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The hospital registrar reports that the case was identified at the hospital because of the referral for chemotherapy for the metastatic GIST. The records from the out-of-state hospital mentioned a history of a small bowel resection in 2003 for a borderline tumor. The registrar went back through the hospital's old records and found the surgery was done for GIST of low malignant potential at her facility. The question is whether to report the case or not, and if reported, is 2003 the diagnosis date.

The rules say to change the behavior and backdate the diagnosiswhen a tumor is presumed benign and islater diagnosed as malignant. Another problem for this case is that the out-of-state hospital did not review the slides from the 2003 surgery.

Example 1: Peripheral smear states: "No morphologic evidence of chronic myelogenous leukemia."

Addendum: Molecular diagnostic studies showed a positive rearrangement for the BCR gene with the M-bcr (CML type) and of bcr-abl transcript expression".

Example 2: Hematopathology is negative.

Molecular diagnostic study: "fluorescent in situ hybridization (FISH) studies exceeded the limits established by the XXX Cytogenetics Laboratory for this probe set, and thus, demonstrated statistical evidence of BCR/ABL fusion."

Pathology report from a shave biopsy states: "...markedly atypical junctional melanocytic proliferation. Changes highly suggestive of early melanoma arising adjacent to superficial congenital nevus." The re-excision pathology report states "biopsy proven melanoma" in the "Clinical History" section of the report (which is a reference to the original shave biopsy). The re-excision final pathology diagnosis states "no evidence of melanoma." The physician states that he thinks this is a melanoma. Should it be reported? Should Diagnostic Confirmation be coded to 1 or 8?

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

2021 Bone marrow biopsy showed erythroid hyperplasia, increased histiocytes with hemophagocytosis and Factor XIIIa positive histocytic cells. Moderate cytoplasmic staining for ALK 1, consistent with bone marrow involvement of ALK-positive histiocytosis. A subsequent kidney lesion biopsy was also found to have ALK-positive histiocytosis. The patient was then treated with clofarabine.

Patient is 3 years old.

07/2020-Chart indicates patient presented in June with fevers and refusing to walk with pancytopenia, bone marrow biopsy showed no leukemia buthistiocytes. Impression: ALK positive histiocytosis involving BM and kidney.

10/2020 Bone marrow final diagnosis states right and left bone marrow aspirates and biopsies: No morphologic or immunohistochemical evidence of involvement by the patient's previously diagnosed ALK+ histiocytosis (see Comments) - Multiple histiocytic collections with prominent hemosiderin; favor reactive - background normocellular bone marrow with maturing trilineage hematopoiesis.

The patient's prior bone marrow samples are reviewed (9/2020 and 7/2020). Similar to the September bone marrow sample, the current marrow shows numerous histiocyte collections with abundant associated hemosiderin deposition. These histiocytes have a stellate/dendritic appearance and lack the atypical features noted in the patient's marrow at diagnosis, favoring a reactive process. This impression is further supported by the lack of immunoreactivity for either Factor XIIIa or ALK1 among these cells. There is no convincing morphologic or immunohistochemical evidence of marrow involvement by the patient's previously diagnosed ALK+ histiocytosis within the sampled material. Of note, the marrow otherwise appears normocellular for the patient's age, indicative of ongoing marrow recovery post therapy.

It is not clear whether this would be equivalent to Langerhans cell histiocytosis, disseminated (9751/3) as there is not a statement of Langerhans cell or whether this is just histiocytosis, NOS and not reportable.