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Patient diagnosed with multinodular and vacuolating neuronal tumor of the cerebrum. My research shows: Multinodular and vacuolating neuronal tumor of the cerebrum is a recently reported benign, mixed glial neuronal lesion that is included in the 2016 updated World Health Organization classification of brain neoplasms as a unique cytoarchitectural pattern of gangliocytoma. There is no code in ICD-O-3 for it, so do I report it and use 9492/0 or not ?

Patient was diagnosed March 2023 with a neuroendocrine tumorlet of the rectum measuring 0.8 mm via excisional biopsy during colonoscopy.

Prior SINQ 20160011 (stomach specific) indicates microcarcinoid and carcinoid tumors are reportable. Microcarcinoid is a designation for neuroendocrine tumors of the stomach when they are less than 0.5 cm. in size.

Is the current rectal tumor a reportable gastrointestinal neuroendocrine tumor if it is less than 5 mm (i.e., is a neuroendocrine tumorlet equivalent to a microcarcinoid)?

SEER Program Coding and Staging Manual: Reportability – Reportable Diagnosis List indicates pancreatic intraepithelial neoplasia (PanIN II) (C250-C259) is reportable.

However, the ICD-O-3.2 lists “Glandular intraepithelial neoplasia, grade II” and “Glandular intraepithelial neoplasia, low grade” as histology code 8148 with behavior of /0 (benign).

According to the literature, PMH is a low-grade malignant vascular neoplasm of different tissue planes including skin and soft tissue. However, the references also state: PMH is a cutaneous tumor that behaves in an indolent fashion. There is no indication that this was a malignant diagnosis.

12/3/18 Foot, left skin lesion, punch biopsy: Superficial squamous epithelium demonstrating hyperkeratosis and fragments of keratin debris, no tumor seen.

Foot, left skin lesion, punch biopsy: Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma, see note.

NOTE: The submitted immunohistochemical slides were reviewed. Positive and negative controls reacted appropriately. The tumor cells demonstrate immunoreactivity to CK AE1/AE3 and CK7. The CD31 immunoreactivity described in the report cannot be confirmed as only the positive control is submitted for review. The tumor cells are negative for desmin, CD45, CD68, S-100, CD34, SMA, CD20, and HHV8. The proliferative index via Ki-67 is approximately 10%. The morphology (described below) and immunohistochemistry performed are compatible with a pseudomyogenic hemangioendothelioma.

12/4/18 Final Pathologic Diagnosis: Foot, left bone lesion, biopsy: Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma, see note.

Note: The patient's imaging findings were reviewed in conjunction with this case, revealing numerous lytic lesions of the tibia, fibula, talus, tarsal, metatarsal, and phalangeal bones. Additionally, as per the medical record, also reviewed in conjunction with this case, there are lesions of the skin. Thus, an extensive immunohistochemical panel was performed in an attempt to support the morphologic findings in this case, which were morphologically similar to the patient's skin biopsy. The tumor cells demonstrate strong immunoreactivity to pancytokeratin (CK AE1/AE3) and vimentin with moderate immunoreactivity to Fli-1. The tumor cells demonstrate weak immunoreactivity to epithelial membrane antigen. INI-1 is retained. There is focal immunoreactivity to CD31 although this is limited to the edges of the tissue fragments. The tumor cells are negative for HHV-8, CD34, smooth muscle actin, CK8/18, desmin, CD99, and Bcl-2. The combination of morphologic (see below for microscopic description) and immunohistochemical findings are consistent with pseudomyogenic hemangioendothelioma. Fresh tissue was submitted for karyotype analysis at the time of intraoperative consultation; however, it revealed only a normal appearing male karyotype. Thus, molecular confirmation was sought. The original slides and a paraffin block were submitted for FOSB rearrangement analysis, as pseudomyogenic hemangioendothelioma is known to have recurrent rearrangements with FOSB. Additional immunohistochemistry performed at (FACILITY) demonstrating immunoreactivity for ERG, supporting a vascular origin for this neoplasm. Fluorescence in situ hybridization demonstrated that 13% of the cells examined show FOSB rearrangement. While this FISH probe is for investigational purposes, the above findings support the diagnosis of pseudomyogenic hemangioendothelioma.

