BACKGROUND Aggressive angiomyolipoma is an extremely rare benign mesenchymal tumor that was originally described as a locally recurrent mucinous spindle cell tumour.Agg-ressive angiomyolipoma originates from myofibrob...BACKGROUND Aggressive angiomyolipoma is an extremely rare benign mesenchymal tumor that was originally described as a locally recurrent mucinous spindle cell tumour.Agg-ressive angiomyolipoma originates from myofibroblasts,vascular smooth muscle cells,or fibroblasts,and displays various phenotypes of myofibroblasts and abnor-mal muscle arteries.Aggressive angiomyolipoma was first identified in 1983 and fewer than 50 male patients have been reported to date.It is an extremely rare mesenchymal tumour and often confused with other diseases.Patients with epididymal aggressive angiomyolipoma lack typical symptoms,most of which occur incidentally,although some patients may experience mild pain,discomfort,and swelling.Pain may be exacerbated by pressure from the mass.CASE SUMMARY A 66-year-old male was admitted to the hospital on January 14,2022 with chief complaint of swelling in the left scrotum for one year.There was no apparent cause for the swelling.The patient did not consult with any doctor or receive any treatment for the swelling.The enlarged scrotum increased in size gradually until it reached approximately the size of a goose egg,and was accompanied by discom-fort and swelling of the left cavity of the scrotum.The patient had no history of any testicular trauma,infection,or urinary tract infection.The patient urinated freely,1-2 times at night,without urgency,dysuria(painful urination),or haematuria.There was no significant family history of malignancy.The patient underwent excision of the enlarged tumour and the left epididymis under general anaes-thesia on January 18,2022.Twelve months of follow-up revealed no recurrence.The patient was satisfied with the treatment.CONCLUSION Aggressive angiomyolipoma is extremely rare clinically and often confused with other diseases.The pathogenesis of aggressive angiomyolipoma is unclear and the clinical presentation is mostly a painless enlarged mass.The diagnosis of aggressive angiomyolipoma requires a combination of medical history,preoperative imaging such as computed tomography and magnetic resonance imaging,cytological examination and preoperative and postoperative pathological biopsy.The preferred treatment is surgery,with the possibility of a new alternative treatment option after hormonal therapy.Aggressive angiomyolipoma should be considered in the differential diagnosis of parametrial tumors of the male genital area that present as clinically significant masses.The high recurrence rate of aggressive angiomyolipoma may be related to incomplete tumor resection,and patients with aggressive angiomyolipoma are advised to undergo annual postoperative follow-up and imaging for recurrence.展开更多
Aggressive angiomyxoma （AAM） is a rare tumour that often occurs in soft tissues of the female genital tract. Eight cases of AAM are reported in this article, and the clinical features and ultrasound and magnetic res...Aggressive angiomyxoma （AAM） is a rare tumour that often occurs in soft tissues of the female genital tract. Eight cases of AAM are reported in this article, and the clinical features and ultrasound and magnetic resonance imaging （MRI） results of the eight cases are reviewed and summarized. The main complaints of all the patients were palpable and painless masses in the vulva or scrotum. The lesions were mainly located in the vulva, pelvis, and perineal region, with a large scope of involvement. The sonographic features of AAM were characteristic. On sonography, all of the masses were of irregular shape and showed hypoechogenicity, with a heterogeneous inner echotexture. Intratumoural and peritumoural blood fows were detected by colour Doppler imaging. On real-time ultrasonic imaging, prominent deformation of the lesions was observed bycompressing the masses with the probe. Some special imaging features were also revealed, including a la-minated or swirled appearance of inner echogenicity, and a fnger-like or tongue-like growth pattern. On MRI imaging, the lesions showed intermediate-intensity signals and intermediate to high-intensity signals on TI-weighted and T2-weighted sequences. A rapid and uneven enhancement pattern was demonstrated. After the comparison of sonographic features with MRIand pathological findings, we found the relevance of the ultrasonographic characteristics with MRI and his-tological features of AAM. Ultrasound can be a valuable imaging method for the preoperative diagnosis, eva-luation of scope, and follow-up of AAM.展开更多
