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Rare Gems: Navigating Goblet Cell Appendiceal Cancer
Background
Goblet cell adenocarcinoma (GCA), also known as goblet cell carcinoid, is a rare and distinct type of cancer originating from the appendix. It is characterized by cells that exhibit both mucinous and neuroendocrine differentiation, presenting a more aggressive nature compared to conventional carcinoids and a higher propensity for metastasis.
Case Presentation
A 60-year-old male presented with complaints of abdominal pain, nausea, vomiting, constipation, and weight loss worsening in the last month. He had a history of heavy alcohol intake, smoking, and family history of colon cancer in his grandfather. Initial workup with abdominal CT revealed findings suggestive of early bowel obstruction and possible malignancy. Subsequent EGD showed esophagitis, and colonoscopy identified a cecal mass. Biopsies confirmed malignant cells of enteric type with goblet cell features. Staging CT during hospitalization did not reveal distant metastasis initially. However, diagnostic laparoscopy later identified widespread peritoneal carcinomatosis, precluding surgical intervention. The case was discussed in tumor boards, leading to the initiation of palliative FOLFOX + Bevacizumab chemotherapy. After completing 7 cycles, restaging imaging showed stable disease. Subsequently, the patient experienced worsening obstructive symptoms with CT abdomen and pelvis demonstrating disease progression. Given his condition, decompressive gastrostomy was not feasible. The patient decided to transition to comfort measures only.
Discussion
Goblet cell adenocarcinoma is a rare appendiceal tumor with amphicrine differentiation, occurring at a rate of 0.01–0.05 per 100,000 individuals annually and comprising approximately 15% of all appendiceal neoplasms. These tumors often disseminate within the peritoneum, contributing to their aggressive behavior and challenging management.
Conclusions
Metastatic goblet cell adenocarcinoma presents significant treatment challenges and is associated with a poor prognosis. Tailored treatment strategies, vigilant monitoring, and ongoing research efforts are essential for optimizing patient outcomes and enhancing quality of life in this aggressive cancer
Background
Goblet cell adenocarcinoma (GCA), also known as goblet cell carcinoid, is a rare and distinct type of cancer originating from the appendix. It is characterized by cells that exhibit both mucinous and neuroendocrine differentiation, presenting a more aggressive nature compared to conventional carcinoids and a higher propensity for metastasis.
Case Presentation
A 60-year-old male presented with complaints of abdominal pain, nausea, vomiting, constipation, and weight loss worsening in the last month. He had a history of heavy alcohol intake, smoking, and family history of colon cancer in his grandfather. Initial workup with abdominal CT revealed findings suggestive of early bowel obstruction and possible malignancy. Subsequent EGD showed esophagitis, and colonoscopy identified a cecal mass. Biopsies confirmed malignant cells of enteric type with goblet cell features. Staging CT during hospitalization did not reveal distant metastasis initially. However, diagnostic laparoscopy later identified widespread peritoneal carcinomatosis, precluding surgical intervention. The case was discussed in tumor boards, leading to the initiation of palliative FOLFOX + Bevacizumab chemotherapy. After completing 7 cycles, restaging imaging showed stable disease. Subsequently, the patient experienced worsening obstructive symptoms with CT abdomen and pelvis demonstrating disease progression. Given his condition, decompressive gastrostomy was not feasible. The patient decided to transition to comfort measures only.
Discussion
Goblet cell adenocarcinoma is a rare appendiceal tumor with amphicrine differentiation, occurring at a rate of 0.01–0.05 per 100,000 individuals annually and comprising approximately 15% of all appendiceal neoplasms. These tumors often disseminate within the peritoneum, contributing to their aggressive behavior and challenging management.
Conclusions
Metastatic goblet cell adenocarcinoma presents significant treatment challenges and is associated with a poor prognosis. Tailored treatment strategies, vigilant monitoring, and ongoing research efforts are essential for optimizing patient outcomes and enhancing quality of life in this aggressive cancer
Background
Goblet cell adenocarcinoma (GCA), also known as goblet cell carcinoid, is a rare and distinct type of cancer originating from the appendix. It is characterized by cells that exhibit both mucinous and neuroendocrine differentiation, presenting a more aggressive nature compared to conventional carcinoids and a higher propensity for metastasis.
