Extraskeletal myxoid chondrosarcoma (EMC) is a lowgrade sarcoma characterized by developing metastases and local recurrence in high rate. It is mainly deep seated in the proximal extremities. The most common metastati...Extraskeletal myxoid chondrosarcoma (EMC) is a lowgrade sarcoma characterized by developing metastases and local recurrence in high rate. It is mainly deep seated in the proximal extremities. The most common metastatic sites are the lungs, soft tissues, lymph nodes, bones and the brain. To our knowledge, no case of clearly defined EMC has been reported to date developing a metastasis in the pancreas. We describe a case of a man suffering from EMC who developed a single pancreatic metastasis 20 years after the initial diagnosis. A 49-year-old man was submitted to surgical excision of an EMC, in left thigh, 20 years ago. Fourteen years after the initial diagnosis a local recurrence in left thigh occurred. Multiple lesions of metastatic origin, in both lungs, were excised via thoracotomies until the time being. In 2003, as a part of a periodically performed imaging control, an abdominal CT scan was performed revealing a solid lesion in the pancreas. Distal pancreatectomy was performed. The histopathology of the excised specimen proved to be the one of metastatic lesion of EMC. The above-mentioned case of EMC is, as far as we know, the first one described developing a certain pancreatic metastasis.展开更多
The coexistence of myelolipoma within adrenal cortical adenoma is extremely rare,for both tumors present usually as separate entities.There are only 16 such cases reported worldwide.To the best of our knowledge,the ca...The coexistence of myelolipoma within adrenal cortical adenoma is extremely rare,for both tumors present usually as separate entities.There are only 16 such cases reported worldwide.To the best of our knowledge,the case we reported here is the first one of myxoid adrenal cortical adenoma associated with myelolipoma reported.A 32-year-old Chinese woman with 4-year history of hypertension was presented in our study.Computed tomography(CT)of the abdomen showed a large heterogene- ously-enhancing mass(4.5 cm in diameter)in the left suprarenal region.Clinical history and laboratory results suggest a metabolic disorder as Conn's syndrome.The patient underwent a left adrenalectomy,and a histopathological study confirmed the mass to be a myxoid adrenal cortical adenoma containing myelolipoma.The patient was postoperatively well and discharged uneventfully.In the present case report,we also discuss the etiology of simultaneous myelolipoma and adrenal adenoma associated with Conn's syndrome,and the methods of the diagnosis and differential diagnosis.展开更多
Objective： To investigate the Clinicopathological （EMC）. Methods： Nine cases of extraskeletal characteristics of extraskeletal myxoid chondrosarcoma myxoid chondrosarcoma were studied. Extensive immunohistochemic...Objective： To investigate the Clinicopathological （EMC）. Methods： Nine cases of extraskeletal characteristics of extraskeletal myxoid chondrosarcoma myxoid chondrosarcoma were studied. Extensive immunohistochemical analysis was performed in all the cases and ultrastructural studies were done in 2 extraskeletal myxoid chondrosarcomas. Follow-up information was available for seven patients. Results： There were 7 males and 2 females whose ages ranged from 31 to 69 years （median 52.78 years）. Local pain or tenderness and the presence of a palpable mass were the main complaints of the patients. The tumors were located mainly in the lower extremities （66.7%）. Most tumors were deep-seated. They usually had a distinct multinodular configuration delineated by fibrous connective tissue. The tumor cells were arranged in delicate intersecting strands, rings, and garlands for the most part. The myxoid matrix was abundant in most cases. Immunohistochemical analysis was performed in all the cases and ultrastructural studies were done in 2 extraskeletal myxoid chondrosarcomas. EMC expressed vimentin （100%, 9/9）, neuron-specific enolase （77.8%, 7/9）, S-100 protein （66.7%, 6/9）, synaptophysin and chromogranin A （22.2%, 2/9）. None of the tumors expressed EMA and desmin. Ultrastructurally： EMC was characterized by distinct cords of cells immersed in a glycosaminoglycan rich matrix. The cells were