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1.
BACKGROUNDPrimary intramedullary melanocytoma is an exceedingly rare type of primary melanocytic tumor in the central nervous system. Unfortunately, primary intramedullary melanocytoma lacks specificity in clinical symptoms and imaging features and there is currently no standard strategy for diagnosis or treatment.CASE SUMMARYA 52-year-old male patient suffered from weakness and numbness involving the bilateral lower limbs for 18 mo, and defecation and erectile dysfunction for 6 mo. Furthermore, these symptoms started to worsen for the last 3 mo. Preoperative magnetic resonance imaging (MRI) revealed an intramedullary tumor located at the T9-T10 level. In subsequently surgery, the maximal safe resection extent approached to 98%. The lesion was confirmed to be melanocytoma by pathological examination. In addition, the possibility of original melanocytoma outside the spinal cord was excluded after the examination of the whole body. Therefore, a diagnosis of primary intramedullary melanocytoma was established. The patient refused to accept radiotherapy or Gamma Knife, but MRI examination on July 28, 2020 showed no sign of development. In addition, on April 10, 2021, the recent review showed that the disorder of defecation and lower limbs improved further but erectile dysfunction benefited a little from the surgery.CONCLUSIONAfter diagnosing intramedullary melanocytoma by postoperative pathology, the inspection of the whole body contributed to excluding the possibility of metastasis from other regions and further suggested a diagnosis of primary intramedullary melanocytoma. Complete resection, adjuvant radiation, and regular review are critical. In addition, maximal safe resection also benefits prognosis while the tumor is difficult to be resected totally.  相似文献   

2.
BackgroundSpinal ependymoma is the most common intramedullary tumor in adults. This study was performed to evaluate whether intraoperative yellow fluorescence use enhances our ability to identify the tumor margin and residual tumor tissue in intramedullary spinal cord ependymoma resection. We also evaluated patients’ clinical conditions at a 3-month follow-up.MethodsWe retrospectively evaluated 56 patients with intramedullary ependymoma. Thirty minutes before anesthesia, the patients received intravenous sodium fluorescein injections. Tumor resection was performed under two illumination modes, traditional white light and yellow fluorescence, and the residual tumor tissue was detected. Magnetic resonance imaging was performed 3 months postoperatively to observe the tumor resection outcome and residual tumor tissue. The McCormick spinal cord function grade was evaluated preoperatively and 3 months postoperatively.ResultsThe total resection rate was 100.0% in all patients. Nine patients had no significant fluorescence imaging. After 3 months, patients with a spinal function grade of I to IV showed significant spinal function improvement. Magnetic resonance imaging showed no residual tumor tissue or recurrence.ConclusionSodium fluorescein aids in total excision of intramedullary spinal cord ependymoma and intraoperative residual tumor tissue identification. At the 3-month follow-up, the patients’ functional outcome in the fluorescein group was good.  相似文献   

3.
BACKGROUNDTuberculosis (TB) mostly attacks the lungs, and extrapulmonary TB involving the central nervous system is uncommon; among these cases, spinal intramedullary TB is even more rare. The clinical manifestations of spinal intramedullary TB are similar to those of intramedullary spinal cord tumors. Therefore, it is necessary to make a careful differential diagnosis of spinal intramedullary lesions to achieve the appropriate treatment and favorable prognosis. We report a rare case of a young male patient with paraplegia due to spinal intramedullary TB, which is uncommon and regrettable.CASE SUMMARYA 23-year-old male presented with fever accompanied by nausea and vomiting lasting for 2 mo and was then diagnosed with tubercular meningitis. After anti-TB treatment, his symptoms were significantly improved. However, 2 mo after the diagnosis of tubercular meningitis, the patient felt numbness below the costal arch level, which lasted for 1 wk, and he paid no attention to this symptom. What followed was paraplegia and urine/fecal incontinence. Magnetic resonance imaging of the thoracic spine showed a ring-enhanced intramedullary cord lesion at T8-T9. Lesion exploration showed enlargement of the spinal cord at T8-T9, and the lesion could be observed by incision. The lesion was adhered to the peripheral tissue and was grayish-white and tough with a poor blood supply and a diameter of approximately 0.8 cm. The lesion was resected completely. The results of pathological examination by both hematoxylin-eosin staining and acid-fast bacilli staining confirmed TB, accompanied by acute and chronic suppurative inflammation and granulation tissue formation. The patient was instructed to continue anti-TB treatment after the operation, but he did not follow the medical advice. Follow-up continued for ten years, the patient had persistent paraplegia, the numbness disappeared and urine/fecal sensation recovered.CONCLUSIONAlthough TB is a kind of benign disease, some cases progress rapidly. Moreover, spinal intramedullary TB may seriously endanger quality of life and still needs timely diagnosis and proper treatment.  相似文献   

