MP/H Rules/Histology: What is the correct histology code for a NUT midline carcinoma?
Code histology to 8010/3.
NUT carcinoma is identified by the NUTM1 gene rearrangement.
NUT midline carcinomas (NMC) are lethal and morphologically indistinguishable from other poorly diff carcinomas. They are epithelial tumors which can range from undifferentiated carcinomas to carcinomas with prominent squamous differentiation.
A new proposed ICD-O-3 code has been suggested for NUT tumors but it is not yet approved for implementation. Do not use the new code until it is approved for use in the United States.
Reportability--Brain: Is benign lymphangioma of the brain (9170/0) reportable? It is not on the list of non-malignant blood vessel tumors in the National Program of Cancer Registries Clarifications for Central Nervous System (CNS) tumors.
Lymphangioma of the brain or CNS is not reportable. Lymphangioma is a malformation of the lymphatic system. Even though it has an ICD-O-3 code, do not report it.
Reportability--Lung: Is a case of pulmonary metastatic leiomyoma (favored) vs. low grade leiomyosarcoma reportable, and if so, what is the primary site and histology code? See Discussion.
Patient presents with an abnormal chest x-ray. PET reveals 4.6 cm left lower lobe mass and several additional bilateral nodules measuring up to 1.6 cm. Biopsy was recommended and is positive for metastatic histologically benign smooth muscle neoplasm. ER/PR are positive. Mayo consult on biopsy agrees with histology. The differential diagnosis includes benign metastasizing leiomyoma and low grade leiomyosarcoma. Comment: If these nodules remain small and do not progressively grow would consider this metastasizing leiomyoma. Physicians state bilateral pulmonary metastatic leiomyoma (favored) vs low grade leiomyosarcoma. Tamoxifen was started. Patient has a history of uterine fibroids. Several months later, imaging reveals stable bilateral multi pulmonary nodules and left lower lobe mass but persistent. Surgery was recommended but cancelled due to insurance.
This case is not reportable based on the information provided. The histologic diagnosis is "metastatic histologically benign smooth muscle neoplasm." The physicians seem to agree with the histologic diagnosis, benign metastasizing leiomyoma (BML). The WHO classification and ICD-O-3 assign 8898/1 to "metastasizing leiomyoma." WHO states "This resembles a typical leiomyoma but it is found in the lungs of women with a history of typical uterine leiomyomas." A recent article states "Because of the hormone-sensitive characteristics of BML, treatments are based on hormonal manipulation along with either surgical or medical oophorectomy." Tamoxifen treatment is in keeping with the BML diagnosis.
MP/H/Histology--Breast: What MP/H Rule, histology, and behavior code for a breast primary apply to the following?
2 foci DCIS, solid, high grade (Grade 3) w/microca++
Apply the Multiple Primaries/Histology, Breast Rule H3: DCIS and a more specific in situ are coded to the more specific histology term which in this case is solid. Code the histology to ductal carcinoma in situ, solid type (8230/2). Based on the information provided, there is no invasive component. The term "microca ++" means micro-calcifications are present, not micro carcinoma.
Reportability/Histology--Head and Neck: Is mammary analogue secretory carcinoma (MASC) of the left submandibular gland reportable and how is it coded? See Discussion.
The physician is calling it an indolent tumor, pT3/NX/M0 stage 3 with positive margins. Is the correct code C509, 8502/3?
Mammary analogue secretory carcinoma (MASC) is reportable. MASC is a recently described tumor that predominantly arises in the parotid gland. In this case, if the primary site is submandibular gland, assign C080. We contacted our expert pathologist and he stated that the best code to use for MASC is 8502/3. Override any edits triggered by the combination of C080 and 8502/3.
Grade/Histology--Digestive System: What is the grade for neuroendocrine tumor (NET) or neuroendocrine carcinoma (NEC) of gastrointestinal morphologies described as: 1) NET G1 (M8240/3) and NET G2 (M8249/3) or 2) neuroendocrine carcinoma, low grade (M8240/3) and neuroendocrine carcinoma, well differentiation (M8240/3) and neuroendocrine carcinoma, moderate differentiation (M8249/3)? The SEER Instructions for Coding Grade for 2014+, Coding for Solid Tumors section, #3 state: Code the grade shown below (6th digit) for specific histologic terms that imply a grade. NET and NEC are not included in the specific terms.
You may code grade as follows.
Grade 1 – NET G1 (M8240/3)
Grade 2 – NET G2 (M8249/3)
Grade 1 – neuroendocrine carcinoma, low grade (M8240/3) or neuroendocrine carcinoma, well differentiation (M8240/3)
Reportability/Histology--Pituitary Gland: How are Rathke cleft cyst and Rathke pouch tumor distinguished and are they both reportable?
Rathke cleft cyst is not reportable. Cysts are not neoplastic. However, Rathke pouch tumor (C751, 9350/1) is a reportable neoplasm for cases diagnosed 2004 and later. The Rathke pouch is coded to the pituitary gland. Benign and borderline pituitary tumors have been reportable since 2004.
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.
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
Reportability/MP/H Rules/Histology--Ovary: What is the histology code for an ovarian tumor described as a mucinous borderline tumor, intestinal type?
Mucinous borderline tumor, intestinal type, of the ovary is not reportable. The behavior is /1. There is no applicable histology code for this histology when it ocurs in the ovary.
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