rich in mitochondria, had well-developed Golgi apparatus and there were numerous smooth vesicles. In many cells, there were also prominent glycogen deposits and lipid droplets. Some tumor cells had intracisternal microtubules. In one of the 2 extraskeletal myxoid chondrosarcomas there were 140-180 nm diameter membrane-bound dense-core secretory granules in cell bodies. Conclusion： Extraskeletal myxoid chondrosarcoma （EMC） is a rare soft tissue sarcoma characterized by distinctive morphological and cytogenetical features. However, the chondroid nature has been a subject of controversy, and its line of differentiation remains to be determined. A substantial proportion of EMC shows immunophenotypic and/or ultrastructural evidence of neuroendocrine differentiation. EMC has high potential of local recurrence and metastasis, and a high disease-associated death rate.展开更多
BACKGROUND Primary intracranial extraskeletal myxoid chondrosarcoma(EMC)is an extremely rare low-to intermediate-grade malignant soft tissue sarcoma,and only 15 cases have been reported in the literature.Due to its ra...BACKGROUND Primary intracranial extraskeletal myxoid chondrosarcoma(EMC)is an extremely rare low-to intermediate-grade malignant soft tissue sarcoma,and only 15 cases have been reported in the literature.Due to its rarity,clinical data and research on this tumor type are extremely limited,the pathogenesis and histological origin are still unclear,and the diagnostic and standard clinical treatment strategies for intracranial EMC remain controversial and undefined.CASE SUMMARY We reported a case of a 52-year-old male who was admitted to the hospital with headache and dizziness for 1 mo,and his health status deteriorated during the last week.CT of the head showed a well-defined low-density lesion situated in the left cavernous sinus.Brain magnetic resonance imaging(MRI)showed a 3.4 cm×3.0 cm sized,well-defined,round-shaped and heterogeneously enhanced lesion located in the left cavernous sinus.The entire lesion was removed via supratentorial craniotomy and microsurgery.Postoperative pathological diagnosis indicated primary intracranial EMC.Subsequently,the patient underwent 45 Gy/15 F stereotactic radiotherapy after discharge.At present,it is 12 mo after surgery,with regular postoperative follow-up and regular MRI examinations,that there are no clinical symptoms and radiographic evidence indicating the recurrence of the tumor,and the patient has returned to normal life.CONCLUSION Currently,the most beneficial treatment for primary intracranial EMC is gross total resection combined with postoperative radiotherapy.Long-term follow-up is also necessary for patients.展开更多
Myxoid adrenal cortical adenoma is a rare tumor and till now only 9 cases have been presented in the world. We here report another case of myxoid adenoma of the adrenal gland in a 45-year-old Chinese man who was admit...Myxoid adrenal cortical adenoma is a rare tumor and till now only 9 cases have been presented in the world. We here report another case of myxoid adenoma of the adrenal gland in a 45-year-old Chinese man who was admitted to hospital because of the right adrenal mass and mild hypertension. At surgery, the mass was well-circumscribed, measured 3.3 cm in diameter. Light-microscopic findings showed most of the tumor region with myxoid stroma, and the tumor cells were benign-look- ing. Immunohistochemical study showed the tumor had the positivity for vimentin, synaptophysin, neuron specific endolase but negative with cytokeratin and epithelial membrane antigen. Moreover, it was negative with alpha-inhibin that is not in accordance with those reported. There was no finding corresponding to malignancy.展开更多