Case Presentation
A 60-year-old male presented with complaints of abdominal pain, nausea, vomiting, constipation, and weight loss worsening in the last month. He had a history of heavy alcohol intake, smoking, and family history of colon cancer in his grandfather. Initial workup with abdominal CT revealed findings suggestive of early bowel obstruction and possible malignancy. Subsequent EGD showed esophagitis, and colonoscopy identified a cecal mass. Biopsies confirmed malignant cells of enteric type with goblet cell features. Staging CT during hospitalization did not reveal distant metastasis initially. However, diagnostic laparoscopy later identified widespread peritoneal carcinomatosis, precluding surgical intervention. The case was discussed in tumor boards, leading to the initiation of palliative FOLFOX + Bevacizumab chemotherapy. After completing 7 cycles, restaging imaging showed stable disease. Subsequently, the patient experienced worsening obstructive symptoms with CT abdomen and pelvis demonstrating disease progression. Given his condition, decompressive gastrostomy was not feasible. The patient decided to transition to comfort measures only.
Discussion
Goblet cell adenocarcinoma is a rare appendiceal tumor with amphicrine differentiation, occurring at a rate of 0.01–0.05 per 100,000 individuals annually and comprising approximately 15% of all appendiceal neoplasms. These tumors often disseminate within the peritoneum, contributing to their aggressive behavior and challenging management.
Conclusions
Metastatic goblet cell adenocarcinoma presents significant treatment challenges and is associated with a poor prognosis. Tailored treatment strategies, vigilant monitoring, and ongoing research efforts are essential for optimizing patient outcomes and enhancing quality of life in this aggressive cancer
UC as a Culprit for Hemolytic Anemia
Introduction
Autoimmune hemolytic anemia (AIHA) can rarely be seen as an extra-intestinal manifestation (EIM) of inflammatory bowel disease (IBD), mostly ulcerative colitis (UC). This case report describes the clinical significance of recognizing AIHA in the context of UC.
Case Presentation
A 32-year-old male presented with profound fatigue, pallor, and dyspnea on exertion for one month. He also recalled intermittent bloody diarrhea for two years for which he never sought medical attention. Physical examination was unremarkable except for mid-abdominal tenderness. Labs revealed microcytosis, hemoglobin of 3.8 g/dL, total bilirubin 2.9 mg/ dL, indirect bilirubin of 2.0 mg/dL, LDH 132 U/L alk-p 459 U/L AST 98 U/L ALT 22 U/L. Direct Coombs test was positive suggesting warm AIHA with pan-agglutinin positive on the eluate test. Further testing revealed negative hepatitis and HIV panels and positive fecal calprotectin. CT abdomen and pelvis showed ascites, right pleural effusion and hepatosplenomegaly. Colonoscopy confirmed the diagnosis of ulcerative colitis, with extensive involvement of the colon. Mesalamine was initiated. Hematology was consulted for AIHA, who started the patient on methylprednisone leading to resolution of hemolytic anemia and improvement in gastrointestinal symptoms.
Discussion
IBD typically manifests as colitis, and the incidence of EIM as an initial symptom is observed in less than 10% cases. However, over the course of their lifetime, approximately 25% of patients will experience EIM, underscoring their relevance to clinical outcomes. Anemia is very common in IBD patients, mostly iron deficiency anemia (IDA) or anemia of chronic disease (ACD). However, AIHA can represent a rare but significant EIM of ulcerative colitis (UC), often posing diagnostic challenges. The underlying pathophysiological mechanisms linking UC and AIHA remain incompletely understood, necessitating a multidisciplinary approach to management. Treatment strategies focus on controlling both the hemolysis and the underlying IBD, emphasizing the importance of tailored interventions.
Conclusion
This case underscores the clinical significance of AIHA as an EIM of ulcerative colitis (UC), particularly when presenting as the primary symptom. Timely recognition is paramount to optimizing patient outcomes and preventing disease progression. Further research is warranted to elucidate the underlying mechanisms and therapeutic strategies for AIHA in the context of UC.
Introduction
Autoimmune hemolytic anemia (AIHA) can rarely be seen as an extra-intestinal manifestation (EIM) of inflammatory bowel disease (IBD), mostly ulcerative colitis (UC). This case report describes the clinical significance of recognizing AIHA in the context of UC.
Case Presentation
A 32-year-old male presented with profound fatigue, pallor, and dyspnea on exertion for one month. He also recalled intermittent bloody diarrhea for two years for which he never sought medical attention. Physical examination was unremarkable except for mid-abdominal tenderness. Labs revealed microcytosis, hemoglobin of 3.8 g/dL, total bilirubin 2.9 mg/ dL, indirect bilirubin of 2.0 mg/dL, LDH 132 U/L alk-p 459 U/L AST 98 U/L ALT 22 U/L. Direct Coombs test was positive suggesting warm AIHA with pan-agglutinin positive on the eluate test. Further testing revealed negative hepatitis and HIV panels and positive fecal calprotectin. CT abdomen and pelvis showed ascites, right pleural effusion and hepatosplenomegaly. Colonoscopy confirmed the diagnosis of ulcerative colitis, with extensive involvement of the colon. Mesalamine was initiated. Hematology was consulted for AIHA, who started the patient on methylprednisone leading to resolution of hemolytic anemia and improvement in gastrointestinal symptoms.
Discussion
IBD typically manifests as colitis, and the incidence of EIM as an initial symptom is observed in less than 10% cases. However, over the course of their lifetime, approximately 25% of patients will experience EIM, underscoring their relevance to clinical outcomes. Anemia is very common in IBD patients, mostly iron deficiency anemia (IDA) or anemia of chronic disease (ACD). However, AIHA can represent a rare but significant EIM of ulcerative colitis (UC), often posing diagnostic challenges. The underlying pathophysiological mechanisms linking UC and AIHA remain incompletely understood, necessitating a multidisciplinary approach to management. Treatment strategies focus on controlling both the hemolysis and the underlying IBD, emphasizing the importance of tailored interventions.
Conclusion
This case underscores the clinical significance of AIHA as an EIM of ulcerative colitis (UC), particularly when presenting as the primary symptom. Timely recognition is paramount to optimizing patient outcomes and preventing disease progression. Further research is warranted to elucidate the underlying mechanisms and therapeutic strategies for AIHA in the context of UC.
Introduction
Autoimmune hemolytic anemia (AIHA) can rarely be seen as an extra-intestinal manifestation (EIM) of inflammatory bowel disease (IBD), mostly ulcerative colitis (UC). This case report describes the clinical significance of recognizing AIHA in the context of UC.
Case Presentation
A 32-year-old male presented with profound fatigue, pallor, and dyspnea on exertion for one month. He also recalled intermittent bloody diarrhea for two years for which he never sought medical attention. Physical examination was unremarkable except for mid-abdominal tenderness. Labs revealed microcytosis, hemoglobin of 3.8 g/dL, total bilirubin 2.9 mg/ dL, indirect bilirubin of 2.0 mg/dL, LDH 132 U/L alk-p 459 U/L AST 98 U/L ALT 22 U/L. Direct Coombs test was positive suggesting warm AIHA with pan-agglutinin positive on the eluate test. Further testing revealed negative hepatitis and HIV panels and positive fecal calprotectin. CT abdomen and pelvis showed ascites, right pleural effusion and hepatosplenomegaly. Colonoscopy confirmed the diagnosis of ulcerative colitis, with extensive involvement of the colon. Mesalamine was initiated. Hematology was consulted for AIHA, who started the patient on methylprednisone leading to resolution of hemolytic anemia and improvement in gastrointestinal symptoms.
Discussion
IBD typically manifests as colitis, and the incidence of EIM as an initial symptom is observed in less than 10% cases. However, over the course of their lifetime, approximately 25% of patients will experience EIM, underscoring their relevance to clinical outcomes. Anemia is very common in IBD patients, mostly iron deficiency anemia (IDA) or anemia of chronic disease (ACD). However, AIHA can represent a rare but significant EIM of ulcerative colitis (UC), often posing diagnostic challenges. The underlying pathophysiological mechanisms linking UC and AIHA remain incompletely understood, necessitating a multidisciplinary approach to management. Treatment strategies focus on controlling both the hemolysis and the underlying IBD, emphasizing the importance of tailored interventions.
