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The patient was diagnosed with Langerhans cell histiocytosis as a child when the disease was not reportable [9751/1]. The patient was disease free until a recurrence in 2011. Langerhans cell histiocytosis is reportable if diagnosed 1/1/2010 and later [9751/3]. The Heme Manual states this is a single primary, but the behavior has changed from borderline to malignant since the initial diagnosis.

In addition to the issue mentioned above, there is a SINQ answer that conflicts with the 2018 ICD-O-3 Histology Update table. SINQ 20130134 indicates indolent systemic mastocytosis is reportable for cases diagnosed 2010 and forward. There is no date restriction indicating the SINQ note applies only for cases diagnosed 2010-2017. Since indolent systemic mastocytosis was changed to borderline (9741/1) for diagnosis year 2018+, should the diagnosis year range be updated for this SINQ answer?

A physician stated the patient likely had secondary polycythemia vera due to cardiac and pulmonary conditions but that a polycythemia vera could not be ruled out. A JAK2 was ordered that was positive for JAK2 V617F mutation. The patient was treated with hydrea.

According to SEER SINQ 20120049, secondary polycythemia vera is not reportable. However, in this case, the patient was positive for JAK2 V617F mutation. Therefore, is this reportable? We looked for guidance in the Hematopoietic and Lymphoid Neoplasms Database and found it confusing that secondary polycythemia vera was not mentioned or discussed under polycythemia vera in the database. The only thing we could find was secondary polycythemia NOS that was discussed under polycythemia.

According to the ICD-O-3, the histology for IPMN adenoma is 8453/0 is non-reportable. However, per SINQ 20021099, this is reportable.

CT a/p read by radiologist shows: "Bosniak 4 cystic lesion of right kidney." Follow-up MRI a month later reads "right kidney cystic lesion with enhancing mural nodule concerning for cystic renal cell carcinoma (RCC)." Urologist consult used the same wording of "Bosniak 4 cystic lesion" and "concerning for renal cell carcinoma." Treatment discussed but due to patient health status recommended repeat imaging. Repeat CT few months later reads: "cystic right renal lesion with enhancing nodule similar to most recent prior and suspicious for cystic RCC."

Though "suspicious for cystic RCC" per latest imaging is reportable, Bosniak 4 is "clearly malignancy, ~100% malignant" by definition, so is the case actually reportable with the first CT a/p date as date of diagnosis?

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

The partial nephrectomy final diagnosis is renal cell neoplasm. The College of American Pathologists (CAP) Summary lists histology as: multilocular clear cell neoplasm of low malignant potential. The diagnosis comment adds: This neoplasm currently termed multilocular clear cell renal cell neoplasm of low malignant potential (WHO 2016), was previously termed cystic renal cell carcinoma.

2015 Rt kidney nephrectomy pathology states: congenital cellular mesoblastic nephroma, tumor sz 5.9cm, tumor limited to kidney, extension into pelvicalyceal system, margin not applicable, LVI negative. Per PubMed.gov: (In newborns) among the low-grade malignant tumors, congenital mesoblastic nephromas can be successfully treated with simple nephrectomy. Per ScienceDirect: ...currently thought that cellular mesoblastic nephroma is actually a renal variant of infantile fibrosarcoma.

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."