, Right ovary, Left ovary, Right fallopian tube, Left fallopian tube, Right parametrium, Left parametrium.
Assign code 50 for total hysterectomy. According to Appendix C Surgery Codes for Corpus Uteri of the 2016 SEER Coding and Staging Manual, total hysterectomy is surgery to remove the entire uterus, including the cervix; whereas, radical hysterectomy includes the vagina.
Reportability/Histology--Skin: Is 'skin, left temporal scalp, low grade adnexal carcinoma, probable sweat gland origin' reportable as 8400/3, skin of temple?
Assign 8390/3 for adnexal carcinoma of skin. 8390/3 is reportable, including 8390/3 of skin.
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