4.
Ewing sarcoma (ES) is a highly aggressive bone and soft tissue tumor that occurs mainly in young children and adolescents and is associated with primary and metastatic disease. Intramedullary ES (either primary or secondary) is rare, and the ideal management remains inconclusive. We herein report intramedullary and extramedullary metastatic ES in a single patient. A 46-year-old woman was referred to our outpatient clinic from the oncology clinic with progressive paraparesis and paresthesia for 1 week prior to presentation. She had developed left clavicular ES 2 years earlier for which surgery and chemoradiotherapy had been performed. At the present evaluation, she was diagnosed with intramedullary thoracic and lumbar extradural masses. Thoracic surgery was performed, and a biopsy of the lesion was obtained. The diagnosis of ES was confirmed histopathologically, and she underwent adjuvant chemotherapy. Her neurological status did not improve after surgery, and she underwent rehabilitation and physical therapy. The lumbar lesion resolved with chemotherapy. Metastasis of ES to the spinal cord, especially intramedullary lesions, is extremely rare, and there is no standard management guideline. However, surgical decompression and adjuvant chemotherapy are the main treatments in these cases.  相似文献   

5.
Primary malignant melanoma originating in the small bowel is extremely rare. We report the case of a 55-year-old man who presented with a preoperative bleeding duodenal tumor. A standard pancreaticoduodenectomy was performed. Histopathological examination ascertained the diagnosis of a duodenal malignant melanoma with locoregional lymphatic spread. A thorough postoperative investigation did not reveal any primary melanotic lesions. Thus, the diagnosis of a primary melanoma originating from the duodenum was suggested. Fourteen months after surgery, the patient had no evidence of recurrence. Primary malignant melanoma of the duodenum is an existing, though unusual, oncologic entity. Aggressive surgery remains the treatment of choice offering both symptom palliation and long-term survival.  相似文献   

6.
卵巢原发性恶性黑色素瘤1例及文献复习   总被引:1,自引:0,他引:1  
目的 探讨卵巢原发性恶性黑色素的临床病理特征,及其与卵巢转移性肿瘤的鉴别诊断。方法 对1例原发于卵巢囊性畸胎瘤的恶性黑色素瘤标本,通过光镜及免疫组化等方法进行病理组织学观察。结果 组织学特征为:肿瘤细胞大小不等,形态多样,界限不清,呈透明细胞和/或印戒细胞样;瘤细胞内外可见一些粗大的黑色素颗粒,瘤组织内血管较多,核分裂现象多见,〉10个/10HPF。免疫组化HMB45、Vimentin及S-100  相似文献   

7.
BACKGROUNDEwing’s sarcoma (ES) is a highly aggressive bone malignancy. Extraskeletal ES (EES) originating in the spinal canal is extremely rare. Herein, we report on a rare case of EES with a primary lumbar spinal nerve root including the complete diagnosis and treatment.CASE SUMMARYA young female patient presented with a complaint of right lower limb pain for 1 mo. Magnetic resonance imaging (MRI) revealed an 11 mm × 14 mm × 31 mm mass in the lumbar epidural region extending at the fifth lumbar spine (L5) level toward the right L5 neural foramen. Our initial diagnosis was an epidural schwannoma. The patient underwent total laminectomy, tumor resection and pedicle screw internal fixation and the L5 root tumor was found to have been completely removed intraoperatively. Histopathological examination of the lesion showed a typical ES with a large number of small, round cells. Immunohistochemistry analysis indicated positive CD99 and S100. After surgery, the patient received chemotherapy and radiotherapy with a 1 year of follow-up and no recurrent tumors or new lesions were found upon spine MRI and positron emission tomography/computed tomography reexamination.CONCLUSIONClinically, ES outside the bone should be considered when nerve root tumors are encountered inside and outside the spinal canal and the diagnosis should be determined by pathological biopsy. After surgical resection, chemotherapy and radiotherapy should be performed. After treatment, active follow-up and regular review should be completed.  相似文献   

8.
BACKGROUNDPrimary malignant melanoma of the lung (PMML) is a rare and highly malignant tumor with a poor prognosis. Here, we report a PMML case diagnosed by computed tomography (CT)-guided percutaneous biopsy, describe its pathological features and review relevant literature to improve our understanding of this tumor.CASE SUMMARYA 64-year-old Chinese female presented with productive cough for 7 mo. A chest CT scan showed a large and space-occupying lesion in Lingual lobe. Positron emission tomography-CT revealed multiple nodules located in the superior lobe apicoposterior segment of her left lung. Brain magnetic resonance imaging showed numerous enhancing nodules, suggesting brain metastasis. Abdominal CT scan did not show any abnormalities. By CT-guided percutaneous biopsy, four pieces of gray and taupe tissues (1 cm length and 0.1 mm in diameter) were obtained. After pathologic examination, the tumor was found to consist of epidermal and nested small round cells, fibrosis and thin-walled blood vessels. The finding was suggestive of malignant melanoma. To confirm the diagnosis, pathological morphology and immunophenotypic features of the biopsy specimens were observed. The patient denied any history of skin tumors. No abnormal lesions were detected in other sites of the body. Molecular testing was positive for wild-type EGFR and KIT gene mutations. Finally, the clinical and pathological findings suggested PMML. CONCLUSIONPMML is very rare, and the percutaneous biopsy tissue is limited. Therefore, comprehensive consideration of histology, immunohistochemistry, imaging, and clinical information is important for the diagnosis of PMML.  相似文献   

9.
BACKGROUNDSynovial sarcoma (SS) is a highly malignant tumor of unknown histological origin. This tumor can occur in various parts of the body, including those without synovial structures, but mainly in and around the joints, mostly in the lower extremities. Primary intracranial SSs are remarkably rare. This paper aims to report a case of primary intracranial SS with hemorrhage.CASE SUMMARYA 35-year-old male patient suffered a headache and slurred speech during manual labor and was sent to the emergency department. Through imaging examination, the patient was considered to have high-grade glioma complicated with hemorrhage and was treated with craniotomy. Postoperative pathology revealed SS. positron emission tomography/computed tomography was performed, which ruled out the possibility of metastasis to the intracranial from other parts of the body. Postoperative radiotherapy was given to the patient, during which radiation necrosis occurred. Sixteen months after craniotomy, cranial magnetic resonance imaging revealed recurrence of the tumor.CONCLUSIONPrimary intracranial SS is a rare malignant tumor. Primary intracranial SS with hemorrhage and radiation necrosis should be carefully monitored during postoperative radiotherapy. Surgical resection of the tumor combined with postoperative radiotherapy and chemotherapy is currently used, but the prognosis is poor.  相似文献   

10.
ObjectiveTo report and compare spinal cord [18F]-fluorodeoxyglucose–positron emission tomography (FDG-PET) metabolism in 51 patients with active myelopathy.Patients and MethodsWe retrospectively identified patients from January 1, 2001, through December 31, 2011, with active myelopathy in whom FDG-PET was performed. Inclusion criteria were (1) intramedullary myelopathy, (2) neoplastic/inflammatory etiology, and (3) FDG-PET performed after myelopathy onset. Exclusion criteria were (1) extramedullary myelopathy, (2) radiation-associated myelopathy, (3) no pathological confirmation of neoplasm, and (4) inactive myelopathy. Diagnostic categories of nonsarcoid inflammatory, neoplastic, and neurosarcoid were based on their final myelopathic diagnosis. Two radiologists who independently assessed FDG-PET for spinal cord hypermetabolism and maximum standardized uptake value (SUVmax) were blinded to the underlying etiology.ResultsFifty-one patients (53% women) with a median age of 60 years (range, 20-82 years) were included. Inflammatory myelopathic diagnoses (n=24) were as follows: paraneoplastic (n=13), autoimmune/other (n=5), inflammatory demyelinating (n=4), and transverse myelitis (n=2). Neoplastic diagnoses (n=21) were as follows: intramedullary metastases (n=12), intramedullary lymphoma/leukemia (n=7), and primary intramedullary neoplasm (n=2). Six patients had neurosarcoid myelopathy. Spinal cord hypermetabolism was more common with neoplastic myelopathy than with nonsarcoid inflammatory myelopathy (17 of 21 [81%] vs 6 of 24 [25%]; P<.001). Agreement between radiologist’s assessments was excellent (κ=0.88). Median SUVmax was greater in neoplastic than in nonsarcoid inflammatory causes of myelopathy (3.3 g/mL vs 1.9 g/mL; P<.001). The FDG-PET hypermetabolism was seen in 3 of the 6 patients (50%) with neurosarcoid myelopathy (median SUVmax, 2.6 g/mL; range, 1.8-12.2 g/mL).ConclusionSpinal cord FDG-PET hypermetabolism in patients with active myelopathy may be reliably detected and was more common in neoplastic than in inflammatory myelopathies in this study. Future investigation of spinal cord FDG-PET is indicated to assess its potential contributions in evaluating active myelopathies.  相似文献   

11.
BACKGROUNDMultiple myeloma is a malignant hematological disease characterized by proliferation of monoclonal plasma cells mainly in the bone marrow. Extraosseous epidural plasmacytoma associated with myeloma arises from lymphoid tissue in the epidural space without focal vertebral involvement, and is rare.CASE SUMMARYA 52-year-old woman was diagnosed with kappa subtype nonsecretory multiple myeloma and presented with bilateral arm weakness 11 mo after completing multiple courses of chemotherapy. Spinal magnetic resonance imaging (MRI) showed a posterior C7–T3 epidural mass with spinal cord compression. After five courses of chemotherapy, follow-up MRI showed resolution of cord compression. A 54-year-old man presented with paraplegia 15 mo after a diagnosis of IgD kappa subtype multiple myeloma and completing multiple courses of chemotherapy. He underwent Th11 and L1 laminectomies for tumor resection because MRI showed an epidural mass causing cord compression. His-topathologic examination was consistent with IgD multiple myeloma. The patients have currently survived for 33 mo and 19 mo, respectively.CONCLUSIONIsolated extraosseous epidural plasmacytoma associated with multiple myeloma without bony involvement is difficult to diagnose by imaging. Definitive diagnosis requires pathological and immunohistochemical examination.  相似文献   