Epithelioid gastrointesinal tumors (GISTs) are less likely to have c-kit gene mutations (and express CD117) than spindle cell GISTs. CD117 negative/c-kit negative GISTs can have platelet-derived growth factor alpha (P...Epithelioid gastrointesinal tumors (GISTs) are less likely to have c-kit gene mutations (and express CD117) than spindle cell GISTs. CD117 negative/c-kit negative GISTs can have platelet-derived growth factor alpha (PDGFRα) gene mutations, overexpress PDGFRα protein and respond to imatinib mesylate. Many cases of CD117-negative/CD117-weakly positive, c-kit mutation negative and PDGFRα mutation positive myxoid epithelioid GISTs and one case of CD117-positive GIST with chondro-myxoid features mimicking chondrosarcoma have been reported. We report a case of myxoid epithelioid GIST with predominance of chondroid and chordoid areas resembling an extraskeletal myxoid chondrosarcoma that was strongly positive for CD117, PDGFRα and DOG1 (Discovered on GIST 1) by immunohistochemistry, but lacked c-kit and PDGFRα gene mutations. It is possible that CD117 is strongly positive if a myxoid epithelioid GIST has chondroid/chordoid appearance, but a larger study is needed to confirm this association. CD117 expression in GISTs is important, because GISTs showing CD117 positivity respond to imatinib. No comment can be made about the prognostic significance of chondroid/chordoid appearance in the GISTs.展开更多
We present a case of a Myxoid Meningioma noticed in a 43-year-old woman. The patient, who had no noteworthy medical antecedents, showed repeated focal epileptic crisis. Imaging studies (MR and CT) showed a solitary an...We present a case of a Myxoid Meningioma noticed in a 43-year-old woman. The patient, who had no noteworthy medical antecedents, showed repeated focal epileptic crisis. Imaging studies (MR and CT) showed a solitary and well delimited tumour located in the frontral lobe. Once extirpated, the histological study revealed a neoplasm of myxoid and benign appearance, with elongated and uniform cells, with no atypias and a very low mitotic index. Immunohistochemisty studies (positivity for Vimentin and Epithelial Membrane Antigen, and negativity for the other markers) as well as physical and radiological examination of the patient (absence of tumours in any other location), suggested the diagnosis of a Myxoid Meningioma, which was confirmed by electron microscopy (by the presence of desmosomes). We also revise the characteristics of Myxoid Meningiomas, an absolutely exceptional primary tumour of the central nervous system.展开更多
BACKGROUND Oral liposarcoma is an extremely rare lesion that is often clinically misdiagnosed as a benign tumor due to its asymptomatic and indolent clinical course.Here,we report a case of massive low-grade myxoid li...BACKGROUND Oral liposarcoma is an extremely rare lesion that is often clinically misdiagnosed as a benign tumor due to its asymptomatic and indolent clinical course.Here,we report a case of massive low-grade myxoid liposarcoma(MLS)of the floor of the mouth.CASE SUMMARY A 71-year-old man presented with a huge mass in the left floor of the mouth.A biopsy was performed,and a diagnosis of a myxoid tumor suspicious for lowgrade MLS or myxoma was made.Gadolinium-enhanced T1-weighted magnetic resonance imaging showed an intensely enhanced tumor lesion that occupies the left sublingual space and extends to the submandibular space.Submandibular dissection,tumor resection,and reconstruction with a radial forearm flap were performed.The surgical specimen exhibited histologically low-grade MLS.Fused in sarcoma(FUS,also known as TLS)and DNA damage-inducible transcript 3(DDIT3,also known as CHOP)break-apart was not detected in the fluorescence in situ hybridization analysis.The tumor was completely encapsulated and did not require additional treatment.Furthermore,no recurrence was reported 40 mo after surgery.CONCLUSION We experienced an extremely rare,massive,low-grade MLS emerging from the floor of the mouth.Oftentimes,an MLS of the floor of the mouth lacks significant clinical findings and is often misdiagnosed.Although no FUS-DDIT3 fusion gene was detected,a low-grade MLS was ultimately diagnosed based on the histological findings.展开更多
Neurogenic tumours constitute a very small percentage of all neoplastic lesions of the head and neck region. Neurogenic tumors of the larynx are extremely rare. Two types of neurogenic tumors must be distinguished: Sc...Neurogenic tumours constitute a very small percentage of all neoplastic lesions of the head and neck region. Neurogenic tumors of the larynx are extremely rare. Two types of neurogenic tumors must be distinguished: Schwannomas and neurofibromas. Neurofibromas arise from the sheath of nerve fibers and are characterized by prominent collagen produc- tion and trapped nerve fibers. In the larynx, the most common sites of origin are arytenoids and aryepiglottic folds. Because of benign nature of this tumor, conservative surgery is recommended as the management of choice. Only few cases of laryngeal myxoid neurofibroma have been reported in literature and this article presents one case of laryngeal myxoid neurofibroma, which at first was misdiagnosed as myxoma, with brief review of literatures.展开更多
We present a case of a myxoid and lipomatous solitary fibrous tumor that was observed in a 67-year-old man. The tumor, which had a maximum diameter of 10 cm, was located in the soft tissues of the dorsal region and ap...We present a case of a myxoid and lipomatous solitary fibrous tumor that was observed in a 67-year-old man. The tumor, which had a maximum diameter of 10 cm, was located in the soft tissues of the dorsal region and appeared macroscopically well delimited and encapsulated. Upon cutting, a markedly gelatinous internal surface was observed. A microscopic study revealed an intense and diffusely myxoid neoplasia, with small areas of adipose aspect, in which histological (staghorn vessels, perivascular hyalinization, fusiform cells of benign aspect) and immunohistochemical (intensive positivity for CD34, Bcl-2 and Cd99 and negativity for muscle markers) data were consistent with a solitary fibrous tumor were observed. To conclude, the main characteristics of this lesion are discussed, and a differential diagnosis is established with other entities.展开更多
Myxoid liposarcoma is the most common form of myxoid sarcoma. Their primary and superficial localizations are very rare compared to the deeper forms. We report the case of myxoid liposarcoma in a 57-year-old patient. ...Myxoid liposarcoma is the most common form of myxoid sarcoma. Their primary and superficial localizations are very rare compared to the deeper forms. We report the case of myxoid liposarcoma in a 57-year-old patient. The clinical picture marked by painless swelling of the right thigh had evolved over a period of about ten years. The diagnosis was guided by ultrasound and magnetic resonance imaging. A pathological examination of the surgical specimen after removal of the tumor helped to clarify the diagnosis. We discuss the clinical presentation and therapeutic management.展开更多
Myxoid degeneration of ovarian tumors represents a rare distinctive benign ovarian stromal neoplasm that occurs predominantly in young women and is hormonally inactive.According to recent bibliography,pathogenesis rem...Myxoid degeneration of ovarian tumors represents a rare distinctive benign ovarian stromal neoplasm that occurs predominantly in young women and is hormonally inactive.According to recent bibliography,pathogenesis remains controversial.Many conducted studies express the strong belief adjusting myxoid ovarian tumors with genetic abnormalities.Therapeutic mapping is associated with histologic confirmation of the lesion.In cases of premenopausal patients,fertility preservation consists of ultimate scope.Abdominal MRI(magnetic resonance imaging)along with transvaginal ultrasound can differentiate and depict all preoperative imaging findings.Myxoid degenerated ovarian tumors can be malignant transformed into myxoid leiomyosarcomas with extremely metastatic possibilities.Meticulous atomic history,proper laboratory and imaging findings reflect successful key concerning ultimate diagnosis and treatment.展开更多
Liposarcoma is a malignant neoplasm of mesodermal origin, which among sarcomas, 10% to 20% are located in the retroperitoneum. The case presented shows a 50-year-old male patient who initially presented weight loss a...Liposarcoma is a malignant neoplasm of mesodermal origin, which among sarcomas, 10% to 20% are located in the retroperitoneum. The case presented shows a 50-year-old male patient who initially presented weight loss and abdominal pain in the left iliac fossa. A tumor mass of hardened consistency was palpated in virtually all the abdomen. An abdominal ultrasound and a computed tomography of the abdomen were performed and confirmed the tumor mass. An exploratory laparotomy was performed, with removal of bulky abdominal mass of greasy consistency. A histopathological study of the piece reported myxoid liposarcoma. Clinical and prognostic features, as well as oncologic outcomes, are well known in this group of patients. The patient has been in the outpatient clinic for 7 years without tumor recurrence. Computed tomography is the fundamental study for the diagnosis of imaging. The treatment of choice consists in an aggressive approach aiming the complete resection, which is a major predictor of local and distant recurrence and survival.展开更多
Myxoid change in adrenocortical carcinoma is a rare phenomenon,and no case has been reported in Chinese population to date.We report here a case of myxoid adrenocortical carcinoma presenting as Cushing syndrome with a...Myxoid change in adrenocortical carcinoma is a rare phenomenon,and no case has been reported in Chinese population to date.We report here a case of myxoid adrenocortical carcinoma presenting as Cushing syndrome with an immunohistochemical study of the tumor and discuss the diagnosis with reference to the current literature.展开更多
Myxoid adrenocortical adenomas are extremely rare .with only 24 cases reported, The first case wasreported by Tang et al in 1979 which was diagnosed as a myxoid adrenocortical carcinoma. Nearly all the reports were of...Myxoid adrenocortical adenomas are extremely rare .with only 24 cases reported, The first case wasreported by Tang et al in 1979 which was diagnosed as a myxoid adrenocortical carcinoma. Nearly all the reports were of single case except Brown et al who described a group of 14 cases. We report here an additional case of myxoid adrenocortical adenoma with an immunohistochemical study of the tumor and discuss the diagnosis with reference to the current literature.展开更多
Myxoid adrenocortical neoplasms are rare. Surgical resection of the mass is the first-line therapy. Here we reported a total of four patients, aged 44-66 years, diagnosed with myxoid adrenocortical tumor. The clinical...Myxoid adrenocortical neoplasms are rare. Surgical resection of the mass is the first-line therapy. Here we reported a total of four patients, aged 44-66 years, diagnosed with myxoid adrenocortical tumor. The clinical characteristics and immunohistochemical features of the tumor are discussed in the current literature.展开更多
文摘Extraskeletal myxoid chondrosarcoma (EMC) is a lowgrade sarcoma characterized by developing metastases and local recurrence in high rate. It is mainly deep seated in the proximal extremities. The most common metastatic sites are the lungs, soft tissues, lymph nodes, bones and the brain. To our knowledge, no case of clearly defined EMC has been reported to date developing a metastasis in the pancreas. We describe a case of a man suffering from EMC who developed a single pancreatic metastasis 20 years after the initial diagnosis. A 49-year-old man was submitted to surgical excision of an EMC, in left thigh, 20 years ago. Fourteen years after the initial diagnosis a local recurrence in left thigh occurred. Multiple lesions of metastatic origin, in both lungs, were excised via thoracotomies until the time being. In 2003, as a part of a periodically performed imaging control, an abdominal CT scan was performed revealing a solid lesion in the pancreas. Distal pancreatectomy was performed. The histopathology of the excised specimen proved to be the one of metastatic lesion of EMC. The above-mentioned case of EMC is, as far as we know, the first one described developing a certain pancreatic metastasis.
文摘The coexistence of myelolipoma within adrenal cortical adenoma is extremely rare,for both tumors present usually as separate entities.There are only 16 such cases reported worldwide.To the best of our knowledge,the case we reported here is the first one of myxoid adrenal cortical adenoma associated with myelolipoma reported.A 32-year-old Chinese woman with 4-year history of hypertension was presented in our study.Computed tomography(CT)of the abdomen showed a large heterogene- ously-enhancing mass(4.5 cm in diameter)in the left suprarenal region.Clinical history and laboratory results suggest a metabolic disorder as Conn's syndrome.The patient underwent a left adrenalectomy,and a histopathological study confirmed the mass to be a myxoid adrenal cortical adenoma containing myelolipoma.The patient was postoperatively well and discharged uneventfully.In the present case report,we also discuss the etiology of simultaneous myelolipoma and adrenal adenoma associated with Conn's syndrome,and the methods of the diagnosis and differential diagnosis.
文摘Objective： To investigate the Clinicopathological （EMC）. Methods： Nine cases of extraskeletal characteristics of extraskeletal myxoid chondrosarcoma myxoid chondrosarcoma were studied. Extensive immunohistochemical analysis was performed in all the cases and ultrastructural studies were done in 2 extraskeletal myxoid chondrosarcomas. Follow-up information was available for seven patients. Results： There were 7 males and 2 females whose ages ranged from 31 to 69 years （median 52.78 years）. Local pain or tenderness and the presence of a palpable mass were the main complaints of the patients. The tumors were located mainly in the lower extremities （66.7%）. Most tumors were deep-seated. They usually had a distinct multinodular configuration delineated by fibrous connective tissue. The tumor cells were arranged in delicate intersecting strands, rings, and garlands for the most part. The myxoid matrix was abundant in most cases. Immunohistochemical analysis was performed in all the cases and ultrastructural studies were done in 2 extraskeletal myxoid chondrosarcomas. EMC expressed vimentin （100%, 9/9）, neuron-specific enolase （77.8%, 7/9）, S-100 protein （66.7%, 6/9）, synaptophysin and chromogranin A （22.2%, 2/9）. None of the tumors expressed EMA and desmin. Ultrastructurally： EMC was characterized by distinct cords of cells immersed in a glycosaminoglycan rich matrix. The cells were rich in mitochondria, had well-developed Golgi apparatus and there were numerous smooth vesicles. In many cells, there were also prominent glycogen deposits and lipid droplets. Some tumor cells had intracisternal microtubules. In one of the 2 extraskeletal myxoid chondrosarcomas there were 140-180 nm diameter membrane-bound dense-core secretory granules in cell bodies. Conclusion： Extraskeletal myxoid chondrosarcoma （EMC） is a rare soft tissue sarcoma characterized by distinctive morphological and cytogenetical features. However, the chondroid nature has been a subject of controversy, and its line of differentiation remains to be determined. A substantial proportion of EMC shows immunophenotypic and/or ultrastructural evidence of neuroendocrine differentiation. EMC has high potential of local recurrence and metastasis, and a high disease-associated death rate.
文摘BACKGROUND Primary intracranial extraskeletal myxoid chondrosarcoma(EMC)is an extremely rare low-to intermediate-grade malignant soft tissue sarcoma,and only 15 cases have been reported in the literature.Due to its rarity,clinical data and research on this tumor type are extremely limited,the pathogenesis and histological origin are still unclear,and the diagnostic and standard clinical treatment strategies for intracranial EMC remain controversial and undefined.CASE SUMMARY We reported a case of a 52-year-old male who was admitted to the hospital with headache and dizziness for 1 mo,and his health status deteriorated during the last week.CT of the head showed a well-defined low-density lesion situated in the left cavernous sinus.Brain magnetic resonance imaging(MRI)showed a 3.4 cm×3.0 cm sized,well-defined,round-shaped and heterogeneously enhanced lesion located in the left cavernous sinus.The entire lesion was removed via supratentorial craniotomy and microsurgery.Postoperative pathological diagnosis indicated primary intracranial EMC.Subsequently,the patient underwent 45 Gy/15 F stereotactic radiotherapy after discharge.At present,it is 12 mo after surgery,with regular postoperative follow-up and regular MRI examinations,that there are no clinical symptoms and radiographic evidence indicating the recurrence of the tumor,and the patient has returned to normal life.CONCLUSION Currently,the most beneficial treatment for primary intracranial EMC is gross total resection combined with postoperative radiotherapy.Long-term follow-up is also necessary for patients.
文摘Myxoid adrenal cortical adenoma is a rare tumor and till now only 9 cases have been presented in the world. We here report another case of myxoid adenoma of the adrenal gland in a 45-year-old Chinese man who was admitted to hospital because of the right adrenal mass and mild hypertension. At surgery, the mass was well-circumscribed, measured 3.3 cm in diameter. Light-microscopic findings showed most of the tumor region with myxoid stroma, and the tumor cells were benign-look- ing. Immunohistochemical study showed the tumor had the positivity for vimentin, synaptophysin, neuron specific endolase but negative with cytokeratin and epithelial membrane antigen. Moreover, it was negative with alpha-inhibin that is not in accordance with those reported. There was no finding corresponding to malignancy.
文摘Epithelioid gastrointesinal tumors (GISTs) are less likely to have c-kit gene mutations (and express CD117) than spindle cell GISTs. CD117 negative/c-kit negative GISTs can have platelet-derived growth factor alpha (PDGFRα) gene mutations, overexpress PDGFRα protein and respond to imatinib mesylate. Many cases of CD117-negative/CD117-weakly positive, c-kit mutation negative and PDGFRα mutation positive myxoid epithelioid GISTs and one case of CD117-positive GIST with chondro-myxoid features mimicking chondrosarcoma have been reported. We report a case of myxoid epithelioid GIST with predominance of chondroid and chordoid areas resembling an extraskeletal myxoid chondrosarcoma that was strongly positive for CD117, PDGFRα and DOG1 (Discovered on GIST 1) by immunohistochemistry, but lacked c-kit and PDGFRα gene mutations. It is possible that CD117 is strongly positive if a myxoid epithelioid GIST has chondroid/chordoid appearance, but a larger study is needed to confirm this association. CD117 expression in GISTs is important, because GISTs showing CD117 positivity respond to imatinib. No comment can be made about the prognostic significance of chondroid/chordoid appearance in the GISTs.
文摘We present a case of a Myxoid Meningioma noticed in a 43-year-old woman. The patient, who had no noteworthy medical antecedents, showed repeated focal epileptic crisis. Imaging studies (MR and CT) showed a solitary and well delimited tumour located in the frontral lobe. Once extirpated, the histological study revealed a neoplasm of myxoid and benign appearance, with elongated and uniform cells, with no atypias and a very low mitotic index. Immunohistochemisty studies (positivity for Vimentin and Epithelial Membrane Antigen, and negativity for the other markers) as well as physical and radiological examination of the patient (absence of tumours in any other location), suggested the diagnosis of a Myxoid Meningioma, which was confirmed by electron microscopy (by the presence of desmosomes). We also revise the characteristics of Myxoid Meningiomas, an absolutely exceptional primary tumour of the central nervous system.
文摘BACKGROUND Oral liposarcoma is an extremely rare lesion that is often clinically misdiagnosed as a benign tumor due to its asymptomatic and indolent clinical course.Here,we report a case of massive low-grade myxoid liposarcoma(MLS)of the floor of the mouth.CASE SUMMARY A 71-year-old man presented with a huge mass in the left floor of the mouth.A biopsy was performed,and a diagnosis of a myxoid tumor suspicious for lowgrade MLS or myxoma was made.Gadolinium-enhanced T1-weighted magnetic resonance imaging showed an intensely enhanced tumor lesion that occupies the left sublingual space and extends to the submandibular space.Submandibular dissection,tumor resection,and reconstruction with a radial forearm flap were performed.The surgical specimen exhibited histologically low-grade MLS.Fused in sarcoma(FUS,also known as TLS)and DNA damage-inducible transcript 3(DDIT3,also known as CHOP)break-apart was not detected in the fluorescence in situ hybridization analysis.The tumor was completely encapsulated and did not require additional treatment.Furthermore,no recurrence was reported 40 mo after surgery.CONCLUSION We experienced an extremely rare,massive,low-grade MLS emerging from the floor of the mouth.Oftentimes,an MLS of the floor of the mouth lacks significant clinical findings and is often misdiagnosed.Although no FUS-DDIT3 fusion gene was detected,a low-grade MLS was ultimately diagnosed based on the histological findings.
文摘Neurogenic tumours constitute a very small percentage of all neoplastic lesions of the head and neck region. Neurogenic tumors of the larynx are extremely rare. Two types of neurogenic tumors must be distinguished: Schwannomas and neurofibromas. Neurofibromas arise from the sheath of nerve fibers and are characterized by prominent collagen produc- tion and trapped nerve fibers. In the larynx, the most common sites of origin are arytenoids and aryepiglottic folds. Because of benign nature of this tumor, conservative surgery is recommended as the management of choice. Only few cases of laryngeal myxoid neurofibroma have been reported in literature and this article presents one case of laryngeal myxoid neurofibroma, which at first was misdiagnosed as myxoma, with brief review of literatures.
