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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?

The diagnosis was made in Oct. 2002 by a CT guided fine needle aspiration of a large iliac mass, right buttock area. The cytology report says:

a. positive for malignant cells, markedly atypical high grade malignant neoplasm.

b. It is impossible to tell from this aspiration biopsy whether or not this represents a high grade sarcoma or a high grade carcinoma, but our consensus opinion is that this lesion is a high grade carcinoma.

The combination of soft tissue topography and carcinoma morphology is Impossible by SEER edits. How should we code this?

The patient has adenopathy and a lymph node biopsy proved CLL/SLL. The patient underwent a peripheral blood smear, but the final diagnosis only indicated there is an abnormal CLL panel, positive for monoallelic or biallelic deletion of 13q. The pathologist noted a CLL related clone was detected, but there was no definitive diagnosis of CLL on the peripheral blood. No bone marrow biopsy was performed. However, the managing physician noted this was Rai Stage I CLL/SLL with adenopathy in the neck.

The SSDI Manual notes, “Rai stage is only applicable for CLL, in which the bone marrow and/or peripheral blood are involved (primary site C421 for bone marrow, see Hematopoietic Manual, Module 3: PH 5, 6).” Should primary site default to C421 if the physician provides a Rai Stage in the absence of definitive peripheral blood or bone marrow involvement documented in the medical record?

Pathology--Final description: Uterus, cervix, bilateral fallopian tubes and ovaries, total hysterectomy and bilateral salpingo-oophorectomy: Endometrial adenocarcinoma, endometrioid type, well differentiated, FIGO 1/3. Myometrial invasion: focal myometrial invasion (30% of myometrium) Tumor size: 2 x2 cm Tumor site: Fundus, exophytic/polypoid lesion Gross description: The 3.0 cm in length by 2.5 cm in diameter triangular endometrium is tan-red and smooth with a 2.0 x 2.0 cm tan-pink, exophytic fundic mass which extends on to both anterior and posterior aspects, 4.1 cm from the os.

Patient presented with large bulky mediastinal mass. CT showed no pleural effusion. Findings also show non-specific parenchymal lung nodules. Biopsy of mediastinal mass showed malignant B-cell lymphoma of follicle center cell origin. Abdomen /Pelvis CT showed borderline lymph nodes in bifurcation. Clinical diagnosis was probable stage 3 if not 4 lymphoma. Per lymphoma guidelines, if extra-nodal primary site is assigned to the extranodal site if an extra-nodal site and its regional lymph nodes are involved. Would the parenchymal lung nodules be indicative of pulmonary involvement? If so, would primary site be lung? Or, would the parenchymal nodules be stage 4 disease and primary site be assigned to lymph nodes?

ICD-O-3 Rule H states to use the topography code provided when a topographic site is not stated in the diagnosis. This topography code should be ignored if the tumor arose in another site. For the following brain and central nervous system (CNS) examples, should the suggested sub-site codes be assigned based on the histology, or should the primary sites be coded as C719 (posterior fossa or suprasellar brain) since the only information available was a tumor in these non-specific sites?

Example 1: Resection of a posterior fossa tumor proved medulloblastoma, WNT-activated. Although medulloblastoma has a site-associated code in the ICD-O-3 (C716, cerebellum), the only information available is that this was a posterior fossa tumor (C719).

Example 2: Resection of a suprasellar brain tumor proved pineoblastoma. The pathologist labeled this as a brain tumor, suprasellar. Although pineoblastoma has a site-associated code in the ICD-O-3 (C753, pineal gland), the only information available is that this was a suprasellar brain tumor (C719).

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.

Should it be coded to where the testis was physically at the time of diagnosis (C621), or should it be coded to C620 to reflect the increased risk for developing malignancy in an undescended testis?