12.
脊髓髓内转移性肿瘤的MRI诊断及鉴别诊断   总被引:1,自引:0,他引:1  
目的分析脊髓髓内转移性肿瘤(ISCM)MRI表现及其同髓内常见肿瘤的鉴别要点,提高对脊髓内肿瘤的认识和诊断水平。方法回顾性分析11例临床及病理证实的ISCM病例的临床与MRI资料,所有病例均行MRI平扫及增强检查。结果4例经手术病理证实,7例经临床确诊[平均发病年龄(46.4±2.8)岁],6例为多发病灶:颈、胸髓、圆锥、马尾均可多节段发生;5例为单发病灶:3例位于圆锥,1例位于颈髓,1例位于颈髓、延髓交界部。MR平扫T1WI多表现为等、低信号,T2WI多表现为不均匀高信号,增强后病灶均明显强化,可表现为斑片状、环形、斑点状及结节状强化,伴随征象包括脊髓增粗、周围水肿、脊髓空洞等。结论MRI能较好地显示ISCM的内部结构及信号特点、明确肿瘤的范围及进展情况,有助于本病的诊断及鉴别诊断,但缺乏明确特征表现,确诊需结合临床资料综合分析。  相似文献   

13.
Primary spinal melanoma is a very rare condition: to date < 60 cases have been reported in the literature. A 48-year-old man presented with a 6-month history of upper- and lower-extremity numbness. Spinal magnetic resonance imaging (MRI) revealed a space-occupying lesion at the C2 - C6 level. This was confirmed as a melanoma by immunohistochemistry. Cerebral MRI showed multiple lesions with the same signal characteristics as those seen in the spinal lesion on MRI. Complete skin, mucosal and retinal examination failed to show any primary lesion, therefore a diagnosis of primary cervical melanoma with brain metastases was made. To our knowledge this is the first report of a primary melanoma of the cervical spine with cerebral metastases at the time of diagnosis. This article presents pertinent reported literature and discusses the aetiology, diagnosis, treatment and prognosis of this unusual condition.  相似文献   

14.
目的:探讨女性尿道恶性黑色素瘤的病理特点及鉴别诊断.方法:分析1例女性尿道原发性恶性黑色素瘤临床表现、组织病理学、免疫组化特征、诊断及鉴别诊断、治疗等.结果:尿道恶性黑色素瘤以尿道外口包块就诊.病理学特点:瘤细胞呈巢状或片状分布,大小不一,形态多样,细胞内外可见粗大黑色素颗粒;免疫组化:HMB45、vimentin及S-100呈阳性表达.结论:女性尿道原发性恶性黑色素少见,易误诊,病理诊断应结合病史,早期诊断,综合治疗.  相似文献   

15.
BACKGROUNDMost melanomas identified in the stomach are metastatic. The primary gastric melanoma (PGM) is extremely rare. As such, clinical reports of PGM are scarce in the literature, lending to the challenge of diagnosis and treatment.CASE SUMMARYA 31-year-old woman presented with a 1-mo history of dysphagia but no symptoms of abdominal pain, abdominal distension, nausea, vomiting, hematemesis, or melena. The patient reported an unintentional weight loss of 6 kg within that time. History-taking revealed no previous medical conditions or surgical events. Abdominal computed tomography at a local hospital had suggested gastric tumor. Endoscopic examination in our hospital found a large, irregular, black mass. Subsequent laparoscopic exploration found the tumor on the side of the stomach fundus penetrating through the serosa, and enlarged lymph nodes (groups 1, 3, 7, and 9) fused into a mass, surrounding the peripheral artery and inseparable. Postoperative immunohistochemistry suggested gastric malignant melanoma. Positron emission tomography-computed tomography confirmed PGM. Treatment with programmed cell death protein 1 antagonist (toripalimab) plus chemotherapy (paclitaxel) was initiated but discontinued upon tumor bleeding. At the last telephone follow-up, the patient reported poor general condition but was alive.CONCLUSIONAlthough unresolved and ongoing, this rare case of PGM expands the overall knowledge about this rare tumor’s diagnosis and management.  相似文献   