Aggressive angiomyxoma is rare mesenchymal tumor that most commonly arises in the vulvo-vaginal region, perineum, and pelvis of women. The term aggressively emphasizes the often infiltrative nature of the tumor and it...Aggressive angiomyxoma is rare mesenchymal tumor that most commonly arises in the vulvo-vaginal region, perineum, and pelvis of women. The term aggressively emphasizes the often infiltrative nature of the tumor and its frequent association with local recurrence. Preoperative diagnosis and treatment may be difficult. We report a 55-year-old patient who presented with a pelvic mass with no significant medical history. The radiological assessment showed a pelvic cystic tumor retro and lateral bladder. A surgical excision was performed with a diagnosis of aggressive angiomyxoma pelvis that misdiagnosed as ovarian tumor. The patient received adjuvant radiotherapy after surgery and remains well with no signs of local recurrence at 12 months follow-up. As surgery is the main treatment, preoperative diagnosis and assessment of extent of the tumor for planning surgery can not be overemphasized. The authors discuss this finding through the diagnostic difficulties and the evolution of this entity.展开更多
Objective To explore the clinicopathologic features of angiomyofibroblastomz and the relationship between angiomyofibroblastoma (AMFB)and aggressive angiomyxoma (AA ). Aethods Seven cases of AMFB and 5 coses of AA wer...Objective To explore the clinicopathologic features of angiomyofibroblastomz and the relationship between angiomyofibroblastoma (AMFB)and aggressive angiomyxoma (AA ). Aethods Seven cases of AMFB and 5 coses of AA were reviewed. Conventional histologic and immunohistochemical features of AMFB were compered with those of AA. Results The lesions of AMFB usually presented as painless masses and were located in the superficial vulvar regional and inguinal areas. Microscopically, the tumors were composed of spindled and epithelioid cells arranged in cords and nests preferentially arrayed around numerous small to medium-sized, thin-walled vessels with some hyalined thick walls. Seven cases manifested zone of both dense and sparse cellularity. Immunohistochemically, the tumor cells were strongly positive for vimentin and desmin. but negative for S-100 and cytokeratin 8. Some cells were positive for SMA, ER and PR. None of the 4 ams followed up over 2 years after simple excision developed a recurrence, in contract to AA that exhibited local recurrence in 3 of 4 cases. AA showed more dense myxoid hackground and thick walled vessels. Myoid bundles tended to be located adjacently to biood vessels in all cases. Ultrastructurally,AMFB tumor cells contained intercellular junction and cytoplastic microfilaments. Intercellular spaces contained a great of collagen fibers. Conclusion The results indicate that conventional morphologic analysis is paramount in the recognition of ANFB. AMFB and AA show different clinicoathologic entity and propose an origin from a perivascular stem cell that is ca- pable of myofibroblastic differentiation.展开更多
Aggressive angiomyxoma(AAM)is a rare soft tissue tumor.Invasiveness and recurrence are important clinical features of AAM.A patient with recurrent AAM was admitted to the hospital.At present,the treatment for AAM is s...Aggressive angiomyxoma(AAM)is a rare soft tissue tumor.Invasiveness and recurrence are important clinical features of AAM.A patient with recurrent AAM was admitted to the hospital.At present,the treatment for AAM is still based on surgery because it does not have lymph nodes and distant metastasis.Moreover,chemotherapy is of little significance.Traditional Chinese medicine believes the formation of tumor is related to blood stasis,so after surgery,a Chinese medicine called Xue-Shuan-Tong was used to improve blood circulation and disperses stasis.The drainage tube was removed on the 5th day after the operation and achieved a good wound healing.This article analyzes and demonstrates the pathogenesis of female vulvar invasive angiomyxoma from the perspective of modern medicine and traditional medicine,in order to provide a better understanding of the disease.展开更多
Aggressive angiomyxoma was first described in 1983 by Steeper and Rosai, and fewer than 150 cases have been reported in the world medical literature. It is a soft-tissue tumour of the pelvis and perineum. The recurren...Aggressive angiomyxoma was first described in 1983 by Steeper and Rosai, and fewer than 150 cases have been reported in the world medical literature. It is a soft-tissue tumour of the pelvis and perineum. The recurrence rate is high, and often extensive resections are performed with considerable morbidity. These tumours are benign, locally infiltrative mesenchymal neoplasms with a predilection for the female pelvis and perineum and they usually tend to recur. Furthermore, these tumours often reach too large dimensions before becoming clinically symptomatic;their incidence is higher in women of the reproductive age group;however a few cases of its occurrence outside the pelvis have also been reported. In this study, we reported three cases with aggressive pelvic angiomyxoma treated with surgical methods and used an approach that described by Kraske in order to get access to lower rectal cancers. Accurate preoperative diagnosis should alert the surgeon to the need for wide excision, which is essential for prevention of local recurrence.展开更多
