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Internal Medicine (Tokyo, Japan) 2012We herein report the case of a 41-year-old male patient with an incidentally identified large adrenal ganglioneuroma (GN). His endocrine examinations were normal except...
We herein report the case of a 41-year-old male patient with an incidentally identified large adrenal ganglioneuroma (GN). His endocrine examinations were normal except for one episode of elevated urinary dopamine and noradrenaline levels. Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) showed a large solid tumor with calcifications and a slightly lobular edge in the right adrenal gland. We performed open tumor excision and diagnosed it as adrenal ganglioneuroma. Adrenal GN is a rare benign tumor, and its hormonal activity and imaging characteristics are occasionally very similar to those of other adrenal tumors. Therefore, it needs careful evaluation by endocrine examinations and multiple imaging procedures to rule out other types of tumors.
Topics: Adrenal Gland Neoplasms; Adult; Biomarkers, Tumor; Dopamine; Ganglioneuroma; Humans; Incidental Findings; Magnetic Resonance Imaging; Male; Norepinephrine; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 22975550
DOI: 10.2169/internalmedicine.51.7726 -
Medicine Apr 2019Ganglioneuromas are benign neoplasm of neuroblastic origin which arise from central or peripheral parts of the autonomic nervous system. They are normally found at... (Review)
Review
RATIONALE
Ganglioneuromas are benign neoplasm of neuroblastic origin which arise from central or peripheral parts of the autonomic nervous system. They are normally found at posterior mediastinum, retroperitoneum, and the adrenal gland but ganglioneuromas are rarely found in the cervical region.
PATIENT CONCERNS
A 12-year-old boy was admitted with a left-lateral neck mass slow growing over a 7-days duration. The tumor was painless and was not associated with any systemic or compression-related symptoms. No symptoms of Horner's syndrome, including ptosis, myosis, ipsilateral facial anhidrosis, and flushing, were observed. Laboratory routine tests were within normal limits, and magnetic resonance imaging demonstrated a solid and well-circumscribed mass in the carotid space.
DIAGNOSIS
Due to the patient's symptoms, laboratory test results together with radiographic investigation findings, the 12 years old boy was diagnosed with cervical ganglioneuroma combined with tetralogy of Fallot.
INTERVENTIONS
Surgical excision.
OUTCOMES
The postoperative period was uneventful with the exception of Horner's syndrome on the left side in short period, and it was finally resolved after 8 months recovery. The patient is now in stable condition after operation, with improvement in symptoms during follow-up recovery.
LESSONS
Ganglioneuromas should be accounted as the differential diagnosis of pediatric soft tissue tumors of the head and neck. The diagnosis for ganglioneuromas in cervical region can only be ascertained with postoperative pathologic examination, and excision is considered as the only effective treatment modality known so far which may cause Horner's syndrome at times. However, patients have a favorable prognosis without recurrence overall.
Topics: Cervical Vertebrae; Child; Diagnosis, Differential; Ganglioneuroma; Head and Neck Neoplasms; Humans; Male
PubMed: 30985715
DOI: 10.1097/MD.0000000000015203 -
Cirugia Y Cirujanos 2022Ganglioneuromas are slow growing tumours arising from tissues of the neural crest, mainly in autonomic nervous system. They are frequently localized in the posterior... (Review)
Review
Ganglioneuromas are slow growing tumours arising from tissues of the neural crest, mainly in autonomic nervous system. They are frequently localized in the posterior mediastinum being the intraspinal involvement very uncommon. We present the case of a female patient with bilateral cervical ganglioneuroma, together with a review of the cases published to date, emphasizing in the main characteristics of these tumours and including them as part of neurofibromatosis type 1 spectrum.