Conclusion
This case underscores the clinical significance of AIHA as an EIM of ulcerative colitis (UC), particularly when presenting as the primary symptom. Timely recognition is paramount to optimizing patient outcomes and preventing disease progression. Further research is warranted to elucidate the underlying mechanisms and therapeutic strategies for AIHA in the context of UC.
Beyond Borders: Tonsillar Squamous Cell Carcinoma with Intriguing Liver Metastasis
Background
Oropharyngeal squamous cell carcinoma (OPSCC) arises in the middle pharynx, including the tonsils, base of the tongue, and surrounding tissues. While OPSCC commonly metastasizes to regional lymph nodes, distant metastases to sites like the liver are rare, occurring in about 1-4% of cases with advanced disease.
Case Presentation
A 66-year-old male presented to the emergency department with recurrent right-sided facial swelling and a two-week history of sore throat. CT imaging revealed a large right tonsillar mass extending to the base of the tongue. Further evaluation with PET scan showed hypermetabolic activity in the right tonsil, multiple hypermetabolic lymph nodes in the right neck (stations 1B, 2, 3, 4, 5), right supraclavicular fossa, and small retropharyngeal nodes. Additionally, PET scan detected a hypermetabolic lesion in the liver and focal activity at T10 suggestive of bone metastasis. Fine needle aspiration (FNA) confirmed squamous cell carcinoma. Biopsy of the liver lesion revealed metastatic squamous cell carcinoma with basaloid differentiation, positive for p40 and p63 stains. Clinical staging was T2b cN2 cM1. The patient’s case was discussed in tumor boards, leading to a treatment plan of palliative radiotherapy with radiosensitizer (weekly carboplatin/paclitaxel) due to recent myocardial infarction, precluding cisplatin or 5FU use. Post-radiotherapy, Pembrolizumab was planned based on 60% PD-L1 expression. The patient opted to forego additional systemic chemotherapy and currently receives Keytruda every three weeks.
Discussion
Liver metastases from head and neck SCC are rare, highlighting the complexity of treatment decisions in such cases. Effective management requires a multidisciplinary approach to optimize therapeutic outcomes while considering patient-specific factors and comorbidities.
Conclusions
This case underscores the challenges and poor prognosis associated with tonsillar SCC with liver metastases. It underscores the need for personalized treatment strategies tailored to the unique characteristics of each patient’s disease.
Background
Oropharyngeal squamous cell carcinoma (OPSCC) arises in the middle pharynx, including the tonsils, base of the tongue, and surrounding tissues. While OPSCC commonly metastasizes to regional lymph nodes, distant metastases to sites like the liver are rare, occurring in about 1-4% of cases with advanced disease.
Case Presentation
A 66-year-old male presented to the emergency department with recurrent right-sided facial swelling and a two-week history of sore throat. CT imaging revealed a large right tonsillar mass extending to the base of the tongue. Further evaluation with PET scan showed hypermetabolic activity in the right tonsil, multiple hypermetabolic lymph nodes in the right neck (stations 1B, 2, 3, 4, 5), right supraclavicular fossa, and small retropharyngeal nodes. Additionally, PET scan detected a hypermetabolic lesion in the liver and focal activity at T10 suggestive of bone metastasis. Fine needle aspiration (FNA) confirmed squamous cell carcinoma. Biopsy of the liver lesion revealed metastatic squamous cell carcinoma with basaloid differentiation, positive for p40 and p63 stains. Clinical staging was T2b cN2 cM1. The patient’s case was discussed in tumor boards, leading to a treatment plan of palliative radiotherapy with radiosensitizer (weekly carboplatin/paclitaxel) due to recent myocardial infarction, precluding cisplatin or 5FU use. Post-radiotherapy, Pembrolizumab was planned based on 60% PD-L1 expression. The patient opted to forego additional systemic chemotherapy and currently receives Keytruda every three weeks.
Discussion
Liver metastases from head and neck SCC are rare, highlighting the complexity of treatment decisions in such cases. Effective management requires a multidisciplinary approach to optimize therapeutic outcomes while considering patient-specific factors and comorbidities.