The pathology diagnosis reads: Diagnosis Right Kidney, Laparoscopic Nephrectomy: 

-Renal Cell Neoplasm of Oncocytosis (pT1a, pNX See Comment and Template). 

-Surgical margins free of tumor.

Kidney, right, nephrectomy:

Tumor histologic type: Renal cell neoplasms of oncocytosis (see Note)

Sarcomatoid features (%) Not identified

Tumor size: 4 cm (greatest dimension largest tumor)

Other dimensions: 2.7 x 2.5 cm

Macroscopic extent of tumor: Limited to kidney

Focality: Multifocal

Number of tumors: 11 grossly visible, range 0.2 4 cm

Fuhrman grade: 2 of 4

Microscopic extent of tumor:

Perinephric fat invasion: Not identified

Renal sinus invasion: Not identified

Other: N/A

Renal vein involvement: Not identified

Adrenal gland present: No

Involved by tumor: N/A

Direct invasion or metastasis: N/A

Cancer at resection margin: Not identified

Location(s): N/A

Pathologic findings in nonneoplastic kidney: Multiple collections of oncocytic cells

Hilar lymph nodes present: No

Number of involved/number present: N/A

"Thank you for sending this fascinating case. In reviewing the H&E-stained slides, we recognize that multiple lesions of varying sizes are present within the specimen, some with features of oncocytoma, some with those of chromophobe RCC, and yet others with features of both. The immunohistochemical studies for CK7 performed at your institution serve to highlight this point with "mass #1" showing focal single cell staining typical of oncocytoma and "mass #2" showing a patchy and confluent staining pattern typical of chromophobe RCC. This second mass was also positive with special stain for Hales colloidal iron. As mentioned, the morphology varies somewhat in each tumor, however, every single mass is comprised of cells with eosinophilic (pink to bright red) cytopolasm. Some tumors show more tightly nested or sheet like growth, others are more tubular or microcystic. Another important feature, present on slides of renal cortex are microscopic tumorlets seemingly emanating from eosinophilic tubules. This finding, along with the presence of numerous oncocytic neoplasms is supportive of the above diagnosis. The absence of clinical features to suggest Birt-Hogg-Dube syndrome is noted. Although these tumors are not recognized in the current classification of renal tumors, we regard these neoplasms as being a distinct entity, unrelated to both oncocytoma and chromophobe renal cell carcinoma, and have applied the designation "renal tumor of oncocytosis" to such lesions (Gobbo S, et al. Renal cell neoplasms of oncocytosis have distinct morphologic, immunohistochemical, and cytogenetic profiles. Am J Surg Patholl 34:620-626, 2010). We concur that the expected behavior in these cases is one of indolence."

10/27/2017 partial nephrectomy final path diagnosis: renal oncocytic neoplasm, favor hybrid oncocytic tumor. Comment:

Serous borderline tumor–micropapillary variant (8460/2, C569) was included in the ICD-O-3 Behavior Code/term updates effective 1/1/2018 but marked as Not Reportable for 2018.

There have been multiple additional updates to the ICD-O but no further clarification as to the reportability of this histology. ICD-O-3.2 currently lists serous borderline tumor, micropapillary variant (C569) as 8460/2 with no mention of reportability and no information provided in Includes/Excludes.

SINQ 20220032 instructs capturing this histology as reportable when diagnosed 1/1/2021 or later and occurring in the testis. The answer indicates this is reportable due to the /2 behavior code in ICD-O-3.2, but it does not specify that it is limited to specific sites.

Is serous borderline tumor, micropapillary variant reportable for ovary? If so, what dates apply? Is serous borderline tumor, micropapillary variant of the testis diagnosed after 1/1/2021 reportable?

Patient had a left thigh soft tissue mass excision on 7/24/24 and was diagnosed with superficial CD34 positive fibroblastic tumor. Margins were narrowly free of disease. Tumor size was 5.5 cm x 4.4 cm x 3.9 cm. The diagnosis was confirmed.