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Default the coding of the Primary Site field for chordomas to the bone where the tumor began in the body if the primary site is not clearly stated to be soft tissue. Bone is often the primary site for chordomas.

Based on advice from pathologist consultants: This is one of those situations where we can be quite comfortable with a default, in this case to bone, not soft tissue. Chordoma is a tumor arising in the nucleus pulposis, presumably from remnants of notochord - thus its exclusive origin is in the sacrococcygeal region, spheno-occipital region, and vertebral bodies, otherwise known collectively as the axial skeleton. Any "chordoma" in soft tissue (with no relationship to axial skeleton) is probably a myxoid chondrosarcoma or parachordoma (extremely rare).

For cases diagnosed 1998-2003: The data item "Surgery of Primary Site" is intended to record only surgeries of the primary site. If the primary site is unknown or ill-defined, it is impossible to determine whether or not a particular surgery was performed on the primary site. "Surgical Procedure of Other Site" collects much less specific information; however, this is the correct data item to record surgery performed when the primary site is unknown or ill-defined.

For the case example, code the primary site as C76.0 [Head, face or neck, NOS]. Code Surgery of Primary Site as 98 [All unknown and ill-defined disease sites, with or without surgical treatment]. Code Surgical Procedure of Other Site as 1 [Non-primary surgical procedure performed].

For cases diagnosed prior to 1/1/2010:Code the primary site to tonsil (C099). This advanced stage lymphoma involves an extranodal site (tonsil) and that site's regional lymph nodes (cervical). The lymphoma has also spread to non-regional lymph nodes (mediastinal and mesenteric). Code the tonsillectomy as surgery of primary site.

For cases diagnosed 1/1/10 and later, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ.

For cases diagnosed prior to 1/1/2010:Primary site:

Code the primary site to C421 [bone marrow] per primary site coding instructions for leukemia in the 2007 SEER manual, page 70.

Surgery of other site:

Since all surgical procedures for hematopoietic diseases are coded in the data item Surgery of Other Site, assign code 1 [Nonprimary surgical procedure performed].

For cases diagnosed 1/1/10 and later, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ.

For tumors diagnosed 1998-2003:

Based on the information provided:

The topography code for this lymphoma is C772 [Intra-abdominal lymph nodes].

Code SEER Summary Stage 2000 to 5 [Regional NOS].

Code EOD Extension to 20. More than one lymph node region below the diaphragm is involved (retroperitoneal and peritoneal). The organs mentioned are not involved by the lymphoma. The bulk of the masses is causing obstruction by displacing and/or encasing organs.

A physician description of "bulky disease" would not change the EOD for this case.

This case is reportable. Code the primary site to C25.9 [pancreas, NOS] and the histology to 8240/3 [neuroendocrine tumor (NET), Grade 1].

Per the 2012 SEER Manual, code the site in which the primary tumor originated. This neoplasm arose in pancreatic tissue and will behave accordingly, even though this pancreatic tissue is not located in the usual place.

Pancreatic endocrine and neuroendocrine neoplasms are essentially the same thing. However, they are described in two different WHO classifications; the endocrine classification and the digestive system classification. The digestive system classification is more recent, and is preferred by our expert pathologist consultant. The term "neuroendocrine" is to be used now, rather than "endocrine." In the pancreas, "well differentiated endocrine tumor" is synonymous with "neuroendocrine tumor (NET) Grade 1" and is coded 8240/3.

Because the site is specifically stated to "skin" of nipple [C44.5], this case is not reportable.

If possible, you may wish to confirm the type of biopsy performed. If the biopsy was done by FNA or needle biopsy, the biopsy tissue should contain a full-thickness of skin and subcutaneous breast (nipple) tissue. If that is the case, this tumor would likely be a reportable squamous cell carcinoma of nipple [C50.0]. If, however, this was a punch biopsy it is more likely a non-reportable squamous cell carcinoma of the skin [C44.5].

Code the breast subsite from the pathology report (C501, central).

The priority order for coding subsite from conflicting reports is

1. Pathology report

2. Operative report

3. Physical examination

4. Mammogram, ultrasound

The primary site of inflammatory breast carcinoma is coded to C509 when there is no palpable tumor.

For cases diagnosed prior to 1/1/2010:These are not "impossible" site/histology edits. You can override them. However, if the lymph nodes are involved and a lymphoma histology is available, the lymphoma histology should be coded rather than leukemia histology. For example, assign histology code 9670 (Malignant lymphoma, small B lymphocytic, NOS) instead of 9823 (B-cell chronic lymphocytic leukemia/small cell lymphocytic lymphoma) if the disease is identified in the lymph nodes.

For cases diagnosed 1/1/10 and later, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ.