Ectopic hamartomatous thymoma is a rare,generally benign neoplasm that mainly arises in the lower neck region and is yet to be completely understood.Herein,we describe a case with two separate and sequential growing n...Ectopic hamartomatous thymoma is a rare,generally benign neoplasm that mainly arises in the lower neck region and is yet to be completely understood.Herein,we describe a case with two separate and sequential growing neoplasms for the first time,distinguished from other reported solitary cases.A 58-year-old Chinese male presented with a 6-month history of two nontender,self-palpated masses in the suprasternal region of his neck.Physical examination revealed two masses with diameters of 5 cm and 2 cm.Ultrasound examination 2 years ago revealed a hyperechoic mass with a fat-derived possibility.These two neoplasms were clinically diagnosed as lipomas without other radiological examinations and were completely excised.Microscopically,they were well circumscribed and characterized by admixture with spindle cells,epithelial nests,and adipocytes in variable amounts,without atypia or mitotic activity.Immunohistochemical staining revealed that both neoplasms showed a biphasic pattern,with an epithelial nature with strong and diffuse positive expression of cytokeratin(CK)and myoepithelial differentiation with CK5/6 and p63 positivity in the spindle cells.There was no evidence of recurrence after a 28-month follow-up period.In this report,we present a unique case of ectopic hamartomatous thymoma to show the existence of multiple tumors in this rare disease.We hope to raise the awareness of the possibility of this disease when the tumor rises in typical lower neck regions,especially when mixed soft tissue or fat components are shown by imaging examination.展开更多
Background Inflammatory linear verrucous epidermal nevus(ILVEN)is a rare type of epidermal nevus that occurs in infancy and develops along Blaschko’s lines.Due to its rarity and clinical/histological similarities wit...Background Inflammatory linear verrucous epidermal nevus(ILVEN)is a rare type of epidermal nevus that occurs in infancy and develops along Blaschko’s lines.Due to its rarity and clinical/histological similarities with other cutaneous disorders,correct diagnosis is sometimes difficult.In the present case,the whole exome sequencing of blood found no germline mutation,but the sequencing of the biopsy specimen revealed a novel potential pathogenic somatic mutation,C>T at rs200881715(p.R698X)in the cspp1 gene,which may provide new insight into the mechanism of ILVEN.Patient concerns A 10-year-old girl presented with linear,pruritic,erythematous,scaly,and exophytic verrucous skin lesions on her left thigh,buttock,and hand unilaterally along Blaschko’s lines from the third day after birth.Diagnosis Biopsy revealed psoriasiform acanthosis,papillary hyperplasia with orthohyperkeratosis,and parakeratosis of squamous epithelium,which are histological characteristics of ILVEN.Interventions Owing to the large lesion size and significant refractory response to previous treatments,multiple-stage surgical resections with a tissue expander were performed.Outcomes After surgical resection and defect reconstruction with a tissue expander,the outcome was satisfactory for the patient and her family.No recurrence was detected during the follow-up visits.Conclusion ILVEN is a rare type of cutaneous hyperplastic disease,and treatment is extremely challenging.Biopsy should be performed at the diagnostician’s discretion or if refractory to conservative treatments.Timely diagnosis and surgical intervention could result in satisfactory outcomes.展开更多
Dear Editor,Diffuse large B-cell lymphoma(DLBCL)is the most common and aggressive subtype of non-Hodgkin lymphoma(NHL),accounting for about 40%of all NHL cases[1].Lacking symptoms at early time and efficient therapeut...Dear Editor,Diffuse large B-cell lymphoma(DLBCL)is the most common and aggressive subtype of non-Hodgkin lymphoma(NHL),accounting for about 40%of all NHL cases[1].Lacking symptoms at early time and efficient therapeutic methods made DLBCL one of the most life-threatening types of hematopoietic malignancy[2,3].Therefore,identifying novel therapeutic biomarker for early detection and prognosis prediction is urgently needed.展开更多
文摘Ectopic hamartomatous thymoma is a rare,generally benign neoplasm that mainly arises in the lower neck region and is yet to be completely understood.Herein,we describe a case with two separate and sequential growing neoplasms for the first time,distinguished from other reported solitary cases.A 58-year-old Chinese male presented with a 6-month history of two nontender,self-palpated masses in the suprasternal region of his neck.Physical examination revealed two masses with diameters of 5 cm and 2 cm.Ultrasound examination 2 years ago revealed a hyperechoic mass with a fat-derived possibility.These two neoplasms were clinically diagnosed as lipomas without other radiological examinations and were completely excised.Microscopically,they were well circumscribed and characterized by admixture with spindle cells,epithelial nests,and adipocytes in variable amounts,without atypia or mitotic activity.Immunohistochemical staining revealed that both neoplasms showed a biphasic pattern,with an epithelial nature with strong and diffuse positive expression of cytokeratin(CK)and myoepithelial differentiation with CK5/6 and p63 positivity in the spindle cells.There was no evidence of recurrence after a 28-month follow-up period.In this report,we present a unique case of ectopic hamartomatous thymoma to show the existence of multiple tumors in this rare disease.We hope to raise the awareness of the possibility of this disease when the tumor rises in typical lower neck regions,especially when mixed soft tissue or fat components are shown by imaging examination.
基金This work was supported by grants from the Youth Doctor Collaborative Innovation Team Project(QC201803)of Shanghai Ninth People’s Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai Youth Top-Notch Talent Program(201809004)Chenguang Program supported by the Shanghai Education Development Foundation and the Shanghai Municipal Education Commission(19CG18)+2 种基金the Science and Technology Commission of Shanghai Municipality(19JC1413)Shanghai Rising Star Program(20QA1405600)innovative research team of high-level local universities in Shanghai(SSMU-ZDCX20180700).
文摘Background Inflammatory linear verrucous epidermal nevus(ILVEN)is a rare type of epidermal nevus that occurs in infancy and develops along Blaschko’s lines.Due to its rarity and clinical/histological similarities with other cutaneous disorders,correct diagnosis is sometimes difficult.In the present case,the whole exome sequencing of blood found no germline mutation,but the sequencing of the biopsy specimen revealed a novel potential pathogenic somatic mutation,C>T at rs200881715(p.R698X)in the cspp1 gene,which may provide new insight into the mechanism of ILVEN.Patient concerns A 10-year-old girl presented with linear,pruritic,erythematous,scaly,and exophytic verrucous skin lesions on her left thigh,buttock,and hand unilaterally along Blaschko’s lines from the third day after birth.Diagnosis Biopsy revealed psoriasiform acanthosis,papillary hyperplasia with orthohyperkeratosis,and parakeratosis of squamous epithelium,which are histological characteristics of ILVEN.Interventions Owing to the large lesion size and significant refractory response to previous treatments,multiple-stage surgical resections with a tissue expander were performed.Outcomes After surgical resection and defect reconstruction with a tissue expander,the outcome was satisfactory for the patient and her family.No recurrence was detected during the follow-up visits.Conclusion ILVEN is a rare type of cutaneous hyperplastic disease,and treatment is extremely challenging.Biopsy should be performed at the diagnostician’s discretion or if refractory to conservative treatments.Timely diagnosis and surgical intervention could result in satisfactory outcomes.
基金This work was supported by the National Natural Science Foundation of China(81802424).
文摘Dear Editor,Diffuse large B-cell lymphoma(DLBCL)is the most common and aggressive subtype of non-Hodgkin lymphoma(NHL),accounting for about 40%of all NHL cases[1].Lacking symptoms at early time and efficient therapeutic methods made DLBCL one of the most life-threatening types of hematopoietic malignancy[2,3].Therefore,identifying novel therapeutic biomarker for early detection and prognosis prediction is urgently needed.