BACKGROUND Hepatoid carcinoma(HC)is an extremely rare neoplasm that is morphologicallysimilar to hepatocellular carcinoma.HC has been described in various organs;however,HC of the pancreas is extremely rare.To our kno...BACKGROUND Hepatoid carcinoma(HC)is an extremely rare neoplasm that is morphologicallysimilar to hepatocellular carcinoma.HC has been described in various organs;however,HC of the pancreas is extremely rare.To our knowledge,only 38 caseshave been reported.We present a case of HC of the pancreas in a 36-year-oldmale patient.CASE SUMMARY A 36-year-old cachexic man with no significant past medical history wastransferred to our hospital with a history of painless jaundice,elevated bloodglucose and significant weight loss.Lab tests showed elevated serumtransaminases,bilirubin and alpha-fetoprotein levels.Magnetic resonanceimaging of the upper abdomen showed a diffusely enlarged pancreas,appearing“sausage-shaped”.Magnetic resonance cholangiopancreatography showedupstream ductal dilation secondary to stricture of the main pancreatic duct andthe common bile duct,which were not visible.Immunohistochemistry ofbiopsied tissue from a percutaneous pancreatic biopsy showed tumor cellpositivity for HepPar1,polyclonal carcinoembryonic antigen and CK19,suggestive of HC of the pancreas.The characteristics of 39 patients with HC ofthe pancreas were reviewed.CONCLUSION HC of the pancreas is more prevalent in males,and patients have a median age of57 years.It is most commonly asymptomatic or presents as abdominal back pain,and the pancreatic tail is the most common location.At the time of diagnosis,liver metastasis is often present.展开更多
BACKGROUND Solitary fibrous tumor(SFT)is an uncommon mesenchymal neoplasm that arises from the pleura.A few SFTs have also been described in extrapleural sites.However,SFT of the thyroid gland is rare.Here,we report a...BACKGROUND Solitary fibrous tumor(SFT)is an uncommon mesenchymal neoplasm that arises from the pleura.A few SFTs have also been described in extrapleural sites.However,SFT of the thyroid gland is rare.Here,we report a case of extrapleural SFT on the thyroid gland,in addition to a literature review.CASE SUMMARY A 59-year-old man visited our hospital in July 2017 complaining of a large mass in his neck.His thyroid function test results,including antibody levels,were within the normal limits.Ultrasonography showed a 4.7 cm×4.0 cm×3.2 cm solitary mass of intermediate suspicion in the left thyroid lobe.A fine-needle aspiration biopsy was subsequently performed.The pathologist reported a benign follicular lesion.However,the size of this nodule increased to 5.5 cm×5.0 cm×3.4 cm by April 2018.After a multidisciplinary discussion,a left lobectomy was performed in May 2018.The specimen showed a well-demarcated,partly encapsulated,soft nodule of whitish and tan/brown color on the cut surface.Light microscopy revealed high cellularity with moderate cytologic atypia.The mitotic count was 5/10 high-power fields.There was no tumor necrosis or lymphovascular invasion.The tumor was CD34-positive and signal transducer and activator of transcription 6-positive.Neither thyroid transcription factor-1 nor cytokeratin expression was detected.The Ki-67 showed intermediate proliferative activity.The final diagnosis was extrapleural SFT of the thyroid gland with a clear resection margin.The patient was discharged without complication three days after the surgery.CONCLUSION In the literature,extrapleural SFT of the thyroid gland has been reported to behave indolently with the capacity for recurrence and rare metastasis,although surgical resection is the treatment of choice.Understanding this disease entity is important for accurate diagnosis and proper management.展开更多
BACKGROUND Primary synovial chondromatosis(PSC) is a rare arthropathy of the synovial joints characterized by the formation of cartilaginous nodules, which may detach and become loose bodies within the joint and may u...BACKGROUND Primary synovial chondromatosis(PSC) is a rare arthropathy of the synovial joints characterized by the formation of cartilaginous nodules, which may detach and become loose bodies within the joint and may undergo secondary proliferation. PSC of the foot and ankle is exceedingly rare, with only a few cases reported in the literature. The diagnosis may be difficult and delayed until operative treatment, when it is confirmed by histological assessment. PSC may degenerate into chondrosarcoma. Operative treatment is the gold standard aiming to minimize pain, improve function, prevent or limit progression of arthritis. Surgical treatment consists in debridement by arthrotomic or arthroscopic management, but there is no consensus in the literature about timing of surgery and surgical technique. Thus, the aim of this study is to report the outcomes of the surgical treatment of two cases, together with a literature review.CASE SUMMARY We report two cases of patients affected by PSC of the foot in stage III, according to the Milgram classification: the former PSC localized in the ankle that underwent open surgery consisted of loose bodies removal;the latter in the subtalar joint, and the choice of treatment was the arthrotomy and debridement from loose bodies, in addition to the subtalar arthrodesis. Both patients returned to complete daily and working life after surgery.CONCLUSION Synovial chondromatosis is a rare benign pathology, even rarer in the ankle joint and especially in the foot. Surgery should be minimal in patients with ankle PSC,choosing the correct timing, waiting if possible until stage III. More aggressive and early surgery should be performed in patients with PSC of the foot,particularly the subtalar joint, due to the high risk of arthritic evolution.展开更多
