SEER Inquiry System - Search

The diagnosis date is 2/24/14.  The pathology report of the pancreas shows: Well-differentiated neuroendocrine tumor (NET), low grade (WHO G1 of 3). Addendum: Ki-67 confirms low grade of pancreatic endocrine tumor (less than 2% Ki-67/MIB-1 index). Chromogranin confirms the endocrine nature of the tumor. The Pre and Post Op Diagnosis is pancreatic neuroendocrine tumor consistent with insulinoma. AJCC Stage as noted on path report: pT1, pNX, pM.

The physician states: The patient has a well-documented insulinoma. Biochemistries confirmed the disease and it is localized in the tail of the pancreas.

The issue with NETs is that pathology report reflects what is seen or what is quantified under the microscope; often, there is a specimen without the accompanying medical history and clinical signs. Many of these NETs are specified on the basis of the hormone, as usually measured in the blood, that is overproduced, something not seen microscopically. All of the islet cell tumors are NETs. The insulinoma in the example above is a well-differentiated NET that is causing insulin to be over-produced. Thus, the diagnoses are not discordant; insulinoma is a more specific NET.

The definition for SSF6 for breast changed from "Unknown if invasive and in situ components present, unknown if tumor size represents mixed tumor or a pure tumor" to an added clarification of "Clinical tumor size coded." Since the clinical tumor size is NOT coded, this does not fit.

The definition for 060 is "Invasive and in situ components present, unknown size of tumor (CS Tumor Size coded 999). Since it is unknown if the tumor is mixed, this definition does not fit either.

It seems that the revised (April 2005) definition for 888 has left a situation that cannot be coded.

The clinical history indicates a destructive, quickly growing intra-oral lesion in the left soft tissue vestibule and the entire left maxilla.

Pathology report final diagnosis: Oral cavity, left maxilla, incisional biopsy: Malignant lymphoma, non-Hodgkin, diffuse large B-cell type, immunoblastic variant.

The clinical documentation clearly identifies residual tumor after the 2011 craniotomy. Scans demonstrated slow enlargement of the tumor over the years, which resulted in a repeat craniotomy. The pathologist noted in the diagnosis comment section of the pathology report that

Is this a single primary per MP/H Rule M3 (A single tumor is always a single primary), or an additional brain malignancy per MP/H Rule M8 (Tumors with ICD-O-3 histology codes on different branches in Chart 1 or Chart 2 are multiple primaries)?

The case was reviewed by AFIP and called "extratesticular." Per our pathology consultant, the site should be coded to unknown because it is very doubtful that the tumor was primary in the soft tissue of the thigh. According to him, such tumors don't originate exclusively in the testes, but tend to occur along the central axis such as the mediastinum or retroperitoneum. If an extratesticular tumor arises in either of these areas, the primary site should be code to the mediastinum or the peritoneum rather than to unknown. Lesions primary in the testicle may also undergo maturation with fibrosis and involution. This process often leaves little evidence of the original tumor in the testis.

The case in question represents an adenocarcinoma with neuroendocrine differentiation that arises in the absence of androgen-deprivation therapy. A 2023 journal article states, “We show that amphicrine prostate cancer is a unique entity and differs in clinical and molecular features from high-grade neuroendocrine carcinomas of the prostate. Our study highlights the need to recognize AMPC as a unique molecularly defined subgroup of prostate cancer.”

Should we be coding this with histology 8140 (Adenocarcinoma, NOS) because we have no specific code for an amphicrine carcinoma? Should we code this as 8045 (Mixed small cell carcinoma) because this is possibly the only way to capture both the adenocarcinoma and neuroendocrine components in a patient without previous treatment?

Our concern about using histology code 8574 (Adenocarcinoma with neuroendocrine differentiation) is that, while a valid histology code, this might confound the data if researchers are trying to separate the truly treatment-related tumors from other histologies captured under 8574.

The bone marrow was involved by lambda-restricted atypical B-cell and plasma cell populations with MYD88 mutation. Together these populations represent 10-15% of the bone marrow cellularity. While the bone marrow biopsy pathology alone did not provide a reportable diagnosis, the oncologist clinically diagnosed this as smoldering WM in the medical record.

Is a diagnosis of smoldering WM similar to a diagnosis of smoldering multiple myeloma (MM), a reportable Heme neoplasm, since smoldering neoplasms may be considered to meet the neoplasm’s threshold in the bone marrow but is otherwise asymptomatic?

The bone marrow biopsy proved bone marrow with blasts comprising 15-19%. Neither the pathologist nor the physician specifically diagnosed this as AML, calling this only high risk disease or early evolving AML prior to starting the patient on Vidaza.

No further information can be obtained from the pathologist or the physician for this case. Should this early evolving AML be accessioned as an additional primary per Rule M10, or is this the same MDS that is now high risk as the blast count is up to 19%, but has not yet reached the threshold of 20% blasts usually required for AML per the Hematopoietic and Lymphoid Neoplasm Database?

The autopsy report indicated a diagnosis of: Skin: Hyperkeratosis and pseudoepitheliomatous hyperplasia as well as reactive angioendotheliomatosis indicating Elephantiasis Nostras Verrucosa.

The article I read (https://www.ncbi.nlm.nih.gov/pubmed/?term=28984674) makes the distinction between mesophrenic adenocarcinoma and mesophrenic-like adenocarcinoma. The authors propose the term mesonephric-like Mullerian adenocarcinoma. So would this be coded as Mullerian adenocarcinoma?