16.
BACKGROUNDGastrointestinal stromal tumor (GIST) usually originates in the stomach, followed by the small intestine, rectum, and other parts of the gastrointestinal tract. The most common sites of metastasis are the liver and peritoneum, whereas spinal metastases from GIST are extremely rare.CASE SUMMARYWe found a case of GIST with the first presentation of multilevel spinal metastases involving the thoracic and lumbar vertebrae. A 61-year-old Chinese man presented to our clinic because of pain in his lower back and hip for 10 d without cause. Subsequently, computed tomography (CT) and magnetic resonance imaging (MRI) revealed abnormal signals in the vertebral appendages of T12 and L4 accompanied by spinal canal stenosis, which was considered as tumor metastasis. As there were no metastases to vital organs, posterior thoracic and lumbar spinal decompression + adnexal mass resection + pedicle internal fixation was adopted to achieve local cure and prevent nerve compression. The results of histopathological studies were consistent with the metastasis of GIST. No local recurrence or new metastases were found at the 6-mo follow-up at the surgical site. The patient has no neurological symptoms at present. It is worth mentioning that a rectal mass was found and surgically removed 1 mo after the patient was discharged from hospital, and the pathological diagnosis of the mass was GIST.CONCLUSIONBy reviewing 26 previously reported cases of spinal metastasis in GIST, it was found that spinal metastasis of GIST has become more common in recent years, so the possibility of early spinal metastasis should be recognized. CT and MRI are of great value in the diagnosis of spinal metastatic tumors, and pathological biopsy is the gold standard for the diagnosis of metastatic tumors. It is safe and feasible to treat isolated spinal metastasis in GIST by excising metastatic masses, decompressing the spinal canal, and stabilizing the spine.  相似文献   

17.
BACKGROUNDSquamous cell carcinoma (SCC) of bone is usually caused by metastasis from the lungs, bladder, or other sites. Primary SCC of bone most frequently involves the skull bones, and primary involvement of other sites in the skeletal system is extremely rare. To date, only three such cases have been reported, which makes the diagnosis, treatment, and prognosis of this disease a challenge.CASE SUMMARYA 76-year-old Chinese man presented to our hospital with nonspecific pain and limited mobility in the right shoulder for 4 mo. He underwent three-dimensional computed tomography reconstruction and magnetic resonance imaging of the right shoulder, which revealed an osteolytic destructive lesion in the right scapula with invasion into the surrounding muscles and soft tissues. Ultrasound-guided core needle biopsy detected a malignant tumor, and immunohistochemical analysis revealed a poorly differentiated SCC. Wide excision of the right scapular bone was performed, and pathological examination of the surgical specimen confirmed the diagnosis. At the last follow-up examination within 2 years, the patient was doing well with the pain significantly relieved in the right shoulder.CONCLUSIONPrimary SCC of bone is extremely rare at sites other than the skull. Clinicians must exhaust all available means for the diagnosis of primary SCC of the bone, so greater attention can be paid to its timely and effective management. Regular and adequate follow-up is essential to help rule out metastasis and judge the prognosis.  相似文献   

18.
BACKGROUNDUveal melanoma is the most common primary intraocular malignant tumor affecting the eyes in adults. Nearly half of all primary uveal melanoma tumors metastasize; yet, there are currently no effective treatments for metastatic uveal melanoma. At the time of diagnosis, less than 4% of patients with uveal melanoma have detectable metastatic disease. Uveal melanoma disseminates hematogenously, with the most common site of metastasis being liver (93%), followed by lung (24%) and bone (16%).CASE SUMMARYA 57-year-old woman was diagnosed with a dysplastic nevus on her eyelid, which was histologically confirmed as malignant melanoma after resection. The patient had no evidence of metastasis to other organs and received both radiation therapy and chemotherapy. After systemic treatment, a metastatic left neck lymph node was found and another round of chemotherapy was performed after resection. Positron emission tomography-Computed Tomography tracking after completion of chemotherapy revealed two metastatic liver nodules. The patient underwent partial liver resection and showed no signs of recurrence at 1 year after surgery.CONCLUSIONSurgery is an effective treatment for metastatic uveal melanoma. In patients with liver metastatic lesions, hepatectomy improves outcome.  相似文献   