BACKGROUND Lymphoepithelioma-like carcinomas(LELCs)are rare,malignant epithelial tumors,generally considered a subtype of squamous cell carcinoma.LELCs are undifferentiated and can occur in multiple tissues,although L...BACKGROUND Lymphoepithelioma-like carcinomas(LELCs)are rare,malignant epithelial tumors,generally considered a subtype of squamous cell carcinoma.LELCs are undifferentiated and can occur in multiple tissues,although LELCs in the urinary tract are extremely rare.As such,evidence does not provide clinicians with guidelines for the best practices.Even though this is a rare disease,it is associated with high morbidity and mortality.Therefore,we must learn to differentiate LELC types and identify risk factors for early identification.AIM To develop an evidence base to guide clinicians treating primary LELCs of the upper urinary tract(UUT-LELC).METHODS We performed a systematic review of all reports on UUT-LELC from the first published case in 1998 until October 2019,according to the PRISMA.A database was then developed by extracting data from previously published reports in order to analyze interactions between clinical characteristics,pathological features,interventions and outcomes.Survival was analyzed using Kaplan–Meier estimates,which were compared using log rank tests.RESULTS A total of 28 previously published cases were identified for inclusion.The median age was 72 years with a male to female ratio of 4:3.Pure type LELCs were most common with 48.3%(n=14),followed by 37.9%(n=11)predominant LELCs and 3.4%(n=1)focal LELCs.Epstein-Barr virus testing was negative in all cases.Fourteen patients received radical nephroureterectomy(RNU)-based intervention.Twenty-three patients survived with no evidence of further metastasis,although six died before the median 18 mo follow-up point.Survival analysis suggests pure histological subtypes,and patients who receive complete tumor resection have more favorable prognoses.As always in cancer care,early identification generally increases the probability of interventional success.CONCLUSION The most effective treatment for UUT-LELC is RNU-based therapy.Since cases are few in number,case reporting must be enhanced and publishing encouraged to both save and prolong lives.展开更多
BACKGROUND Enteropathy associated T-cell lymphoma(EATL) is a rare form of peripheral Tcell lymphoma and makes up less than 5% of gastrointestinal lymphomas. EATL can be divided into type 1 which is associated with cel...BACKGROUND Enteropathy associated T-cell lymphoma(EATL) is a rare form of peripheral Tcell lymphoma and makes up less than 5% of gastrointestinal lymphomas. EATL can be divided into type 1 which is associated with celiac disease, and monomorphic epitheliotropic intestinal T-cell lymphoma(MEITL), formally type 2, which is not associated with celiac disease.CASE SUMMARY We present a 60-year-old African American female, without celiac disease, who presented with abdominal pain, diarrhea, and 30 lb. weight loss over a 3 month period. She was subsequently diagnosed with EATL throughout her entire gastrointestinal tract. She is currently undergoing chemotherapy with EOCH(Etoposide, Oncovin, Cyclophosphamide, and Hydroxydaunorubicin). EATL is most common in the Asian and Hispanic population yet the incidence in African Americans is uncertain and emphasizes the rarity of this case. A literature review was included to further emphasize similarities and differences between our case and previously reported cases of MEITL.CONCLUSION The patient was diagnosed with EATL, immunochemical testing was not conclusive for MEITL however was suggestive of the disease.展开更多
A 72-year-old male underwent neck dissection and parotidectomy with facial nerve preservation. Endotracheal intubation was facilitated with succinylcholine. Prolonged muscle paralysis which was first detected after fa...A 72-year-old male underwent neck dissection and parotidectomy with facial nerve preservation. Endotracheal intubation was facilitated with succinylcholine. Prolonged muscle paralysis which was first detected after failure to stimulate the facial nerve with electrocautery, lasted five hours. Laboratory tests indicated pseudocholinesterase (PChE) deficiency. A thyroidectomy one month later was performed uneventfully using rocuronium as a muscle relaxant. Literature review revealed a total of 40 PChE deficiency cases being reported since 1956.展开更多
The maxillomandibular fusion is a very rare condition, with no more than forty cases described in the literature. Adhesions of bone and/or soft tissue between the mandible and maxilla manifest themselves in the inabil...The maxillomandibular fusion is a very rare condition, with no more than forty cases described in the literature. Adhesions of bone and/or soft tissue between the mandible and maxilla manifest themselves in the inability to open the mouth added to impacts on mandibular growth, nutrition and speech. This condition can be isolated or, when congenital, associated with other anomalies such as cleft lip and palate, aglossia, or Van der Woude syndrome. In the present paper, we report a case of maxillomandibular fusion treated in our department at University Hospital, between February 2011 and June 2014. The case is a congenital maxillomandibular fusion in a two-year-old infant, associated with a syndrome of Van der Woude. We discuss the diagnostic and treatment difficulties on the anaesthetic and surgical levels and the action to be taken to avoid recurrence. To date, some classifications have been suggested in the literature, but there is no standard treatment protocol. Early treatment is necessary to allow freedom of the upper airway, and ensure proper nutrition and good growth of facial bones. The success of surgery is conditioned by an adequate physiotherapy follow-up likely to guarantee the non-recurrence of the lesion.展开更多
We report a case of natural killer(NK)/T-cell lymphoma with concomitant syndrome of inappropriate antidiuretic hormone secretion(SIADH).The patient was a 64-yearold woman with a history of nasopharyngeal carcinoma of ...We report a case of natural killer(NK)/T-cell lymphoma with concomitant syndrome of inappropriate antidiuretic hormone secretion(SIADH).The patient was a 64-yearold woman with a history of nasopharyngeal carcinoma of over 30 years.She was admitted with a chief complaint of intermittent fever for 2 mo.Palpation after admission indicated a swollen lymph node below the left jaw.Multiple imaging examinations on admission indicated multiple enlarged lymph nodes throughout the body.We performed a left submandibular lymph node biopsy,and the results revealed NK/T-cell lymphoma.A biochemical examination indicated Epstein-Barr virus positivity.At the same time,the patient developed hyponatremia.Based on her laboratory examination and clinical manifestation,decreased plasma osmolality,urine osmolality greater than plasma osmolality,lack of skin swelling,normal blood pressure,normal renal function,no adrenal function detected on serology,and no abnormalities in imaging examination of the adrenal glands,the likelihood of SIADH in the patient was high.After fluid restriction and administration of sodium chloride,the patient’s blood sodium level gradually increased.Subsequently,the immune function of the patient declined,there were severe symptoms of infection,and she died of respiratory failure.NK/T-cell lymphoma associated with SIADH has not,to our knowledge,been previously reported in PubMed.This case emphasizes the importance of monitoring serum ion levels,especially serum sodium,in patients with NK/T-cell lymphoma.展开更多
Objective To explore the diagnosis and treatment features of tuberous sclerosis complex associated renal cell carcinoma. Methods A 22-year-old boy with a childhood history of epilepsy and mental retardation pres-
基金the National Natural Science Foundation for Young Scientists of China,No.81800458the Science and Technology Planning Project of Guangdong Province,No.2016A020216014the Basic Research Program of Young Teachers’Training Project of Sun Yat-Sen University,No.17ykpy52.
文摘BACKGROUND Hepatoid carcinoma(HC)is an extremely rare neoplasm that is morphologicallysimilar to hepatocellular carcinoma.HC has been described in various organs;however,HC of the pancreas is extremely rare.To our knowledge,only 38 caseshave been reported.We present a case of HC of the pancreas in a 36-year-oldmale patient.CASE SUMMARY A 36-year-old cachexic man with no significant past medical history wastransferred to our hospital with a history of painless jaundice,elevated bloodglucose and significant weight loss.Lab tests showed elevated serumtransaminases,bilirubin and alpha-fetoprotein levels.Magnetic resonanceimaging of the upper abdomen showed a diffusely enlarged pancreas,appearing“sausage-shaped”.Magnetic resonance cholangiopancreatography showedupstream ductal dilation secondary to stricture of the main pancreatic duct andthe common bile duct,which were not visible.Immunohistochemistry ofbiopsied tissue from a percutaneous pancreatic biopsy showed tumor cellpositivity for HepPar1,polyclonal carcinoembryonic antigen and CK19,suggestive of HC of the pancreas.The characteristics of 39 patients with HC ofthe pancreas were reviewed.CONCLUSION HC of the pancreas is more prevalent in males,and patients have a median age of57 years.It is most commonly asymptomatic or presents as abdominal back pain,and the pancreatic tail is the most common location.At the time of diagnosis,liver metastasis is often present.