Topics: Cervical Vertebrae; Female; Ganglioneuroma; Humans; Neck; Neurofibromatosis 1
PubMed: 35944427
DOI: 10.24875/CIRU.20000991 -
Journal of the American Veterinary... Jun 2017
Topics: Adrenal Gland Neoplasms; Animals; Animals, Newborn; Diagnosis, Differential; Dog Diseases; Dogs; Fatal Outcome; Female; Ganglioneuroma; Neoplasms, Multiple Primary; Neurofibromatoses; Skin Neoplasms
PubMed: 28569633
DOI: 10.2460/javma.250.12.1391 -
Thorax Jul 1971Thirty-two mediastinal neural tumours were seen in the East Anglian Regional Thoracic Surgical Unit at Cambridge between October 1952 and July 1970. The descending order... (Review)
Review
Thirty-two mediastinal neural tumours were seen in the East Anglian Regional Thoracic Surgical Unit at Cambridge between October 1952 and July 1970. The descending order of frequency was neurofibroma, ganglioneuroma, neurilemmoma, neurofibrosarcoma, and neuroblastoma. The literature relating to these tumours is reviewed and the pathological and clinical complications encountered in this series and in the literature are described.
Topics: Adolescent; Adult; Age Factors; Child; Child, Preschool; Deglutition Disorders; Female; Ganglioneuroma; Horner Syndrome; Humans; Hypertension; Infant; Infant, Newborn; Lung Diseases; Lung Neoplasms; Male; Mediastinal Neoplasms; Mediastinum; Meningocele; Middle Aged; Neoplasm Regression, Spontaneous; Neoplasms, Nerve Tissue; Neurilemmoma; Neuroblastoma; Neurofibroma; Neurofibromatosis 1; Neurologic Manifestations; Osteoarthropathy, Secondary Hypertrophic; Pain; Paraganglioma, Extra-Adrenal; Pheochromocytoma; Sex Factors; Vitamin B 12
PubMed: 4327710
DOI: 10.1136/thx.26.4.392 -
Asian Journal of Surgery Nov 2023
Topics: Humans; Ganglioneuroma; Adrenal Gland Neoplasms; Tomography, X-Ray Computed
PubMed: 37474382
DOI: 10.1016/j.asjsur.2023.07.048 -
Medicine Nov 2022Adrenal mixed corticomedullary tumors (MCMTs) are single tumor masses composed of an intimately admixed population of both adrenal cortical cells and medullary...
RATIONALE
Adrenal mixed corticomedullary tumors (MCMTs) are single tumor masses composed of an intimately admixed population of both adrenal cortical cells and medullary components. Most medullary tumor components are pheochromocytomas; however, MCMTs composed of ganglioneuroma and adrenal cortical adenoma are extremely rare. The current case is a rare case of adrenal MCMT composed of ganglioneuroma and adrenal cortical adenoma with primary aldosteronism.
PATIENT CONCERNS
A 49-year-old male was admitted because of hypokalemia and an adrenal mass. He was diagnosed with hypertension in his 20s and was taking blood pressure medications.
DIAGNOSIS
Plasma aldosterone concentration 376.5 pg/dL (normal 37.8~233.0 pg/mL) and potassium 2.8 mmol/L (normal 3.4~4.9 mmol/L) were detected. The aldosterone-to-renin ratio [the ratio of plasma aldosterone concentration (ng/dL) to PRA (ng/mL/hour)] was 38. The saline loading test showed that serum aldosterone (49.4 ng/dL) was not suppressed, compared with the basal level (28.4 ng/dL). The adrenal venous sampling test showed that the aldosterone level markedly increased to 1521.2 pg/mL. Abdominal computed tomography revealed an enlarged relatively well-circumscribed multinodular mass (35 × 13 × 30 mm) in the right adrenal gland.
INTERVENTIONS
Laparoscopic right adrenalectomy was performed under the clinical diagnosis of a functioning adrenal cortical adenoma.
OUTCOMES
After laparoscopic right adrenalectomy, the serum aldosterone and renin levels returned to normal. The patient maintained a normal aldosterone level without recurrence for 16 months.