19.
BACKGROUNDColorectal mucinous adenocarcinoma is a distinct subtype of colorectal adenocarcinoma that is not sensitive to chemotherapy and radiotherapy, and its prognosis is worse than that of nonmucinous adenocarcinoma. Early diagnosis and aggressive surgical treatment may be the key to improving the prognosis of patients. Ascending colon mucinous adenocarcinoma with the primary manifestation of a local abscess caused by non-intestinal perforation has never been reported. Moreover, since the lumen of the ascending colon is large, and early stage ascending colon cancer lacks typical clinical manifestations, the diagnosis may be delayed easily. We herein report three cases of delayed diagnosis of colorectal mucinous adenocarcinoma.CASE SUMMARYWe present three patients (two females and one male) with mucinous ascending colon mucinous adenocarcinoma with the primary manifestation of a local abscess (the right area of the lumbar spine, right groin, and lower right abdomen) caused by non-intestinal perforation. At the initial clinical visit, the common causes of those abscesses, including spinal tuberculosis and urinary tract infection, were excluded. The treatment of the abscess was through an incision and drainage. However, the source of the abscess was not made clear, which led to an abscess recurrence and a delayed diagnosis of colorectal mucinous adenocarcinoma. After the patients were referred to our hospital, a definitive diagnosis of ascending colon mucinous adenocarcinoma was made with the help of tumor markers and colonoscopic findings. Because of the delayed diagnosis of the disease, two patients (case 1 and case 2) missed the chance of surgery due to disease progression and died in a short follow-up period. Only case 3 underwent radical surgery for the tumor in the right colon and partial abdominal wall resection and achieved a better prognosis.CONCLUSIONAbscesses in the right area of the lumbar spine, right groin, or right lower quadrant caused by non-intestinal perforation as the primary clinical manifestation of ascending colon mucinous adenocarcinoma are extremely rare. Mucinous adenocarcinoma of the ascending colon may be one of the causes of such abscesses. Performing colonoscopy as soon as possible is of great significance in the diagnosis and treatment of the disease.  相似文献   

20.
BACKGROUNDPrimary pancreatic paragangliomas are extremely rare tumors. Limited by the diagnostic efficacy of histopathological examination, their malignant behavior is thought to be associated with local invasion or metastasis, with only four malignant cases reported in the literature to date. As pancreatic paragangliomas share similar imaging features with other types of pancreatic neuroendocrine neoplasms, they are difficult to diagnose accurately without the support of pathological evidence. As primary pancreatic paragangliomas are rare, especially those accompanied by lymph node metastasis, there is currently no consensus on treatment. Herein, we report a case of primary pancreatic paraganglioma with lymph node metastasis.CASE SUMMARYA mass located in the pancreatic body was incidentally discovered on computed tomography in a 41-year-old Tibetan man. Distal pancreatectomy was subsequently performed and a 4.1 cm × 4.2 cm tumor was found embedded in the body of the pancreas during surgery. Histological examination confirmed the characteristics of paraganglioma in which the neoplastic chief cells were arranged in a classic Zellballen pattern under hematoxylin-eosin staining. Further, immunohistochemistry demonstrated that the sustentacular cells in the tumor tissue were positive for S-100 protein, and neoplastic cells and pancreatic draining lymph nodes were positive for chromogranin A and synaptophysin; thus, the presence of lymph node metastasis (two of the eight resected pancreatic draining lymph nodes) was also confirmed. A diagnosis of primary pancreatic paraganglioma with lymph node metastasis was finally established. The patient remained disease-free for 1 year after the surgery.CONCLUSIONA definite diagnosis of pancreatic paraganglioma mainly depends on postoperative histopathological and immunohistochemical examinations. Surgical resection may be the first treatment of choice for patients with primary pancreatic paraganglioma that has metastasized to the lymph nodes.  相似文献   

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