Primary site--Heme & Lymphoid Neoplasms: What the primary site for a diagnosis of Langerhans cell histiocytosis with multifocal multisystem involvement of the skin, chest, CNS and thyroid, but no evidence of involvement on a bone scan?
Langerhans Cell Histiocytosis (LCH) includes three major groups:
Unifocal: Slowly progressing disease, involves one organ.
Multifocal unisystem: Involves several organs in one system.
Multifocal multisystem: Involves several organs in two or more systems.
When the disease is both multifocal and multisystem, code the primary site to unknown [C809] because there is no way to identify the origin of the neoplasm in this situation.
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.
Primary Site--Kaposi Sarcoma: Would the following Kaposi primaries be examples of cases not coded to skin for primary site? See discussion.
1. KS developed initially as a lesion in the oral cavity and followed by the appearance of skin lesions.
2. KS found in a resected parotid gland with metastasis to the parotid gland lymph node. No skin lesions identified.
3. KS discovered in a biopsied 3 cm axillary lymph node. Clinically, the patient had hepatosplenomegaly, ascites, and extensive mesenteric lymph nodes. (No mention of skin.)
Code the Primary Site field as follows:
1. C44.9 [Skin, NOS] as the default value when lesions develop simultaneously in skin and non-skin areas.
2. C07.9 [Parotid gland]
3. C44.9 [Skin, NOS] as the default value when there is no mention of lesions in the skin or other primary site.
Edward Klatt states in Practical AIDS Pathology, "...Visceral Kaposi (involving one or more internal organ sites) is also present in three-fourths of cases, but may not be diagnosed prior to autopsy. Visceral involvement frequently includes the lung, lymph nodes and gastro-intestinal tract."
Primary site--Kidney, renal pelvis: Should primary site be coded C809 [unknown] or C649 [kidney] when a patient is diagnosed with renal cell carcinoma in a transplanted kidney?
Code the primary site to C649 [kidney]. Per the SEER Manual, code the site where the neoplasm originated. There are no separate instructions for coding primary site for transplanted organs. This patient's renal cell carcinoma originated in the kidney [C649].
Primary site--Lung: What is the code for primary site if a small cell carcinoma presents as mediastinal masses?
Code the primary site to main bronchus [C340].
Primary small cell carcinoma in the thymus/mediastinum is rare. A bronchial lesion with extension into the mediastinum is much more likely. In a case like this, it is difficult to be sure exactly where the tumor arose, however, it is recommended the default site be the main bronchus when there is no information to the contrary.
Primary Site--Lymphoma: How should this field be coded when a diffuse large B-cell lymphoma is found in the femur and in the soft tissue of the anterior chest wall but all CT scans are negative for lymphadenopathy?
For cases diagnosed prior to 1/1/2010:Code the Primary Site field to C809 [Unknown primary site]. The primary site of diffuse large B cell lymphoma can be either nodal or extranodal. The case described above is likely extranodal because there is no evidence of lymph node involvement. Because the extranodal site of origin is unknown, code the Primary Site to C809.
For cases diagnosed 2010 forward, 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.
Primary Site--Lymphoma: How should you code the primary site for a lymphoma that presents with involvement of an extranodal site and regional lymph nodes? See discussion.
1. Lymphoma involves the spleen and the splenic lymph nodes.
2. MALT Lymphoma involves the stomach and the gastric and iliac lymph nodes.
1. Code the Primary Site field to C42.2 [spleen].
2. Code the Primary Site field to C16._ [stomach].
When lymphoma presents in an extranodal site and in the regional lymph nodes for that extranodal site, code the Primary Site field to the extranodal site. The typical disease process is that lymphoma can spread from an extranodal organ to its regional lymph nodes. It cannot metastasize from the regional lymph node to the extranodal organ. The exception to this would be if the lymph nodes presented as one large mass that extended into the regional organ.
Primary Site--Lymphoma: Is the primary site likely to be extranodal for a lymphoma that presents in an extranodal site and lymph nodes which are regional for that site? Is the primary site also likely to be extranodal if an extranodal site and lymph nodes are excised? See discussion.
Example: Work-up included a negative CXR. A CT showed multiple dilated loops of small bowel consistent with obstruction and nodular prominence at the base of bladder. Laparotomy with resection of small bowel and multiple biopsies of enlarged mesentric lymph nodes performed. Final path diagnosis: Lymphoma in a "mesenteric mass" and in "small bowel." There was no mention of lymph nodes in the final diagnosis and the detailed micro described the mesenteric mass as just adipose tissue replaced by lymphoma. However, the gross for that specimen states 4 lymph nodes were found in the fat. The small bowel micro described an ulcerated lesion of the small bowel extending into muscularis.
For cases diagnosed prior to 1/1/2010:Code the Primary Site field to C17.9 [small bowel] for the example. When an extranodal organ and that organ's regional nodes are involved, the extranodal site is most likely the primary, unless there is extension from the regional nodes to the organ. If the primary site cannot be determined for a lymphoma diagnosed in both a nodal and extranodal site, code to C77.9 [lymph nodes NOS].
For cases diagnosed 2010 forward, 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.
Primary Site--Melanoma: How would this field be coded for a pleural effusion consistent with metastatic melanoma and "no skin lesions?"
Code primary site as C44.9 [Skin, NOS]. ICD-O-3 does not list a suggested site code for 8720/3 because melanoma can arise in other parts of the body. However, C44.9 [Skin, NOS] is the default when the primary cannot be found.
Primary site--Melanoma: Should melanoma of the nipple be coded to C50.0 [Nipple] or C44.5 [Skin of the trunk]?
Code to C44.5 [skin of trunk]. External melanoma is an epidermal malignancy, beginning in melanocytes in the basal layer of the epidermis. C50.0 excludes skin of breast.
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