LESSONS
Adrenal MCMTs of the ganglioneuroma and cortical adenomas in the ipsilateral adrenal gland are extremely rare. Adrenal MCMTs exhibit benign clinical behavior, with no metastasis or death due to the tumor. With the development of diagnostic imaging technology, it is possible to identify mixed tumors. However, surgical resection of adrenal gland is a common treatment and a final diagnosis should be made based on the pathological results after surgery. Because this is to rule out the occurrence of rare malignant tumors and confirm the pattern of mixed tumors.
Topics: Male; Humans; Middle Aged; Adrenocortical Adenoma; Ganglioneuroma; Aldosterone; Renin; Adrenal Glands; Adrenal Gland Neoplasms; Adenoma, Pleomorphic
PubMed: 36451378
DOI: 10.1097/MD.0000000000031403 -
Diagnostic Pathology Feb 2022SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily A, member 4-deficient thoracic sarcoma (SMARCA4-DTS) is a rare disease that has...
BACKGROUND
SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily A, member 4-deficient thoracic sarcoma (SMARCA4-DTS) is a rare disease that has recently been described as an entity. It is characterized by an aggressive clinical course and specific genetic alterations. As an immunohistological feature, the tumors are deficient in SMARCA4 and SMARCA2 and express sex-determining region Y (SRY)-box 2 (SOX2). Occasionally, there are cases that are less frequent and difficult to distinguish from SMARCA4-deficient non-small cell lung carcinoma (SMARCA4-dNSCLC). Therefore, the 5th edition of the World Health Organization (WHO) classification describes thoracic SMARCA 4-deficient undifferentiated tumors (SMARCA4-UT). In contrast, Carney's triad is a syndrome that combines three rare soft tissue tumors: gastric leiomyosarcoma, pulmonary chondroma, and extra-adrenal paraganglioma. Protein kinase cAMP-dependent type I regulatory subunit alpha (PRKAR1A) has been proposed as the causative gene. Both diseases are valuable cases; moreover, there have been no previous reports of their coexistence.
CASE PRESENTATION
A 43-year-old man visited our hospital because of respiratory distress. Computed tomography revealed a large mass measuring 55 mm in the upper lobe of the right lung and front mediastinum, with metastases in the surrounding lymph nodes. Needle biopsy was performed for diagnosis, and histological examination of the samples revealed monotonous epithelioid-like cells with loose binding and sheet-form proliferation. The tumor cells had distinct nuclei with some rhabdoid-like cells. Immunohistochemical analysis revealed that the tumor cells were positive for AE1AE3, SOX2, CD34, and p53 and negative for SMARCA4 and SMARCA2. The patient died 6 months after admission, without any treatment. Autopsy revealed ganglioneuroma and enchondroma suggestive of an incomplete Carney complex.
CONCLUSION
SMARCA4-UT is a rare and recently established disease. While it is difficult to diagnose, it is necessary to distinguish undifferentiated carcinoma, large cell carcinoma, Ewing sarcoma, and epithelioid sarcoma when diagnosing tumors involving the mediastinum. Moreover, cases of SMARCA4-UT with ganglioneuroma and enchondroma are very rare. We discuss and report a case of SMARCA4-UT in which we also examined ARID1A and SLC7A11expression.
Topics: Adult; Amino Acid Transport System y+; Biomarkers, Tumor; Chondroma; DNA Helicases; DNA-Binding Proteins; Ganglioneuroma; Humans; Lung Neoplasms; Male; Nuclear Proteins; Thoracic Neoplasms; Transcription Factors
PubMed: 35151345
DOI: 10.1186/s13000-022-01205-8 -
Frontiers in Surgery 2021To investigate the clinical characteristics, diagnosis, differential diagnosis, therapy options, and outcomes of retroperitoneal ganglioneuroma. In this retrospective...
