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Patient underwent excision of an oropharyngeal soft tissue mass revealing plasma cell neoplasm with extensive amyloid deposition. During work-up, bone marrow biopsy also revealed involvement by plasma cell neoplasm, with 5-10% of marrow cellularity. No amyloid seen in bone marrow. Patient was referred for radiation of the oropharyngeal mass. Per medical oncology qualifying best for the diagnosis of solitary extramedullary plasmacytoma with minimal marrow involvement. Decision made for observation by medical oncology in view of “minimal” bone marrow involvement. Question: Is rule M11 correct, and I abstract this case as a plasma cell myeloma, 9732/3, C421?

Patient complained of pain in the right side and back right upper flank area. CT shows an anterior mediastinal mass and abnormal appearance of skeleton. CXR: Age indeterminate T8 compression fracture. CT chest: abnormal appearance of skeleton. Correlate clinically for myeloma or mets. Acute T5 or T8 compression fractures. Anterior mediastinal mass which may represent thymoma, lymph nodes or metastases. 03/22/12 Metastatic Series: Nonspecific hypodensities in pelvis, left hip and right acromion. Possibility of myeloma can't be totally excluded. Bone marrow right post iliac crest core biopsy, clot section and aspirate: plasma cell myeloma.

Should the primary site be coded to the bone marrow because the diagnosis of plasma cell myeloma was supported by radiologic evidence of multiple lytic lesions? The bone marrow biopsy confirmed the radiology reports.

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.

Per rule PH11, "Code the primary site to the site of origin (lymph node region(s), tissue, or organ) and code the histology diffuse large B-cell lymphoma (DLBCL) (9680/3) when DLBCL and any other non-Hodgkin lymphoma are present in the same lymph node(s), lymph node region(s), organ(s), tissue(s) or bone marrow."

Should the focal diffuse large B cell lymphoma be ignored in this case and rule PH11 not be applied? To apply rule PH11, does the follicular lymphoma have to be NOS [9690/3] or does PH11 include all grades of follicular lymphoma [9695/3, 9691/3, 9698/3]?

In this case, the lymphoma arose in the posterior orbit and the primary site was coded as C696 (orbit, NOS). The mass directly extended to at least one "adjacent" site, the lacrimal gland. Should SS2000 be coded to 1 (localized) or 5 (regional, NOS) when an ocular adnexal lymphoma arises in the posterior orbit and extends to involve the lacrimal gland? Although both the posterior orbit and the lacrimal gland are parts of the orbit, they have separate ICD-O-3 topography codes. Should extension to multiple sites within the orbit be classified as localized disease?

The issue is what constitutes "adjacent" structures for a tumor that arises in the orbit. In an article published by the Indian Journal of Opthamology it states, "According to the Ann-Arbor staging system, lymphoma confined to the orbit is designated as Stage I, involvement of adjacent structures (sinuses, tonsil and nose) makes it Stage II." Does SEER agree with this definition of "adjacent" structures? Or are the lacrimal gland, ciliary body, retina, conjunctiva and/or choroid "adjacent" structures for a lymphoma stated to arise in the posterior orbit?

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.

Pathology report states: Prominent T-cell infiltrate with frequent immunoblast-like cells. COMMENT: Findings consistent with an acute EBV-associated hemophagocytic process. In addition, there is a prominent CD8 + T-cell infiltrate with many large, activated forms. This T-cell process may represent an EBV-associated lymphoproliferative disorder in children.

EBV-associated lymphoproliferative disorder in children is listed in the Heme database. However, throughout multiple admissions, the oncologist states the diagnosis as "hemophagocytic lymphohistiocytosis". Are the two the same condition?

The patient is being treated with Etoposide.