Goblet Cell Carcinoid Presenting as Appendicitis
Radiology Case Reports Volume 2, Issue 2, 2007
Goblet Cell Carcinoid Presenting as Appendicitis 1.5 Years After a Normal CT Scan David Dang, Larry A. DeRenne
A 60-year-old man presented with signs and symptoms of acute appendicitis and was found to have goblet cell carcinoid (GCC) of the appendix. The patient had no evidence of appendiceal disease on a computed tomography study one and a half year before. Due to the rarity of this tumor, previous case reports and series did not discuss the time course of the tumor's onset. This case report provides evidence that GCC can occur within a period of 1.5 years. This case report also discusses relevant histopathological aspects of GCC as pertain to cross-sectional imaging findings and illustrates the importance of considering other types of appendiceal disease in the diagnosis of a patient presenting with acute appendicitis. Case Report A 60-year-old man presented to the emergency room for a second time within one week for a two-week history of intense lower quadrant abdominal pain. The patient was not febrile and had no leukocytosis. A CT study showed an enlarged distal appendix with a 1.8 x 3.8 cm fluid-like or cystic lesion with mild mural thickening (Figure 1). There was also mild wall thickening of the more proximal appendix. There were no enlarged periappendiceal lymph nodes. The patient had a normal appendix on a CT exam obtained approximately one and a half year before (Figure 2). A diagnosis of acute appendicitis was made. The patient had a history of previous small bowel resection and exploratory laprotomy, nine and twenty-two years Figure 1A. 60-year-old man with goblet cell carcinoid. CT at presentation shows enlarged distal appendix containing a 1.8 x 3.8 cm cystic lesion (Hounsfield unit of 10, consistent with fluid) (thick arrow). The thin arrow in A indicates normal collapsed terminal ileum.
Citation: Dang D, DeRenne LA. Goblet cell carcinoid presenting as appendicitis 1.5 years after a normal CT scan. Radiology Case Reports. [Online] 2007;2:100. Copyright: © 2007 David Dang. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs 2.5 License, which permits reproduction and distribution, provided the original work is properly cited. Commercial use and derivative works are not permitted. Abbreviations: CT, computed tomography, GCC, goblet cell carcinoid
ago, respectively. The reason for these surgeries was small bowel obstruction, otherwise unknown. Due to prior surgeries and adhesions on laproscopy, open appendectomy was performed. The distal appendix was found to be dilated with tan-pink and markedly eroded mucosa with a wall thickness of 0.3 cm. The remainder of the resected
David Dang ([email protected]) is in the Department of Radiology, The Ohio State University Medical Center, Columbus, Ohio, United States. Published: June 27, 2007 DOI: 10.2484/rcr.v2i2.100
RCR Radiology Case Reports | radiology.casereports.net
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Goblet Cell Carcinoid Presenting as Appendicitis
Figure 1B. Mild mural thickening around the cystic lesion and in the more proximal appendix (arrowheads) are pre-
Figure 2. CT study approximately 1.5 years before presentation shows normal appendix, without evidence of inflam-
B
A Figure 3A. 60-year-old man with goblet cell carcinoid. High power photomicrograph of chromogranin immunostain highlights the tumor cells that exhibit neuroendocrine differentiation.
Figure 3B. 60-year-old man with goblet cell carcinoid. High power photomicrograph of an H & E stained section illustrates the signet ring cells of the neoplasm. Note the peripheral location of the cell's nuclei with respect to the cytoplasm.
appendix had tan mucosa and wall thickness of 0.7 cm. Pathologic examination showed features of goblet cell carcinoid (Figure 3) with transmural extension into the mesoappendiceal adipose tissue with perineural and intravascular involvement of the mesoappendiceal adipose tissue and subserosa. Immunohistochemical analysis revealed positivity for AE1/AE3 cytokeratins, polyclonal CEA and chromogranin, which were consistent with goblet cell carcinoid.