基金Supported by the National Research Foundation of Korea grant funded by the Korea government,No.2019R1G1A1004679
文摘BACKGROUND Solitary fibrous tumor(SFT)is an uncommon mesenchymal neoplasm that arises from the pleura.A few SFTs have also been described in extrapleural sites.However,SFT of the thyroid gland is rare.Here,we report a case of extrapleural SFT on the thyroid gland,in addition to a literature review.CASE SUMMARY A 59-year-old man visited our hospital in July 2017 complaining of a large mass in his neck.His thyroid function test results,including antibody levels,were within the normal limits.Ultrasonography showed a 4.7 cm×4.0 cm×3.2 cm solitary mass of intermediate suspicion in the left thyroid lobe.A fine-needle aspiration biopsy was subsequently performed.The pathologist reported a benign follicular lesion.However,the size of this nodule increased to 5.5 cm×5.0 cm×3.4 cm by April 2018.After a multidisciplinary discussion,a left lobectomy was performed in May 2018.The specimen showed a well-demarcated,partly encapsulated,soft nodule of whitish and tan/brown color on the cut surface.Light microscopy revealed high cellularity with moderate cytologic atypia.The mitotic count was 5/10 high-power fields.There was no tumor necrosis or lymphovascular invasion.The tumor was CD34-positive and signal transducer and activator of transcription 6-positive.Neither thyroid transcription factor-1 nor cytokeratin expression was detected.The Ki-67 showed intermediate proliferative activity.The final diagnosis was extrapleural SFT of the thyroid gland with a clear resection margin.The patient was discharged without complication three days after the surgery.CONCLUSION In the literature,extrapleural SFT of the thyroid gland has been reported to behave indolently with the capacity for recurrence and rare metastasis,although surgical resection is the treatment of choice.Understanding this disease entity is important for accurate diagnosis and proper management.
文摘BACKGROUND Primary synovial chondromatosis(PSC) is a rare arthropathy of the synovial joints characterized by the formation of cartilaginous nodules, which may detach and become loose bodies within the joint and may undergo secondary proliferation. PSC of the foot and ankle is exceedingly rare, with only a few cases reported in the literature. The diagnosis may be difficult and delayed until operative treatment, when it is confirmed by histological assessment. PSC may degenerate into chondrosarcoma. Operative treatment is the gold standard aiming to minimize pain, improve function, prevent or limit progression of arthritis. Surgical treatment consists in debridement by arthrotomic or arthroscopic management, but there is no consensus in the literature about timing of surgery and surgical technique. Thus, the aim of this study is to report the outcomes of the surgical treatment of two cases, together with a literature review.CASE SUMMARY We report two cases of patients affected by PSC of the foot in stage III, according to the Milgram classification: the former PSC localized in the ankle that underwent open surgery consisted of loose bodies removal;the latter in the subtalar joint, and the choice of treatment was the arthrotomy and debridement from loose bodies, in addition to the subtalar arthrodesis. Both patients returned to complete daily and working life after surgery.CONCLUSION Synovial chondromatosis is a rare benign pathology, even rarer in the ankle joint and especially in the foot. Surgery should be minimal in patients with ankle PSC,choosing the correct timing, waiting if possible until stage III. More aggressive and early surgery should be performed in patients with PSC of the foot,particularly the subtalar joint, due to the high risk of arthritic evolution.
文摘BACKGROUND Lymphoepithelioma-like carcinomas(LELCs)are rare,malignant epithelial tumors,generally considered a subtype of squamous cell carcinoma.LELCs are undifferentiated and can occur in multiple tissues,although LELCs in the urinary tract are extremely rare.As such,evidence does not provide clinicians with guidelines for the best practices.Even though this is a rare disease,it is associated with high morbidity and mortality.Therefore,we must learn to differentiate LELC types and identify risk factors for early identification.AIM To develop an evidence base to guide clinicians treating primary LELCs of the upper urinary tract(UUT-LELC).METHODS We performed a systematic review of all reports on UUT-LELC from the first published case in 1998 until October 2019,according to the PRISMA.A database was then developed by extracting data from previously published reports in order to analyze interactions between clinical characteristics,pathological features,interventions and outcomes.Survival was analyzed using Kaplan–Meier estimates,which were compared using log rank tests.RESULTS A total of 28 previously published cases were identified for inclusion.The median age was 72 years with a male to female ratio of 4:3.Pure type LELCs were most common with 48.3%(n=14),followed by 37.9%(n=11)predominant LELCs and 3.4%(n=1)focal LELCs.Epstein-Barr virus testing was negative in all cases.Fourteen patients received radical nephroureterectomy(RNU)-based intervention.Twenty-three patients survived with no evidence of further metastasis,although six died before the median 18 mo follow-up point.Survival analysis suggests pure histological subtypes,and patients who receive complete tumor resection have more favorable prognoses.As always in cancer care,early identification generally increases the probability of interventional success.CONCLUSION The most effective treatment for UUT-LELC is RNU-based therapy.Since cases are few in number,case reporting must be enhanced and publishing encouraged to both save and prolong lives.
