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Kidney, right interpolar neoplasm, partial nephrectomy: Oncocytic renal neoplasm of low malignant potential (ORNLMP).

Within part B, right interpolar kidney neoplasm, the neoplasm shows oncocytic features, with abundant granular eosinophilic cytoplasm and enlarged vesicular nuclei with prominent central nucleoli. The cells are arranged in small nests and tubules with hypocellular fibrous stroma identified within the background. Scattered binucleated cells are present, and rare cells with irregular nuclear membranes are present. No perinuclear halos or prominent cell membranes are present. Given the histologic features, the neoplasm is best classified as an oncocytic renal neoplasm of low malignant potential (ORNLMP).

Patient has a history of human immunodeficiency virus and diffuse large B cell lymphoma diagnosed in 2012, and is status/post systemic therapy and in remission since completing first course treatment. In 2022, the patient has imaging suspicious for recurrence. A biopsy of a deep left cervical lymph node showed atypical lymphoid infiltrate with the comment: “This is a challenging case. The constellation of findings is most in keeping with early / focal and subtle involvement by a nodular lymphocyte predominant B-cell lymphoma. We find no evidence of involvement by a diffuse large B-cell lymphoma.” The managing physician later states, “Cervical lymph node biopsy (06/2022) was consistent with nodular lymphocyte predominant B cell lymphoma.”

Imaging identified a mass involving the hypothalamus and pituitary gland and excision of the mass proved “histiocytosis.” The case was extensively reviewed, and the physician notes this patient has a pituitary tumor that is a “non-Langerhans cell histiocytosis,” or a “non-LCH histiocytic neoplasm.”

There is no histology for histiocytosis (NOS) or non-Langerhans cell histiocytosis. However, this does appear to be a non-malignant histiocytic neoplasm. If this were a Langerhans cell histiocytic neoplasm in the CNS it would be reportable. Should this non-Langerhans cell histiocytic neoplasm also be accessioned as a reportable CNS neoplasm? If so, how is the histology coded?

10/25/2023:  Patient presents to dermatology office with a questionable drug eruption having 3 weeks of papular eruptions of Trunk (Left Chest). Punch biopsies were taken that came back as immature hemopoietic infiltrate with monocytic differentiation. Comment: Myelodysplastic syndrome and leukemia cutis are possibilities. Addendum Report: Additional stains were prepared. ERG is strongly positive. CD1a and S100 do not stain the atypical cells.The controls stain appropriately. CD123 perform with appropriate control is also negative. The pattern is that of so-called "leukemia cutis" which could be seen in the clinical setting of myelodysplasia, chronic myelomonocytic leukemia (CMML) or precursor to acute myelomonocytic leukemia (AMML). Recommend work up. The only available information at present is a diagnosis of leukemia cutis, and that there was no prior history of a hematological malignancy in this patient.

Per the Heme Manual, Appendix F - Non-Reportable list for Heme Diseases, under Polycythemia, the Comment states that polycythemia is also known as erythrocytosis. Because polycythemia is equivalent to erythrocytosis, can we assume that "primary erythrocytosis" is equivalent to "primary polycythemia" and thus reportable as 9950/3 per the Heme DB? Or is the case nonreportable because the exact term of "primary erythrocytosis" is not listed as an alternate name for polycythemia vera, only "primary polycythemia" is listed?

The patient had a mediastinal mass biopsy showing fibrosclerotic tissue with patchy lymphohistiocytic foci and scattered plasma cells, followed by a retroperitoneal mass biopsy showing fibrohistiocytic infiltrate. Erdheim-Chester disease is not reportable per the Heme Database. However, the physician specifically states this is a malignant disorder.

Pathologists are increasingly using the terms "myeloid stem cell disorder" and "myeloid stem cell neoplasm" to describe reportable myeloid neoplasms. If the pathologist uses these terms and indicates the differential diagnosis includes only reportable neoplasms such as myelodysplastic syndrome, myeloproliferative neoplasm, and acute myeloid leukemia (AML), should this be accessioned as a reportable primary?

Example: The 01/2023 peripheral blood shows high grade myeloid stem cell disorder, and the differential diagnosis includes chronic myelomonocytic leukemia(CMML) and AML. The patient refused further work-up and expired several days later. No additional information is available.

Patient was admitted in July 15, 2002. Per the H&P, patient was diagnosed 5 years ago with myelodysplasia. Patient had bone marrow biopsy about 5 years ago and then again on 6-10-02. Patient has become transfusion dependent since mid-March. Bone marrow on 6-10-02 was consistent with myelodysplastic syndrome with refractory anemia with bilineage dysplasia with excessive blasts. Impression: Myelodysplastic syndrome slowly evolving toward acute leukemia. Plan: start chemo. 7-16-02 bone marrow biopsy showed acute myeloid leukemia.

Can we assume that the myelodysplasia diagnosed 5 years ago was refractory anemia and therefore, patient's first reportable diagnosis would be the AML? Or is the 6-10-02 bone marrow biopsy showing refractory anemia to be the first reportable diagnosis because the term "myelodysplasia" is non-specific?

The physician is calling it an indolent tumor, pT3/NX/M0 stage 3 with positive margins. Is the correct code C509, 8502/3?