To investigate the clinical characteristics, diagnosis, differential diagnosis, therapy options, and outcomes of retroperitoneal ganglioneuroma. In this retrospective study, we collected and analyzed the clinical data of 32 patients diagnosed with retroperitoneal ganglioneuroma and admitted to Peking Union Medical College Hospital from October 2012 to August 2019. Among our 32 cases with retroperitoneal ganglioneuroma, the male-to-female ratio was 1:3 and the mean age was 35. Only 25% of the cases presented with abdominal pain while more than 65% had no specific symptoms. The masses could be found through physical examination in only five patients. Most of the tumors are located near the renal area. They were usually single and displayed an embedded growth pattern with diameters <10 cm, clear borders, and soft texture. For radiological imaging, the majority of tumors demonstrated soft tissue density with mild-to-moderate enhancement on CT imaging and showed hypoecho with moderate blood flow signals in ultrasound. No significantly abnormal laboratory examinations were found in most patients. Of all the 32 patients, 2 chose surveillance after biopsy due to difficulties in operation, while others chose surgical resection. The mean follow-up time was 15.8 months among 26 patients. The tumor remained stable in the surveillance cases. Residual tumors were found in four cases receiving operations with no progress and discomfort. No recurrence was seen in all patients. The retroperitoneal ganglioneuroma is a benign tumor without specific clinical manifestations or significant laboratory findings. Typically, it is shown as low density with a clear border and an embedded growth pattern in radiological imaging. The overall prognosis is good. Surgery is an effective approach with possible severe complications. Incomplete resection or surveillance can be considered for some cases where complete resection is difficult to achieve.
PubMed: 34095202
DOI: 10.3389/fsurg.2021.642451 -
International Journal of Surgery Case... Oct 2023Retroperitoneal ganglioneuroma is an infrequent, benign tumor originating from the sympathetic nervous system. The diagnosis predominantly relies on histological...
INTRODUCTION AND IMPORTANCE
Retroperitoneal ganglioneuroma is an infrequent, benign tumor originating from the sympathetic nervous system. The diagnosis predominantly relies on histological assessment, often as an incidental discovery. Surgical removal stands as the primary treatment modality, and the overall prognosis tends to be favorable.
CASE PRESENTATION
Objective: In order to comprehensively examine the diagnostic, therapeutic, and progressive aspects of retroperitoneal ganglioneuroma, this study aims to analyze five cases of retroperitoneal ganglioneuroma that were surgically managed between 1993 and 2013.
PATIENTS AND METHODS
We present a series of five cases involving retroperitoneal ganglioneuromas that underwent surgical intervention in the urology department of Charles Nicolle Teaching Hospital of Tunis between the years 1993 and 2013. The study cohort comprised three females and two males, with an average age of 36.4 years (ranging from 10 to 88 years). Among these cases, four instances showcased incidental tumor detection, while one case manifested with cruralgia due to nerve compression. Complete tumor excision was achieved in all cases, with a single instance necessitating concurrent nephrectomy. Accurate diagnosis was ascertained through meticulous histological examination.
CLINICAL DISCUSSION
Results: The case series encompassed three women and two men, with an average age of 36.4 years (ranging from 10 to 88 years). The majority of cases (four out of five) unveiled tumors incidentally, whereas one case presented with cruralgia. Surgical removal resulted in complete resection of all tumors, although nephrectomy was necessary in one instance. Postoperative complications were minimal, and effective anticoagulant treatment addressed one thrombotic event. While recurrence was noted in a solitary case, subsequent imaging confirmed its stability. The average follow-up duration extended to 23.2 months (ranging from 6 to 72 months).
CONCLUSION
Retroperitoneal ganglioneuroma, being a rare and benign neoplasm, mandates meticulous pathological assessment and precise imaging for precise localization. Unquestionably, complete surgical resection remains paramount to forestall recurrence and mitigate the likelihood of malignant transformation. Overall, the prognosis associated with retroperitoneal ganglioneuroma tends to be favorable.
PubMed: 37734125
DOI: 10.1016/j.ijscr.2023.108840