MP/H/Histology--Kidney, renal pelvis: What is the histology code for renal cell carcinoma translocation type?
Code renal cell carcinoma translocation type as renal cell carcinoma, NOS, 8312. While WHO recognizes renal cell carcinomas with associated translocations, there is no specific ICD-O-3 code for this variant of renal cell carcinoma.
MP/H Rules/Histology--Bladder: What is the correct histology code for a diagnosis of urothelial plasmacytoma carcinoma of the bladder per pathology report?
Assign code 8120/3, urothelial carcinoma, NOS, to urothelial plasmacytoma carcinoma of the bladder. The WHO classification describes plasmacytoid variants of urothelial carcinoma. There is no specific ICD-O-3 code for these variants; however, and 8120/3 must be used.
Seq no-central--Brain and CNS: How should subsequent tumors be sequenced when the patient has a history of a brain tumor, with no information on the behavior of the brain tumor? According to the sequencing rules, it appears some assumption must be made regarding the behavior of the brain tumor.
Sequence the brain tumor in the 60-87 series when you do not know the behavior. If you have reason to believe the brain tumor was malignant, sequence it in the 00-59 series.
Reportability/MP/H Rules/Histology: Is malignant perivascular epithelioid cell tumor (PEComa) reportable, and if so, what is the histology code?
Malignant perivascular epithelioid cell tumor (PEComa) is reportable because it is malignant. Assign 8005/3 to malignant PEComa.
We consulted an ICD-O-3 expert who explained that some PEComas such as angiomyolipoma and lymphangiomyomatosis have specific ICD-O codes and their malignant counterparts may be coded to 8860/3 and 9174/3 respectively. There are no separate ICD-O codes for other specific PEComas, e.g., clear cell “sugar” tumor of lung, clear cell myomelanocytic tumor of the falciform ligament and some “unusual” clear cell tumors occurring in other organs—or for PEComa, NOS. These PEComas may therefore be coded to 8005 as clear cell tumors NOS; in other words as clear cell tumors that are not clear cell variants of carcinomas, sarcomas, or other specific tumor type.
Please note, PEComa is non-specific as to behavior. Unless the pathologist states that it is malignant, (as was the case for this question), the default code is 8005/1 (non-reportable).
Reportability--Appendix: Is the appendix the primary site for a low grade mucinous appendiceal neoplasm (LAMN) with diffuse peritoneal dissemination? See discussion.
Patient had an appendectomy revealing a low grade mucinous appendiceal neoplasm (LAMN) with diffuse peritoneal dissemination. Patient now with cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC), which revealed metastatic disease in the abdomen, omentum, pelvic peritoneum, peri-cecal, and gallbladder.
For cases diagnosed prior to 1/1/2022
Low-grade appendiceal mucinous neoplasm (LAMN) is not reportable, even when it spreads within the peritoneal cavity, according to our expert pathologist consultant. Peritoneal spread of this /1 neoplasm does not indicate malignancy. It is still /1 when there is spread of LAMN in the peritoneal cavity.
Reportability/Behavior: Is the following reportable, and if so, what is the histology code? Final Diagnosis (on multiple conjunctive excisions): Conjunctiva - primary acquired melanosis with atypia (see note). Note: "In all 3 specimens the process extends to the margins of excision. Complete extirpation is recommended (primary acquired melanosis with atypia is considered melanoma in situ).
Do not report primary acquired melanosis with atypia.
According to our expert pathologist consultant, "There has been a lot of debate in the literature about the diagnostic criteria, terminology, and natural history of primary acquired melanosis [PAM]. Your case comes down squarely on the main issue, which is whether PAM with atypia should be regarded as melanoma in situ. In most studies it appears that PAMs with no atypia or mild atypia do not progress to melanoma, and only a small percentage of those with severe atypia do so." "PAM, even with atypia, is not melanoma in situ, and should not be reported."
For further information, see this article for a review of a large number of patients: Shields, Jerry A, Shields, Carol L, et al. Primary Acquired Melanosis of the Conjunctiva: Experience with 311 Eyes. Trans. Am Ophthalmol Soc 105:61-72, Dec 2007.
MP/H Rules/Histology--Skin: How is histology coded for an "endocrine mucin-producing sweat gland carcinoma with transformation to mucinous carcinoma"? See Discussion.
Endocrine mucin-producing sweat gland carcinoma (EMPSCG) is a rare type of low-grade sweat gland carcinoma. Some journal articles indicate that most patients with EMPSCG have coexisting mucinous carcinomas, suggesting that EMPSCG is a precursor to invasive mucinous carcinoma of the skin. Sweat gland carcinoma has its own histology code per the ICD-O-3 (8400/3); should an endocrine mucin-producing sweat gland carcinoma also be coded as 8400/3? If so, would the correct histology for the skin case above be mucinous carcinoma (8480/3) per Rule H17? Conversely, if the terms "mucin-producing" are referring to mucin-producing carcinoma, and not referring to the sweat gland carcinoma, would the histology be coded 8481/3 (mucin-producing carcinoma)?
Assign 8480/3.
There is no mixed ICD-O-3 code for EMPSCG. Both histologies are in the mucinous family: mucinous adenocarcinoma (8480/3) and sweat gland carcinoma (8400/3). Apply Other sites rule H17 and code the numerically higher ICD-O-3 code (8480/3).
Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare low-grade sweat gland carcinoma with a strong predilection to the eyelid region. It is histologically analogous to endocrine ductal carcinoma/solid papillary carcinoma of the breast and is characterized by a multinodular solid cystic mucinous tumor with immunoreactivity to neuroendocrine markers. Only 20 cases of this unusual tumor have been reported.
Reportability--Stomach: Is a well-differentiated neuroendocrine tumor of the stomach reportable?
Well-differentiated neuroendocrine tumor (NET) of the stomach is reportable. The WHO classification of digestive system tumors uses the term NET G1 (grade 1) as a synonym for carcinoid and well-differentiated NET, 8240/3.
Grade--Breast: Do you take grade from the most representative specimen along with the histology? What is the correct histology/grade combination? See discussion.
Breast biopsy (from hospital A): DCIS, solid, cribriform, comedo type, high nuclear grade
MP/H rules are to code histology based on the specimen with the most tumor tissue. That would be the lumpectomy in this case. The histology is DCIS, cribriform type.
The general rule for grade is to code the highest grade specified within the applicable grading system. For the case information provided, follow instruction #5, nuclear grade: use Coding for Solid Tumors #7: 2-, 3-, or 4- grade system. High nuclear grade (grade code 3 for breast) is higher than nuclear grade 1 (grade code 1).
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