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The Solid Tumor histology coding guideline #3 for Lung states that an ambiguous histology can only be coded over an NOS when a physician clinically confirms it or the patient receives treatment based on the ambiguous histology; similar instructions exist in rules H3 and H12.  We are in a central registry and don't typically have access to physician notes or treatment plans; unfortunately our hospital abstracts rarely document physician confirmation of ambiguous histology and we are uncertain if we should accept their coding of the more specific histology, assuming they did find clinical confirmation that was not documented.  If not, our understanding of the Solid Tumor rules is that the histology in such a case would have to be coded as malignancy NOS (8000/3) per the non-ambiguous final diagnosis, and that we cannot use the more specific but ambiguous squamous cell carcinoma since we don't have definite clinical confirmation.  We also have a fair number of cytology-only lung cases without any hospital information to clinically confirm an ambiguous histology.

Patient was diagnosed with carcinosarcoma of Mullerian origin on omental/pelvic biopsies in March 2021. First course treatment was neoadjuvant chemotherapy followed by July 2021 resection showing residual primary endometrial carcinosarcoma with cervical stromal invasion and involvement of bilateral tubes/ovaries, omentum, and mesenteric nodule. Additional findings included endometrium with background endometroid intraepithelial neoplasia (EIN).

11/24/20 Adnexal mass, cyst fluid: Positive for malignant cells. Clusters of inhibin-positive, CK7-negative cells, consistent with adult granulosa cell tumor cells. Groups of inhibin-negative, CK7-positive epithelial cells consistent with serous borderline tumor cells. Peritoneal washing: Positive for malignant cells. Small groups of inhibin-positive, CK7-negative cells, consistent with adult granulosa cell tumor cells.

A. Left ovarian mass: Adult granulosa cell tumor (AGCT) of ovary (see note). pTNM Stage: pT1c3 pNX - Serous borderline tumor (SBT) of ovary (see note). pTNM Stage: pT1a pNX. Fallopian tube; unremarkable.

B. Right ovary: - Serous cystadenofibroma of ovary. Fallopian tube; unremarkable.

C. Left pelvic wall nodule: Fibro-calcified nodule, consistent with necrotic appendix epiploica.

D. Uterus (hysterectomy): Uterine leiomyomas. Endosalpingiosis of uterine serosa and paracervical tissue. Atrophic endometrium.

Note: The left ovarian mass is involved by a combined adult granulosa cell tumor and a serous borderline tumor. The AGCT mainly involves the thick-walled cystic area while the SBT the thin-walled cyst/s. The 2 neoplastic elements do, however, demonstrate areas of intimate and close intermingling. From the current literature, it appears that, based on FOXL2 mutation, the AGCT component of combined AGCT and ovarian epithelial tumors is either a true neoplastic processes or an AGCT- like proliferation morphologically indistinguishable from AGCT. To further evaluate the nature of the AGCT component, a FOXL2 analysis is in progress and an addendum will follow.

The following cases were submitted to the central registry as separate primaries. First case submitted as C772 with histology coded 9591/3 (Lymphoma, NOS). Second case submitted as C162 with histology coded 9699/3 (MALT Lymphoma).

Sequence 01 - 5/2016, Excisional biopsy pancreatic tail lymph node: suspicious for malignant B-cell lymphoma. No treatment recommended or administered.

Sequence 02 - 2/2019, Stomach biopsy: MALT Lymphoma. Unknown if treatment was recommended or administered. Biopsy was only at this facility.

Using the Hematopoietic and Lymphoid Neoplasm Multiple Primaries/Histology rules, Rule M7 makes this a single primary. Note 4 instructs to change the histology of the initial abstract to the more specific histology (9699/3). If this is done, they would be multiple primaries per the exception within Rule M2. Should the histology on sequence 01 be changed to the MALT lymphoma and the cases would be multiple primaries or is this a single primary?

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.

Final Pathologic Diagnosis:

Vulvar cyst, excision: Focal mammary-type ductal carcinoma in situ, intermediate grade, arising within cystically dilated duct (See Comment)

Size of DCIS: 0.7 CM.

Margins: Negative.

Comment

Sections demonstrate a cystically dilated duct. Focally, at the periphery of the duct, there is a neoplastic monomorphic proliferation of ductal cells with intermediate grade nuclei. No associated necrosis is identified. Immunostains for GATA-3 and estrogen receptor are strongly positive within the neoplastic cells, supporting origin from mammary-like epithelium. Immunostain for p63 demonstrates preservation of a basal layer around the dilated duct, including the region involved by DCIS. Immunostain for cytokeratin 5/6 shows loss of expression within the DCIS. No stromal invasion is identified. The cyst appears to be completely excised.

12/01/2020 post op visit with surgeon:

Ductal carcinoma in situ (DCIS) of the left vulva in an excised cystic lesion.

PLAN: I reviewed the pathologic findings from the excision of the left vulvar cyst. This appears to be a cystic lesion in the mammary line with focal DCIS. It was excised completely with negative margins. It would not warrant any additional treatment except expectant management.

Currently, First Course Therapy instruction for expectant management (also referred to as active surveillance, watchful waiting, etc.) instructs one to code 0 or 00 (not done) for all data items when the physician opts for expectant management.

We find that the treatment decisions can be driven by the patient, physician, or combination of both patient and physician depending on the options presented.