文摘We present a case of a myxoid and lipomatous solitary fibrous tumor that was observed in a 67-year-old man. The tumor, which had a maximum diameter of 10 cm, was located in the soft tissues of the dorsal region and appeared macroscopically well delimited and encapsulated. Upon cutting, a markedly gelatinous internal surface was observed. A microscopic study revealed an intense and diffusely myxoid neoplasia, with small areas of adipose aspect, in which histological (staghorn vessels, perivascular hyalinization, fusiform cells of benign aspect) and immunohistochemical (intensive positivity for CD34, Bcl-2 and Cd99 and negativity for muscle markers) data were consistent with a solitary fibrous tumor were observed. To conclude, the main characteristics of this lesion are discussed, and a differential diagnosis is established with other entities.
文摘Myxoid liposarcoma is the most common form of myxoid sarcoma. Their primary and superficial localizations are very rare compared to the deeper forms. We report the case of myxoid liposarcoma in a 57-year-old patient. The clinical picture marked by painless swelling of the right thigh had evolved over a period of about ten years. The diagnosis was guided by ultrasound and magnetic resonance imaging. A pathological examination of the surgical specimen after removal of the tumor helped to clarify the diagnosis. We discuss the clinical presentation and therapeutic management.
文摘Myxoid degeneration of ovarian tumors represents a rare distinctive benign ovarian stromal neoplasm that occurs predominantly in young women and is hormonally inactive.According to recent bibliography,pathogenesis remains controversial.Many conducted studies express the strong belief adjusting myxoid ovarian tumors with genetic abnormalities.Therapeutic mapping is associated with histologic confirmation of the lesion.In cases of premenopausal patients,fertility preservation consists of ultimate scope.Abdominal MRI(magnetic resonance imaging)along with transvaginal ultrasound can differentiate and depict all preoperative imaging findings.Myxoid degenerated ovarian tumors can be malignant transformed into myxoid leiomyosarcomas with extremely metastatic possibilities.Meticulous atomic history,proper laboratory and imaging findings reflect successful key concerning ultimate diagnosis and treatment.
文摘Liposarcoma is a malignant neoplasm of mesodermal origin, which among sarcomas, 10% to 20% are located in the retroperitoneum. The case presented shows a 50-year-old male patient who initially presented weight loss and abdominal pain in the left iliac fossa. A tumor mass of hardened consistency was palpated in virtually all the abdomen. An abdominal ultrasound and a computed tomography of the abdomen were performed and confirmed the tumor mass. An exploratory laparotomy was performed, with removal of bulky abdominal mass of greasy consistency. A histopathological study of the piece reported myxoid liposarcoma. Clinical and prognostic features, as well as oncologic outcomes, are well known in this group of patients. The patient has been in the outpatient clinic for 7 years without tumor recurrence. Computed tomography is the fundamental study for the diagnosis of imaging. The treatment of choice consists in an aggressive approach aiming the complete resection, which is a major predictor of local and distant recurrence and survival.
文摘Myxoid change in adrenocortical carcinoma is a rare phenomenon,and no case has been reported in Chinese population to date.We report here a case of myxoid adrenocortical carcinoma presenting as Cushing syndrome with an immunohistochemical study of the tumor and discuss the diagnosis with reference to the current literature.
文摘Myxoid adrenocortical adenomas are extremely rare .with only 24 cases reported, The first case wasreported by Tang et al in 1979 which was diagnosed as a myxoid adrenocortical carcinoma. Nearly all the reports were of single case except Brown et al who described a group of 14 cases. We report here an additional case of myxoid adrenocortical adenoma with an immunohistochemical study of the tumor and discuss the diagnosis with reference to the current literature.
基金This work was supported by the grants from the Shanghai Municipal Natural Science Foundation （No. 09ZR1418500） and the Innovation Program of Shanghai Municipal Education Commission （No. llYZ58）.
文摘Myxoid adrenocortical neoplasms are rare. Surgical resection of the mass is the first-line therapy. Here we reported a total of four patients, aged 44-66 years, diagnosed with myxoid adrenocortical tumor. The clinical characteristics and immunohistochemical features of the tumor are discussed in the current literature.