文摘BACKGROUND Aggressive angiomyolipoma is an extremely rare benign mesenchymal tumor that was originally described as a locally recurrent mucinous spindle cell tumour.Agg-ressive angiomyolipoma originates from myofibroblasts,vascular smooth muscle cells,or fibroblasts,and displays various phenotypes of myofibroblasts and abnor-mal muscle arteries.Aggressive angiomyolipoma was first identified in 1983 and fewer than 50 male patients have been reported to date.It is an extremely rare mesenchymal tumour and often confused with other diseases.Patients with epididymal aggressive angiomyolipoma lack typical symptoms,most of which occur incidentally,although some patients may experience mild pain,discomfort,and swelling.Pain may be exacerbated by pressure from the mass.CASE SUMMARY A 66-year-old male was admitted to the hospital on January 14,2022 with chief complaint of swelling in the left scrotum for one year.There was no apparent cause for the swelling.The patient did not consult with any doctor or receive any treatment for the swelling.The enlarged scrotum increased in size gradually until it reached approximately the size of a goose egg,and was accompanied by discom-fort and swelling of the left cavity of the scrotum.The patient had no history of any testicular trauma,infection,or urinary tract infection.The patient urinated freely,1-2 times at night,without urgency,dysuria(painful urination),or haematuria.There was no significant family history of malignancy.The patient underwent excision of the enlarged tumour and the left epididymis under general anaes-thesia on January 18,2022.Twelve months of follow-up revealed no recurrence.The patient was satisfied with the treatment.CONCLUSION Aggressive angiomyolipoma is extremely rare clinically and often confused with other diseases.The pathogenesis of aggressive angiomyolipoma is unclear and the clinical presentation is mostly a painless enlarged mass.The diagnosis of aggressive angiomyolipoma requires a combination of medical history,preoperative imaging such as computed tomography and magnetic resonance imaging,cytological examination and preoperative and postoperative pathological biopsy.The preferred treatment is surgery,with the possibility of a new alternative treatment option after hormonal therapy.Aggressive angiomyolipoma should be considered in the differential diagnosis of parametrial tumors of the male genital area that present as clinically significant masses.The high recurrence rate of aggressive angiomyolipoma may be related to incomplete tumor resection,and patients with aggressive angiomyolipoma are advised to undergo annual postoperative follow-up and imaging for recurrence.
基金Supported by the International S and T Cooperation Program of China,No.2015DFA30440the National Natural Science Foundation of China,No.81301268Beijing Nova Plan,No.xxjc201812 and No.Z131107000413063
文摘Aggressive angiomyxoma （AAM） is a rare tumour that often occurs in soft tissues of the female genital tract. Eight cases of AAM are reported in this article, and the clinical features and ultrasound and magnetic resonance imaging （MRI） results of the eight cases are reviewed and summarized. The main complaints of all the patients were palpable and painless masses in the vulva or scrotum. The lesions were mainly located in the vulva, pelvis, and perineal region, with a large scope of involvement. The sonographic features of AAM were characteristic. On sonography, all of the masses were of irregular shape and showed hypoechogenicity, with a heterogeneous inner echotexture. Intratumoural and peritumoural blood fows were detected by colour Doppler imaging. On real-time ultrasonic imaging, prominent deformation of the lesions was observed bycompressing the masses with the probe. Some special imaging features were also revealed, including a la-minated or swirled appearance of inner echogenicity, and a fnger-like or tongue-like growth pattern. On MRI imaging, the lesions showed intermediate-intensity signals and intermediate to high-intensity signals on TI-weighted and T2-weighted sequences. A rapid and uneven enhancement pattern was demonstrated. After the comparison of sonographic features with MRIand pathological findings, we found the relevance of the ultrasonographic characteristics with MRI and his-tological features of AAM. Ultrasound can be a valuable imaging method for the preoperative diagnosis, eva-luation of scope, and follow-up of AAM.
