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.
Update to Current Manual/2018 EOD Manual/EOD Primary Tumor--Bladder: According to the American Joint Commission on Cancer (AJCC), a transurethral resection of the bladder (TURB) cannot make a distinction between involvement of the superficial muscle-inner half (Stage T2a) and the deep muscle-outer half (Stage T2b). Is this same criteria applied to Extent of Disease (EOD)?
EOD follows AJCC criteria in this situation and we have confirmed with AJCC that Stage T2a (superficial muscle) and Stage T2b (deep muscle) cannot be assigned when only a TURB is done.
For EOD Primary Tumor, Bladder, codes 200, 250, 300, 350, can only be used when
Cystectomy is performed
Muscularis propria is involved, AND
Pathology report states superficial muscle (200, 250) or deep muscle (300, 350) is involved
If a TURB is done and there is mention of the muscularis propria invasion (superficial muscle or deep muscle), use EOD codes 370 or 400. If a TURB is done and the pathology report states superficial or deep muscle, ignore and coded as “invasion of muscularis propria, NOS” (EOD codes 370 or 400).
Instructions and code descriptions for EOD Primary Tumor have been updated to indicate this. These updated instructions and code descriptions will be available when SEER*RSA is updated for 2024, Version 3.1 (Sept/Oct 2023). These updates are included here for reference and can be applied for cases diagnosed 2018+.
First course treatment--Heme & Lymphoid Neoplasms: How is a "donor lymphocyte infusion" that is used in the treatment of CLL coded?
Donor lymphocyte infusion (DLI) is coded as immunotherapy. The lymphocytes are donated by the same person who donated the original stem cell transplant. The lymphocyte infusion creates an immune response in which the T-cells are activated to attack the cancer cells.