文摘BACKGROUND Enteropathy associated T-cell lymphoma(EATL) is a rare form of peripheral Tcell lymphoma and makes up less than 5% of gastrointestinal lymphomas. EATL can be divided into type 1 which is associated with celiac disease, and monomorphic epitheliotropic intestinal T-cell lymphoma(MEITL), formally type 2, which is not associated with celiac disease.CASE SUMMARY We present a 60-year-old African American female, without celiac disease, who presented with abdominal pain, diarrhea, and 30 lb. weight loss over a 3 month period. She was subsequently diagnosed with EATL throughout her entire gastrointestinal tract. She is currently undergoing chemotherapy with EOCH(Etoposide, Oncovin, Cyclophosphamide, and Hydroxydaunorubicin). EATL is most common in the Asian and Hispanic population yet the incidence in African Americans is uncertain and emphasizes the rarity of this case. A literature review was included to further emphasize similarities and differences between our case and previously reported cases of MEITL.CONCLUSION The patient was diagnosed with EATL, immunochemical testing was not conclusive for MEITL however was suggestive of the disease.
文摘A 72-year-old male underwent neck dissection and parotidectomy with facial nerve preservation. Endotracheal intubation was facilitated with succinylcholine. Prolonged muscle paralysis which was first detected after failure to stimulate the facial nerve with electrocautery, lasted five hours. Laboratory tests indicated pseudocholinesterase (PChE) deficiency. A thyroidectomy one month later was performed uneventfully using rocuronium as a muscle relaxant. Literature review revealed a total of 40 PChE deficiency cases being reported since 1956.
文摘The maxillomandibular fusion is a very rare condition, with no more than forty cases described in the literature. Adhesions of bone and/or soft tissue between the mandible and maxilla manifest themselves in the inability to open the mouth added to impacts on mandibular growth, nutrition and speech. This condition can be isolated or, when congenital, associated with other anomalies such as cleft lip and palate, aglossia, or Van der Woude syndrome. In the present paper, we report a case of maxillomandibular fusion treated in our department at University Hospital, between February 2011 and June 2014. The case is a congenital maxillomandibular fusion in a two-year-old infant, associated with a syndrome of Van der Woude. We discuss the diagnostic and treatment difficulties on the anaesthetic and surgical levels and the action to be taken to avoid recurrence. To date, some classifications have been suggested in the literature, but there is no standard treatment protocol. Early treatment is necessary to allow freedom of the upper airway, and ensure proper nutrition and good growth of facial bones. The success of surgery is conditioned by an adequate physiotherapy follow-up likely to guarantee the non-recurrence of the lesion.
文摘We report a case of natural killer(NK)/T-cell lymphoma with concomitant syndrome of inappropriate antidiuretic hormone secretion(SIADH).The patient was a 64-yearold woman with a history of nasopharyngeal carcinoma of over 30 years.She was admitted with a chief complaint of intermittent fever for 2 mo.Palpation after admission indicated a swollen lymph node below the left jaw.Multiple imaging examinations on admission indicated multiple enlarged lymph nodes throughout the body.We performed a left submandibular lymph node biopsy,and the results revealed NK/T-cell lymphoma.A biochemical examination indicated Epstein-Barr virus positivity.At the same time,the patient developed hyponatremia.Based on her laboratory examination and clinical manifestation,decreased plasma osmolality,urine osmolality greater than plasma osmolality,lack of skin swelling,normal blood pressure,normal renal function,no adrenal function detected on serology,and no abnormalities in imaging examination of the adrenal glands,the likelihood of SIADH in the patient was high.After fluid restriction and administration of sodium chloride,the patient’s blood sodium level gradually increased.Subsequently,the immune function of the patient declined,there were severe symptoms of infection,and she died of respiratory failure.NK/T-cell lymphoma associated with SIADH has not,to our knowledge,been previously reported in PubMed.This case emphasizes the importance of monitoring serum ion levels,especially serum sodium,in patients with NK/T-cell lymphoma.
文摘Objective To explore the diagnosis and treatment features of tuberous sclerosis complex associated renal cell carcinoma. Methods A 22-year-old boy with a childhood history of epilepsy and mental retardation pres-
