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Neuroendocrine (Merkel cell) carcinoma of the retroperitoneum with no identifiable primary site.

Boghossian V, Owen ID, Nuli B, Xiao PQ - World J Surg Oncol (2007)

Bottom Line: Since Merkel cell precursors have never been identified within lymph nodes, the latter theory seems more befitting.Moreover, metastasis to the retroperitoneal lymph nodes has been reported as relatively common when compared to other sites such as liver, bone, brain and skin.Wide local excision of the primary tumor is the surgical treatment of choice for localized disease.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Surgery, Brooklyn Hospital Center, 121 Dekalb Ave, Brooklyn, New York, NY 11201, USA. vanboghossian@aol.com

ABSTRACT

Background: Neuroendocrine carcinoma is an aggressive neoplasm that mainly affects elderly Caucasians and typically arises in sun-exposed areas of the skin. The disease is rather rare and only a relatively few cases present with no apparent primary lesion.

Case presentation: We report a case of an 81-year-old Caucasian male with neuroendocrine carcinoma, which initially presented as a large retroperitoneal mass. Pathological and immunohistochemical analysis of the transabdominal CT-guided biopsy specimen revealed tissue consistent with neuroendocrine carcinoma. The patient underwent exploratory laparotomy and the mass was successfully excised along with an associated mesenteric lymph node.

Discussion: There are currently two possible explanations for what occurred in our patient. First, the retroperitoneal mass could be a massively enlarged lymph node where precursor cells became neoplastic. This would be consistent with a presumptive diagnosis of primary nodal disease. Alternatively, an initial skin lesion could have spontaneously regressed and the retroperitoneal mass represents a single site of metastasis. Since Merkel cell precursors have never been identified within lymph nodes, the latter theory seems more befitting. Moreover, metastasis to the retroperitoneal lymph nodes has been reported as relatively common when compared to other sites such as liver, bone, brain and skin.

Conclusion: Wide local excision of the primary tumor is the surgical treatment of choice for localized disease. We propose that further studies are needed to elucidate the true efficacy of chemotherapy in conventional as well as unconventional patients with neuroendocrine carcinoma.

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CT scan of pelvis showing right lower quadrant mass.
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Figure 1: CT scan of pelvis showing right lower quadrant mass.

Mentions: On examination, vital signs were normal. The abdomen was non-distended with a 3-cm, midline, infraumbilical scar. Tenderness was noted on deep palpation of the right lower quadrant and a large mass was felt measuring approximately 5-cm × 5-cm. The lower margin could not be felt. The mass was also palpable on digital rectal exam. Femoral pulses were normal. Laboratory values were unremarkable. Fecal occult blood test was negative. Colonoscopy showed the cecum high in the right upper quadrant with no evidence of a cecal mass. Contrast computed tomography (CT) of both abdomen and pelvis (Figure 1) revealed a 5-cm × 5-cm × 7.5-cm, enhancing, rounded, heterogeneous, well-defined mass in the right lower quadrant, ventral to the psoas muscle and displacing the terminal ileum anteriorly.


Neuroendocrine (Merkel cell) carcinoma of the retroperitoneum with no identifiable primary site.

Boghossian V, Owen ID, Nuli B, Xiao PQ - World J Surg Oncol (2007)

CT scan of pelvis showing right lower quadrant mass.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2117014&req=5

Figure 1: CT scan of pelvis showing right lower quadrant mass.
Mentions: On examination, vital signs were normal. The abdomen was non-distended with a 3-cm, midline, infraumbilical scar. Tenderness was noted on deep palpation of the right lower quadrant and a large mass was felt measuring approximately 5-cm × 5-cm. The lower margin could not be felt. The mass was also palpable on digital rectal exam. Femoral pulses were normal. Laboratory values were unremarkable. Fecal occult blood test was negative. Colonoscopy showed the cecum high in the right upper quadrant with no evidence of a cecal mass. Contrast computed tomography (CT) of both abdomen and pelvis (Figure 1) revealed a 5-cm × 5-cm × 7.5-cm, enhancing, rounded, heterogeneous, well-defined mass in the right lower quadrant, ventral to the psoas muscle and displacing the terminal ileum anteriorly.

Bottom Line: Since Merkel cell precursors have never been identified within lymph nodes, the latter theory seems more befitting.Moreover, metastasis to the retroperitoneal lymph nodes has been reported as relatively common when compared to other sites such as liver, bone, brain and skin.Wide local excision of the primary tumor is the surgical treatment of choice for localized disease.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Surgery, Brooklyn Hospital Center, 121 Dekalb Ave, Brooklyn, New York, NY 11201, USA. vanboghossian@aol.com

ABSTRACT

Background: Neuroendocrine carcinoma is an aggressive neoplasm that mainly affects elderly Caucasians and typically arises in sun-exposed areas of the skin. The disease is rather rare and only a relatively few cases present with no apparent primary lesion.

Case presentation: We report a case of an 81-year-old Caucasian male with neuroendocrine carcinoma, which initially presented as a large retroperitoneal mass. Pathological and immunohistochemical analysis of the transabdominal CT-guided biopsy specimen revealed tissue consistent with neuroendocrine carcinoma. The patient underwent exploratory laparotomy and the mass was successfully excised along with an associated mesenteric lymph node.

Discussion: There are currently two possible explanations for what occurred in our patient. First, the retroperitoneal mass could be a massively enlarged lymph node where precursor cells became neoplastic. This would be consistent with a presumptive diagnosis of primary nodal disease. Alternatively, an initial skin lesion could have spontaneously regressed and the retroperitoneal mass represents a single site of metastasis. Since Merkel cell precursors have never been identified within lymph nodes, the latter theory seems more befitting. Moreover, metastasis to the retroperitoneal lymph nodes has been reported as relatively common when compared to other sites such as liver, bone, brain and skin.

Conclusion: Wide local excision of the primary tumor is the surgical treatment of choice for localized disease. We propose that further studies are needed to elucidate the true efficacy of chemotherapy in conventional as well as unconventional patients with neuroendocrine carcinoma.

Show MeSH
Related in: MedlinePlus