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.
Reportability/Histology-Gallbladder: Is high grade biliary intraepithelial neoplasia of the gallbladder reportable?
High grade biliary intraepithelial neoplasia of the gallbladder is reportable. Assign code 8148/2. It is also known as biliary intraepithelial neoplasia grade 3, or BilIN-3.
MP/H Rules/Histology--Brain and CNS: What is the histology code for a tumor originating in the cerebellum and extending into the fourth venrticle described as a glioblastoma with primitive neuroectodermal tumor component (WHO Grade IV)?
The WHO Classification of CNS tumours lists glioblastoma with primitive neuroectodermal tumor component as a subtype of glioblastoma and assigns 9440/3. Also referred to as glioblastoma with a primitive neuronal component.
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.
Reportability--Breast: Is mammary fibromatosis reportable and if so, what histology code is assigned? See discussion.
The pathologist completed a CAP protocol using soft tissue. Pathology revealed a 2.5 cm tumor with invasion of skeletal muscle with deep margins positive for tumor.
Mammary fibromatosis is not reportable. The WHO classification for breast tumors assigns mammary fibromatosis a behavior code of /1. According to WHO, mammary fibromatosis "is a locally infiltrative lesion without metastatic potential…"
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--Pituitary: Would you code Crooke cell adenoma as 8272/0 pituitary adenoma?
Yes, code Crooke cell adenoma to 8272/0 pituitary adenoma. According to the WHO classification, it is a variant of adrenocorticotropic hormone (ACTH) producing adenoma (8272/0).
Surgery of Primary Site--Breast: If the diagnosis is a single primary involving both breasts, do we code 41 Surgery Primary site with 1 in Surgery Other site, or code 76 Surgery Primary site with 0 in Surgery Other site? See discussion.
In Appendix C- Breast (SEER Manual 2015) it states under the codes for TOTAL MASTECTOMY (Codes 40-49, 75): For single primaries only, code removal of involved contralateral breast under the data item Surgical Procedure/Other Site (NAACCR Item # 1294). [SEER Note: Example of single primary with removal of involved contralateral breast--Inflammatory carcinoma involving both breasts. Bilateral simple mastectomies. Code Surgery of Primary Site 41 and code Surgical Procedure of Other Site 1.] HOWEVER, underneath that it states code 76 is used for: 76 Bilateral mastectomy for a single tumor involving both breasts, as for bilateral inflammatory carcinoma. So
Assign code 41 with 1 in surgery other site for simple mastectomy. Assign code 76 with 0 in surgery other site for a more extensive mastectomy.
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.
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.