Conclusions
This case underscores the challenges and poor prognosis associated with tonsillar SCC with liver metastases. It underscores the need for personalized treatment strategies tailored to the unique characteristics of each patient’s disease.
Background
Oropharyngeal squamous cell carcinoma (OPSCC) arises in the middle pharynx, including the tonsils, base of the tongue, and surrounding tissues. While OPSCC commonly metastasizes to regional lymph nodes, distant metastases to sites like the liver are rare, occurring in about 1-4% of cases with advanced disease.
Case Presentation
A 66-year-old male presented to the emergency department with recurrent right-sided facial swelling and a two-week history of sore throat. CT imaging revealed a large right tonsillar mass extending to the base of the tongue. Further evaluation with PET scan showed hypermetabolic activity in the right tonsil, multiple hypermetabolic lymph nodes in the right neck (stations 1B, 2, 3, 4, 5), right supraclavicular fossa, and small retropharyngeal nodes. Additionally, PET scan detected a hypermetabolic lesion in the liver and focal activity at T10 suggestive of bone metastasis. Fine needle aspiration (FNA) confirmed squamous cell carcinoma. Biopsy of the liver lesion revealed metastatic squamous cell carcinoma with basaloid differentiation, positive for p40 and p63 stains. Clinical staging was T2b cN2 cM1. The patient’s case was discussed in tumor boards, leading to a treatment plan of palliative radiotherapy with radiosensitizer (weekly carboplatin/paclitaxel) due to recent myocardial infarction, precluding cisplatin or 5FU use. Post-radiotherapy, Pembrolizumab was planned based on 60% PD-L1 expression. The patient opted to forego additional systemic chemotherapy and currently receives Keytruda every three weeks.
Discussion
Liver metastases from head and neck SCC are rare, highlighting the complexity of treatment decisions in such cases. Effective management requires a multidisciplinary approach to optimize therapeutic outcomes while considering patient-specific factors and comorbidities.
Conclusions
This case underscores the challenges and poor prognosis associated with tonsillar SCC with liver metastases. It underscores the need for personalized treatment strategies tailored to the unique characteristics of each patient’s disease.
Unexpected Findings: A Rare Case of Signet Ring Cell Adenocarcinoma in the Small Intestine
Introduction
Signet ring cell carcinoma (SRCC) of the small intestine is very rare. It is characterized by the presence of malignant cells that contain mucin that push nuclei to the periphery. It is more aggressive compared to other adenocarcinomas due to early metastasis and poorer prognosis.
Case Presentation
A 59-year-old male with a history of HIV/AIDS, presented with complaints of anorexia, vomiting and weight loss. Initial abdominal CT showed a retroperitoneal mass causing gastric outlet obstruction. The patient elected to go home after supportive treatment and follow up as an outpatient, however, he presented 10 days later with worsening symptoms. Evaluation with CT abdomen and pelvis showed enlarging soft tissue density in the retrocrural space extending into the retroperitoneum around the aorta, as well as a 1.5 cm intraluminal cystic lesion in the duodenum. Endoscopic ultrasound revealed lymphadenopathy of celiac and porta hepatis regions, along with duodenal stenosis, stent placement for decompression was not feasible and biopsies were inconclusive. The decision was made to proceed with laparotomy for decompression and additional biopsies from the retroperitoneal mass and omental lymph nodes, which confirmed poorly differentiated adenocarcinoma with signet ring cells. The presence of a mass in the duodenum strongly suggested adenocarcinoma of small intestine origin. As the patient’s symptoms worsened, imaging revealed progression with lung metastases. The patient continued to deteriorate rapidly requiring dialysis and gangrenous cholecystitis. Given his complex medical history, patient decided to transition to comfort care.
Discussion
SRCC can present with any GI symptoms. Most important step in diagnosing SRCC is biopsy. Current treatment options for small intestinal malignancies include wide resection that includes the mesentery and corresponding lymph nodes. The use of adjuvant chemotherapy has been described only in small retrospective studies. Due to its scarcity, there isn’t sufficient data for optimal treatment strategies compared to gastric SRCC.