cluding adenocarcinoid, mucinous carcinoid, intermediate type of carcinoid, crypt cell carcinoma, amphicrine (endoexocrine) neoplasia, composite tumor and microglandular carcinoma. All names except GCC have been omitted from the current World Health Organization (WHO) classification (1). Goblet cell carcinoid tumors accounts for 6% of appendiceal carcinoid tumors. Histologically, GCC has features of both epithelial and carcinoid tumors (2) and is considered intermediate between classic appendiceal carcinoid tumors and adenocarcinomas. Although some authors believe that this tumor is of low-grade malignancy (3), it has a more aggressive natural history than the classic carcinoid tumors with variable malignant potential (4). At diagnosis the tu-
Discussion Goblet cell carcinoid is nearly exclusively a tumor of the appendix. It has been reported under different names, in-
RCR Radiology Case Reports | radiology.casereports.net
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2007:2(2):69-71
Goblet Cell Carcinoid Presenting as Appendicitis mor most commonly has transmural extension with diffuse infiltration into the periappendiceal fat and perineural invasion. Although simple appendectomy may be adequate for most patients, some patients may require a more radical procedure including right hemicolectomy, particularly if there is involvement at the base of the appendix with cecal wall inflammation, lymph node metastasis, tumor size greater than 2 cm, or high-grade tumor histology (5, 8). In most case reports and also in the patient reported here, the diagnosis was only made at histological analysis; and appendectomy was already performed when a diagnosis is reached. A second operation may be needed for some patients if the tumor margin is not clear as recurrence and metastasis are possible. Because of the possibility of ovarian metastasis, bilateral oophorectomy has also been advocated in female patients (6). Anatomically, goblet cell carcinoid tumors are usually located near the tip of the appendix. Macroscopically, usually there is no well-defined mass. There may be areas of indurations in the wall or diffuse fibrous thickening of the appendix that may provide clues to this tumor at surgery. Cross-sectional imaging typically shows mild diffuse mural thickening, reflecting the infiltrative nature of the tumor (1). As shown in Figure 1 there is a fluid-like or cystic lesion at the tip of the appendix. Proximal to this is mural thickening of the appendix (Figure 1B). Both of these findings were confirmed at pathologic evaluation. The cystic portion was likely a mucocele, which occasionally may be present around the neoplastic portion of the appendix (3). In this patient it caused mucosal erosions and inflammation and was likely the cause of the patient's presenting symptoms. A rare complication of appendico-vesical fistula may also occur, causing urological symptoms (7). In this case the patient had no evidence of macroscopic disease of the appendix on cross-sectional imaging one and a half year before. A comprehensive review of the literature from 1966 to 2004 by Pahlavan and Kanthan did not discuss the time course or onset of GCC in patients who had no antecedent appendiceal abnormality (8). Over the past two decades cross-sectional imaging has played a pivotal role in the management of patients presenting with signs and symptoms of acute appendicitis. As the number of patients receiving imaging study and the number of imaging study per patient both increase, the ability to follow the natural history of a disease improves. This case report provides evidence that GCC can occur within a period of 1.5 years. This case report also indicates the importance of considering appendiceal neoplasm in the diagnosis of a patient presenting with signs and symptoms of appendicitis even if a previous CT was negative for such disease. A major presentation of tumors of the appendix is acute appendicitis as the lumen may be obstructed, causing inflammation. It is important to characterize cecal involvement as a more radical surgical approach may be considered instead of the typical simple appendectomy.
RCR Radiology Case Reports | radiology.casereports.net
