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This is a reportable case. Even though the pathology from the orchiectomy stated mature teratoma, NOS, the presence of lymph node metastases proves that this tumor is malignant. Code the histology as 9065/3 [germ cell tumor non-seminomatous].

The majority of germ cell tumors show the presence of multiple histologies. While the original tumor showed only mature teratoma, there were obviously yolk sac cells that were not detected on the sections taken from the primary tumor. Both teratoma and yolk sac are germ cell tumors. This explains why the pathologist gave you the diagnosis of germ cell tumor. The classification of "non-seminomatous" simply means that there was no seminomas present in the mixture of germ cell histologies.

Abstract as a single primary: dermatofibrosarcoma protuberans [8832/3] of the left upper inner arm [C446] diagnosed in August 2008.

The rationale for this answer was provided by subject matter experts. The physician specialists for soft tissue and bone replied as follows:

Low-grade sarcomas tend to recur locally. Because this tumor recurred in same area, i.e. scar of prior surgery, and recurred in this period of time, this is a local recurrence. Dermatofibrosarcoma Protuberans is a low grade tumor which can recur many years following tumor excision.

This is a metaplastic carcinoma as stated in the path diagnosis. Rule H14 applies. Assign code 8575/3. According to the WHO Classification, metaplastic carcinoma is a general term for a group of neoplasms characterized by a mixture of adenocarcinoma with dominant areas of spindle cell, squamous, and/or mesenchymal differentiation.

Use the Multiple Primary and Histology Coding Rules Manual for cases diagnosed 2007 or later to determine the histology for this case. Code histology to 8575/3 [metaplastic carcinoma] as stated in the pathology diagnosis.

Open the Multiple Primary and Histology Coding Rules manual. Choose one of the three formats (i.e., flowchart, matrix or text) under the Breast Histo rules determine histology for the case.

Go to the SINGLE TUMOR: INVASIVE CARCINOMA ONLY module. The rules are intended to be reviewed in consecutive order within the module from Rule H10 to Rule H19. You stop at the first rule that applies to the case you are processing.

Code the histology when only one histologic type is identified. According to the WHO Classification, metaplastic carcinoma is a general term for a group of neoplasms characterized by a mixture of adenocarcinoma with dominant areas of spindle cell, squamous, and/or mesenchymal differentiation.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Per Rule M10, abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm AND there is a second diagnosis of an acute neoplasm more than 21 days after the chronic diagnosis. Two primaries should be accessioned for this case: refractory anemia with excess blasts (RAEB) [9983/3] (a chronic neoplasm), and acute myeloid leukemia [9861/3] (an acute neoplasm).

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

For cases diagnosed 2007 or later, code one primary with histology and behavior coded to 8522/2 [intraductal carcinoma and lobular carcinoma in situ].

The steps used to arrive at this decision are as follows

Open the Multiple Primary and Histology Coding Rules manual. Choose one of the three formats (i.e., flowchart, matrix or text) under the Breast Histology rules. The module you use depends on the behavior and number of tumors identified in the primary site. The information provided does not specify whether this was a single tumor with DCIS and LCIS or multiple tumors with DCIS and LCIS. In this case, the number of tumors does not change the histology code for this patient. For this example, assume this disease process was a single tumor.

Start at the SINGLE TUMOR: In Situ Carcinoma Only module. The rules are intended to be reviewed in consecutive order from Rule H1 to Rule H8. Stop at the first rule that applies to the case you are processing. Code the histology as 8522/2 (intraductal carcinoma and lobular carcinoma in situ) when there is a combination of in situ lobular (LCIS) [8520] and intraductal carcinoma (DCIS).

Do not code the behavior as invasive in this case. The pathologist indicated that these findings were "suspicious" but not definite in the microscopic examination. If the pathologist decided that this was truly an invasive tubular element, it would have been included in the final diagnosis.

Code histology to 8153/3 [malignant gastrinoma].

According to the WHO Classification of Tumors of the Digestive System, pages 64-65, carcinoid is a synonym for gastric neuroendocrine tumor (NET) and gastrinoma is synonymous with gastrin-producing NET. Gastrin-producing NET (gastrinoma) is coded 8153/3.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

This case is accessioned as two primaries per Rule M10 which states to abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm and there is a second diagnosis of an acute neoplasm more than 21 days after the chronic diagnosis. The first primary is CLL [9823/3] and it is a chronic neoplasm. The second primary is diffuse large B-cell lymphoma (DLBCL) [9680/3] and it is an acute neoplasm.

Richter syndrome (RS) is a complication of B cell chronic lymphocytic leukemia (CLL) or hairy cell leukemia (HCL) in which the leukemia changes into DLBCL. There is also a less common variant in which the CLL changes into a Hodgkin lymphoma. Richter's transformation affects about 5% of CLL patients. Richter syndrome is listed under the Alternate Names section in the Heme DB for diffuse large B-cell lymphoma [9680/3].

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

The expert pathologist recommends coding histology to 8980/3 [Carcinosarcoma] for this combination histology.

Expert consultation: The designation "carcinosarcoma" is given when the pathology shows differentiation in both the sarcomatous (rhabdomyoblastic) and carcinomatous (adenoca) elements. This is emphasized in the path for this case with the term "biphasic." The term "heterologous" mean that the sarcomatous component is of a type not normal to lung. Rhabdomyoblastic means skeletal muscle differentiation. Because skeletal muscle is not normally found in lung it is heterologous. If it were smooth muscle, it would be homologous because smooth muscle is found in lung (as a part of the bronchi).

Multiplicity Counter field was initially coded 02. Change the code to 03 because the subsequent, additional tumor was determined to be the same primary. Update the Multiplicity Counter field only once. If additional tumors are determined to be the same primary for this case, it is not necessary to update this field again.

Date of Multiple Tumors field was initially coded November 2007. Multiple tumors were present at the time of the initial diagnosis. Do not change the date of this field when additional tumors are subsequently diagnosed. This data item reflects the earliest date that multiple tumors were present. See example 2 under #3 on page 81 of the 2010 SEER manual.