Casefinding--Heme & Lymphoid Neoplasms: Is the 2010 casefinding code of 289.6 (Familial Polycythemia) addressed anywhere in the Hematopoietic Database? See Discussion.
When you enter "familial polycythemia" into the Heme DM, polycythemia vera (PV) appears; however, the term "familial polycythemia" is not listed as one of the synonyms for PV.
Familial polycythemia by itself is not reportable. This is a benign condition which occurs within families. Familial polycythemia can progress to polycythemia vera (9950/3), which would then be reportable. The code, 289.6, which is the ICD-9-CM code for Familial polycythemia is not included on the reportable list for casefinding. There is only one ICD-9-CM code for Polycythemia vera, 238.4. "Familial polycythemia" is listed in Appendix F: Non-Reportable List for Hematopoietic Diseases.
EOD-Size of Primary Tumor: Should the code 001 in tumor size be used for tumors described as having "focal" involvement? See discussion.
Is tumor size coded to 001 for the following examples:
Example 1: Focal adenoca in left lobe on prostatectomy.
Example 2: Multifocal ductal carcinoma of breast on mastectomy.
Example 1 and 2: There is insufficient information in the examples to determine whether EOD-Size of Primary Tumor should be coded to 001.
The instructions are that code 001 is used for a microscopic focus or foci of tumor only. That means that the tumor is small enough that it could not be seen by the naked eye, nor would it be palpable. Be careful with the term "focal" because it is most often used to describe tumor cells grouped or concentrated in one area as in example 1. There is no implication that this focus was microscopic only. Was it mentioned in the gross or macroscopic portion of the pathology report? If so, it is not coded to 001. Was it palpable? If so, it is not coded to 001.
Example 2 cites a multifocal breast cancer. Again, did the pathologist visualize the cancer (was it reported on the gross or macroscopic portion of the pathology?) If so, do not use code 001. Was the lesion palpable? If so, do not use code 001.
Reportability/Histology--Heme & Lymphoid Neoplasms: Is a physician statement that a patient has a malignant histiocytic disorder best described as Erdheim-Chester disease reportable? If reportable, should histology be coded to 9751/3? See Discussion.
The patient had a mediastinal mass biopsy showing fibrosclerotic tissue with patchy lymphohistiocytic foci and scattered plasma cells, followed by a retroperitoneal mass biopsy showing fibrohistiocytic infiltrate. Erdheim-Chester disease is not reportable per the Heme Database. However, the physician specifically states this is a malignant disorder.
The WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues states that Erdheim-Chester disease is a possible adult form of disseminated juvenile xanthogranuloma with bone and lung involvement; no histology code is provided.
Reportability/Behavior--Kidney: Is epithelioid angiomyolipoma (AML) of the kidney a reportable malignancy? See Discussion.
The addendum final diagnosis on a pathology report for a kidney core needle biopsy included the results of additional stains performed on the tissue. It indicated the morphology was most consistent with epithelioid angiomyolipoma. Further comments in the body of the report indicate these tumors are now considered malignant neoplasms with the capacity to be locally aggressive and they can potentially metastasize. There is no mention of a metastasis in this particular case.
Epithelioid angiomyolipoma (AML) [8860/0] of the kidney is not reportable unless stated to be malignant.
If the pathologist confirms this is a malignancy, apply ICD-O-3 Rule F (Matrix principle) and assign the behavior code /3. If confirmation is received, accession the case using the morphology code 8860/3 [malignant angiomyolipoma].
Reportability/Histology--Brain and CNS: Is epidermoid tumor of the cerebellopontine angle (CPA) and trigeminal vesicle nerve reportable, and if so, what is the correct histology code? See discussion.
Patient presented to hospital ED and had brain MRI that revealed 3.2 cm space occupying lesion in region of the left CPA and trigeminal vesicle nerve compatible with epidermoid tumor.
Epidermoid tumor of the brain is not reportable. There is no ICD-O-3 code for epidermoid tumor or epidermoid cyst. This type of tumor is often referred to as a cyst because it has a thin wall that secretes a soft material into the center.
Reportability/Histology--Endometrium: Is endometrial hyperplasia with atypia equivalent to atypical hyperplasia of the endometrium (8380/2) and thus reportable?
Endometrial hyperplasia with atypia is equivalent to atypical hyperplasia of the endometrium (8380/2) and thus reportable for cases diagnosed 2021 and later. Our expert pathologist consultant confirmed this for us.
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.
First Course Treatment--Unknown & ill-defined site: We have a case with an unknown primary site and the patient had chemoembolization into the hepatic artery. We don't know how to code this treatment. See Discussion.
We were told to code as surgery (10) and chemo (01). However an unknown primary automatically gets a (98) surgery code & the chemo is coded (01) but we can't code as systemic therapy. This is an edit. Chemo coded but no date of systemic therapy.
Effective for cases coded prior to the change in policy made on January 9, 2008, code chemoembolization of a metastatic site as 1 [nonprimary surgical procedure performed] in Surgical Procedure of Other Site.
Surgery of Primary Site code 98 is assigned to all cases with an unknown primary.
In the case of a liver primary, it would be coded 10 [local tumor destruction, NOS] in Surgical Procedure of Primary Site.
EOD 2018/Lymph Nodes--Corpus Uteri: Are lymph nodes found on imaging post-surgery included in Extent of Disease (EOD) Regional Nodes if surgery is already completed? See Discussion.
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.
EOD includes all information available within four months of diagnosis in the absence of disease progression or upon completion of surgery(ies) in first course of treatment, whichever is longer. Since the imaging was within the four-month window, and the nodes could have been positive during surgery but not assessed by the surgeon, use the information from the imaging.
Assign code 600 for EOD Regional Nodes for involvement of the aortocaval and retroperitoneal nodes (para-aortic nodes), size unknown.
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