References 1. Pickhardt PJ, Levy AD, Rohrmann CA Jr, Kende AI. Primary neoplasms of the appendix: radiologic spectrum of disease with pathologic correlation. Radiographics. 2003;23:645-62. [PubMed] 2. Carr NJ, Remotti H, Sobin LH. Dual carcinoid/ epithelial neoplasia of the appendix. Histopathology. 1995;27:557-62. [PubMed] 3. Chen V, Qizilbash AH. Goblet cell carcinoid tumor of the appendix. Report of five cases and review of the literature. Arch Pathol Lab Med. 1979;103:180-2. [PubMed] 4. Butler JA, Houshiar A, Lin F, Wilson SE. Goblet cell carcinoid of the appendix. Am J Surg 1994;168:685-7. [PubMed] 5. Varisco B, McAlvin B, Dias J, Franga D. Adenocarcinoid of the appendix: is right hemicolectomy necessary? A meta-analysis of retrospective chart reviews. Am Surg. 2004;70:593-9. [PubMed] 6. Mandai M, Konishi I, Tsuruta Y, Suginami N, Kusakari T, Iwasaki T, Fujii S. Krukenberg tumor from an occult appendiceal adenocarcinoid: a case report and review of the literature. Eur J Obstet Gynecol Reprod Biol. 2001;97:90-5. [PubMed] 7. Di Paola M, Stockwell WS. Appendico-vesical fistula due to an appendix abscess with associated goblet cell carcinoid of the appendix. Br J Urol. 1976;48:436. [PubMed] 8. Pahlavan PS, Kanthan R. Goblet cell carcinoid of the appendix. World J Surg Oncol. 2005;3:36. [PubMed]
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Radiology Case Reports Volume 2, Issue 2, 2007
Goblet Cell Carcinoid Presenting as Appendicitis 1.5 Years After a Normal CT Scan David Dang, Larry A. DeRenne
A 60-year-old man presented with signs and symptoms of acute appendicitis and was found to have goblet cell carcinoid (GCC) of the appendix. The patient had no evidence of appendiceal disease on a computed tomography study one and a half year before. Due to the rarity of this tumor, previous case reports and series did not discuss the time course of the tumor's onset. This case report provides evidence that GCC can occur within a period of 1.5 years. This case report also discusses relevant histopathological aspects of GCC as pertain to cross-sectional imaging findings and illustrates the importance of considering other types of appendiceal disease in the diagnosis of a patient presenting with acute appendicitis. Case Report A 60-year-old man presented to the emergency room for a second time within one week for a two-week history of intense lower quadrant abdominal pain. The patient was not febrile and had no leukocytosis. A CT study showed an enlarged distal appendix with a 1.8 x 3.8 cm fluid-like or cystic lesion with mild mural thickening (Figure 1). There was also mild wall thickening of the more proximal appendix. There were no enlarged periappendiceal lymph nodes. The patient had a normal appendix on a CT exam obtained approximately one and a half year before (Figure 2). A diagnosis of acute appendicitis was made. The patient had a history of previous small bowel resection and exploratory laprotomy, nine and twenty-two years Figure 1A. 60-year-old man with goblet cell carcinoid. CT at presentation shows enlarged distal appendix containing a 1.8 x 3.8 cm cystic lesion (Hounsfield unit of 10, consistent with fluid) (thick arrow). The thin arrow in A indicates normal collapsed terminal ileum.
Citation: Dang D, DeRenne LA. Goblet cell carcinoid presenting as appendicitis 1.5 years after a normal CT scan. Radiology Case Reports. [Online] 2007;2:100. Copyright: © 2007 David Dang. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs 2.5 License, which permits reproduction and distribution, provided the original work is properly cited. Commercial use and derivative works are not permitted. Abbreviations: CT, computed tomography, GCC, goblet cell carcinoid
ago, respectively. The reason for these surgeries was small bowel obstruction, otherwise unknown. Due to prior surgeries and adhesions on laproscopy, open appendectomy was performed. The distal appendix was found to be dilated with tan-pink and markedly eroded mucosa with a wall thickness of 0.3 cm. The remainder of the resected
David Dang ([email protected]) is in the Department of Radiology, The Ohio State University Medical Center, Columbus, Ohio, United States. Published: June 27, 2007 DOI: 10.2484/rcr.v2i2.100
RCR Radiology Case Reports | radiology.casereports.net
69
2007:2(2):69-71
Goblet Cell Carcinoid Presenting as Appendicitis
Figure 1B. Mild mural thickening around the cystic lesion and in the more proximal appendix (arrowheads) are pre-
Figure 2. CT study approximately 1.5 years before presentation shows normal appendix, without evidence of inflam-
B
A Figure 3A. 60-year-old man with goblet cell carcinoid. High power photomicrograph of chromogranin immunostain highlights the tumor cells that exhibit neuroendocrine differentiation.
Figure 3B. 60-year-old man with goblet cell carcinoid. High power photomicrograph of an H & E stained section illustrates the signet ring cells of the neoplasm. Note the peripheral location of the cell's nuclei with respect to the cytoplasm.
appendix had tan mucosa and wall thickness of 0.7 cm. Pathologic examination showed features of goblet cell carcinoid (Figure 3) with transmural extension into the mesoappendiceal adipose tissue with perineural and intravascular involvement of the mesoappendiceal adipose tissue and subserosa. Immunohistochemical analysis revealed positivity for AE1/AE3 cytokeratins, polyclonal CEA and chromogranin, which were consistent with goblet cell carcinoid.