Conclusions
This case report highlights the importance of how rare and aggressive signet ring cell adenocarcinoma of the small intestine. There are only a few cases documented in the literature, which is why we lack data on how to manage the disease.
Introduction
Signet ring cell carcinoma (SRCC) of the small intestine is very rare. It is characterized by the presence of malignant cells that contain mucin that push nuclei to the periphery. It is more aggressive compared to other adenocarcinomas due to early metastasis and poorer prognosis.
Case Presentation
A 59-year-old male with a history of HIV/AIDS, presented with complaints of anorexia, vomiting and weight loss. Initial abdominal CT showed a retroperitoneal mass causing gastric outlet obstruction. The patient elected to go home after supportive treatment and follow up as an outpatient, however, he presented 10 days later with worsening symptoms. Evaluation with CT abdomen and pelvis showed enlarging soft tissue density in the retrocrural space extending into the retroperitoneum around the aorta, as well as a 1.5 cm intraluminal cystic lesion in the duodenum. Endoscopic ultrasound revealed lymphadenopathy of celiac and porta hepatis regions, along with duodenal stenosis, stent placement for decompression was not feasible and biopsies were inconclusive. The decision was made to proceed with laparotomy for decompression and additional biopsies from the retroperitoneal mass and omental lymph nodes, which confirmed poorly differentiated adenocarcinoma with signet ring cells. The presence of a mass in the duodenum strongly suggested adenocarcinoma of small intestine origin. As the patient’s symptoms worsened, imaging revealed progression with lung metastases. The patient continued to deteriorate rapidly requiring dialysis and gangrenous cholecystitis. Given his complex medical history, patient decided to transition to comfort care.
Discussion
SRCC can present with any GI symptoms. Most important step in diagnosing SRCC is biopsy. Current treatment options for small intestinal malignancies include wide resection that includes the mesentery and corresponding lymph nodes. The use of adjuvant chemotherapy has been described only in small retrospective studies. Due to its scarcity, there isn’t sufficient data for optimal treatment strategies compared to gastric SRCC.
Conclusions
This case report highlights the importance of how rare and aggressive signet ring cell adenocarcinoma of the small intestine. There are only a few cases documented in the literature, which is why we lack data on how to manage the disease.
Introduction
Signet ring cell carcinoma (SRCC) of the small intestine is very rare. It is characterized by the presence of malignant cells that contain mucin that push nuclei to the periphery. It is more aggressive compared to other adenocarcinomas due to early metastasis and poorer prognosis.
Case Presentation
A 59-year-old male with a history of HIV/AIDS, presented with complaints of anorexia, vomiting and weight loss. Initial abdominal CT showed a retroperitoneal mass causing gastric outlet obstruction. The patient elected to go home after supportive treatment and follow up as an outpatient, however, he presented 10 days later with worsening symptoms. Evaluation with CT abdomen and pelvis showed enlarging soft tissue density in the retrocrural space extending into the retroperitoneum around the aorta, as well as a 1.5 cm intraluminal cystic lesion in the duodenum. Endoscopic ultrasound revealed lymphadenopathy of celiac and porta hepatis regions, along with duodenal stenosis, stent placement for decompression was not feasible and biopsies were inconclusive. The decision was made to proceed with laparotomy for decompression and additional biopsies from the retroperitoneal mass and omental lymph nodes, which confirmed poorly differentiated adenocarcinoma with signet ring cells. The presence of a mass in the duodenum strongly suggested adenocarcinoma of small intestine origin. As the patient’s symptoms worsened, imaging revealed progression with lung metastases. The patient continued to deteriorate rapidly requiring dialysis and gangrenous cholecystitis. Given his complex medical history, patient decided to transition to comfort care.
Discussion
SRCC can present with any GI symptoms. Most important step in diagnosing SRCC is biopsy. Current treatment options for small intestinal malignancies include wide resection that includes the mesentery and corresponding lymph nodes. The use of adjuvant chemotherapy has been described only in small retrospective studies. Due to its scarcity, there isn’t sufficient data for optimal treatment strategies compared to gastric SRCC.
Conclusions
This case report highlights the importance of how rare and aggressive signet ring cell adenocarcinoma of the small intestine. There are only a few cases documented in the literature, which is why we lack data on how to manage the disease.