文摘Aggressive angiomyxoma is rare mesenchymal tumor that most commonly arises in the vulvo-vaginal region, perineum, and pelvis of women. The term aggressively emphasizes the often infiltrative nature of the tumor and its frequent association with local recurrence. Preoperative diagnosis and treatment may be difficult. We report a 55-year-old patient who presented with a pelvic mass with no significant medical history. The radiological assessment showed a pelvic cystic tumor retro and lateral bladder. A surgical excision was performed with a diagnosis of aggressive angiomyxoma pelvis that misdiagnosed as ovarian tumor. The patient received adjuvant radiotherapy after surgery and remains well with no signs of local recurrence at 12 months follow-up. As surgery is the main treatment, preoperative diagnosis and assessment of extent of the tumor for planning surgery can not be overemphasized. The authors discuss this finding through the diagnostic difficulties and the evolution of this entity.
文摘Objective To explore the clinicopathologic features of angiomyofibroblastomz and the relationship between angiomyofibroblastoma (AMFB)and aggressive angiomyxoma (AA ). Aethods Seven cases of AMFB and 5 coses of AA were reviewed. Conventional histologic and immunohistochemical features of AMFB were compered with those of AA. Results The lesions of AMFB usually presented as painless masses and were located in the superficial vulvar regional and inguinal areas. Microscopically, the tumors were composed of spindled and epithelioid cells arranged in cords and nests preferentially arrayed around numerous small to medium-sized, thin-walled vessels with some hyalined thick walls. Seven cases manifested zone of both dense and sparse cellularity. Immunohistochemically, the tumor cells were strongly positive for vimentin and desmin. but negative for S-100 and cytokeratin 8. Some cells were positive for SMA, ER and PR. None of the 4 ams followed up over 2 years after simple excision developed a recurrence, in contract to AA that exhibited local recurrence in 3 of 4 cases. AA showed more dense myxoid hackground and thick walled vessels. Myoid bundles tended to be located adjacently to biood vessels in all cases. Ultrastructurally,AMFB tumor cells contained intercellular junction and cytoplastic microfilaments. Intercellular spaces contained a great of collagen fibers. Conclusion The results indicate that conventional morphologic analysis is paramount in the recognition of ANFB. AMFB and AA show different clinicoathologic entity and propose an origin from a perivascular stem cell that is ca- pable of myofibroblastic differentiation.
文摘Aggressive angiomyxoma(AAM)is a rare soft tissue tumor.Invasiveness and recurrence are important clinical features of AAM.A patient with recurrent AAM was admitted to the hospital.At present,the treatment for AAM is still based on surgery because it does not have lymph nodes and distant metastasis.Moreover,chemotherapy is of little significance.Traditional Chinese medicine believes the formation of tumor is related to blood stasis,so after surgery,a Chinese medicine called Xue-Shuan-Tong was used to improve blood circulation and disperses stasis.The drainage tube was removed on the 5th day after the operation and achieved a good wound healing.This article analyzes and demonstrates the pathogenesis of female vulvar invasive angiomyxoma from the perspective of modern medicine and traditional medicine,in order to provide a better understanding of the disease.
文摘Aggressive angiomyxoma was first described in 1983 by Steeper and Rosai, and fewer than 150 cases have been reported in the world medical literature. It is a soft-tissue tumour of the pelvis and perineum. The recurrence rate is high, and often extensive resections are performed with considerable morbidity. These tumours are benign, locally infiltrative mesenchymal neoplasms with a predilection for the female pelvis and perineum and they usually tend to recur. Furthermore, these tumours often reach too large dimensions before becoming clinically symptomatic;their incidence is higher in women of the reproductive age group;however a few cases of its occurrence outside the pelvis have also been reported. In this study, we reported three cases with aggressive pelvic angiomyxoma treated with surgical methods and used an approach that described by Kraske in order to get access to lower rectal cancers. Accurate preoperative diagnosis should alert the surgeon to the need for wide excision, which is essential for prevention of local recurrence.