cluding adenocarcinoid, mucinous carcinoid, intermediate type of carcinoid, crypt cell carcinoma, amphicrine (endoexocrine) neoplasia, composite tumor and microglandular carcinoma. All names except GCC have been omitted from the current World Health Organization (WHO) classification (1). Goblet cell carcinoid tumors accounts for 6% of appendiceal carcinoid tumors. Histologically, GCC has features of both epithelial and carcinoid tumors (2) and is considered intermediate between classic appendiceal carcinoid tumors and adenocarcinomas. Although some authors believe that this tumor is of low-grade malignancy (3), it has a more aggressive natural history than the classic carcinoid tumors with variable malignant potential (4). At diagnosis the tu-
Discussion Goblet cell carcinoid is nearly exclusively a tumor of the appendix. It has been reported under different names, in-
RCR Radiology Case Reports | radiology.casereports.net
70
2007:2(2):69-71
Goblet Cell Carcinoid Presenting as Appendicitis mor most commonly has transmural extension with diffuse infiltration into the periappendiceal fat and perineural invasion. Although simple appendectomy may be adequate for most patients, some patients may require a more radical procedure including right hemicolectomy, particularly if there is involvement at the base of the appendix with cecal wall inflammation, lymph node metastasis, tumor size greater than 2 cm, or high-grade tumor histology (5, 8). In most case reports and also in the patient reported here, the diagnosis was only made at histological analysis; and appendectomy was already performed when a diagnosis is reached. A second operation may be needed for some patients if the tumor margin is not clear as recurrence and metastasis are possible. Because of the possibility of ovarian metastasis, bilateral oophorectomy has also been advocated in female patients (6). Anatomically, goblet cell carcinoid tumors are usually located near the tip of the appendix. Macroscopically, usually there is no well-defined mass. There may be areas of indurations in the wall or diffuse fibrous thickening of the appendix that may provide clues to this tumor at surgery. Cross-sectional imaging typically shows mild diffuse mural thickening, reflecting the infiltrative nature of the tumor (1). As shown in Figure 1 there is a fluid-like or cystic lesion at the tip of the appendix. Proximal to this is mural thickening of the appendix (Figure 1B). Both of these findings were confirmed at pathologic evaluation. The cystic portion was likely a mucocele, which occasionally may be present around the neoplastic portion of the appendix (3). In this patient it caused mucosal erosions and inflammation and was likely the cause of the patient's presenting symptoms. A rare complication of appendico-vesical fistula may also occur, causing urological symptoms (7). In this case the patient had no evidence of macroscopic disease of the appendix on cross-sectional imaging one and a half year before. A comprehensive review of the literature from 1966 to 2004 by Pahlavan and Kanthan did not discuss the time course or onset of GCC in patients who had no antecedent appendiceal abnormality (8). Over the past two decades cross-sectional imaging has played a pivotal role in the management of patients presenting with signs and symptoms of acute appendicitis. As the number of patients receiving imaging study and the number of imaging study per patient both increase, the ability to follow the natural history of a disease improves. This case report provides evidence that GCC can occur within a period of 1.5 years. This case report also indicates the importance of considering appendiceal neoplasm in the diagnosis of a patient presenting with signs and symptoms of appendicitis even if a previous CT was negative for such disease. A major presentation of tumors of the appendix is acute appendicitis as the lumen may be obstructed, causing inflammation. It is important to characterize cecal involvement as a more radical surgical approach may be considered instead of the typical simple appendectomy.
RCR Radiology Case Reports | radiology.casereports.net
References 1. Pickhardt PJ, Levy AD, Rohrmann CA Jr, Kende AI. Primary neoplasms of the appendix: radiologic spectrum of disease with pathologic correlation. Radiographics. 2003;23:645-62. [PubMed] 2. Carr NJ, Remotti H, Sobin LH. Dual carcinoid/ epithelial neoplasia of the appendix. Histopathology. 1995;27:557-62. [PubMed] 3. Chen V, Qizilbash AH. Goblet cell carcinoid tumor of the appendix. Report of five cases and review of the literature. Arch Pathol Lab Med. 1979;103:180-2. [PubMed] 4. Butler JA, Houshiar A, Lin F, Wilson SE. Goblet cell carcinoid of the appendix. Am J Surg 1994;168:685-7. [PubMed] 5. Varisco B, McAlvin B, Dias J, Franga D. Adenocarcinoid of the appendix: is right hemicolectomy necessary? A meta-analysis of retrospective chart reviews. Am Surg. 2004;70:593-9. [PubMed] 6. Mandai M, Konishi I, Tsuruta Y, Suginami N, Kusakari T, Iwasaki T, Fujii S. Krukenberg tumor from an occult appendiceal adenocarcinoid: a case report and review of the literature. Eur J Obstet Gynecol Reprod Biol. 2001;97:90-5. [PubMed] 7. Di Paola M, Stockwell WS. Appendico-vesical fistula due to an appendix abscess with associated goblet cell carcinoid of the appendix. Br J Urol. 1976;48:436. [PubMed] 8. Pahlavan PS, Kanthan R. Goblet cell carcinoid of the appendix. World J Surg Oncol. 2005;3:36. [PubMed]
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