scholarly journals Functional Adrenocortical Adenoma in a Child with Beckwith–Wiedemann Syndrome

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Leen Jamel Doya ◽  
Naya Talal Hassan ◽  
Hanin Ahmed Mansour ◽  
Mohammad Ahmad Almahmod Alkhalil ◽  
Abdul Alrahman Almahmod Alkhalil ◽  
...  

Beckwith–Wiedemann syndrome (BWS) is a rare congenital condition characterized by complex overgrowth of different body parts. Children with Beckwith–Wiedemann syndrome, particularly those with hemihypertrophy, experience an increased risk of developing benign and malignant tumors. This case report presents an 18-month-old girl with features suggestive of Beckwith–Wiedemann syndrome who developed pubic hair, high levels of testosterone, and DHEAS with normal cortisol and progesterone levels. Computed tomography revealed a left adrenal mass. Histopathological examination of the resected mass showed an adrenocortical tumor. Her postoperative evaluation showed normal testosterone and DHEAS levels. Early diagnosis and detection of intra-abdominal neoplasms in infants with Beckwith–Wiedemann syndrome are essential to avoid serious clinical complications.

2019 ◽  
Vol 2019 (1) ◽  
Author(s):  
Eman Abdalla Ali Elnaw ◽  
Awad Rhmattalla Abdalla ◽  
Mohamed Ahmed Abdullah

Abstract Background We report a case of right adrenocortical adenoma in a girl with features suggestive of Beckwith Wiedemann syndrome to show the importance of tumor surveillance in patients with Beckwith Wiedemann syndrome. Case presentation A 4-years-old female with features suggestive of Beckwith-Wiedemann syndrome presented with 9 months history of virilization. Hormonal investigations results showed high levels of testosterone (2.3 ng/ml, normal values 0.1–0.4 ng/ml), and DHEAS (73 ng/ml normal values 1-6 ng/ml) with normal cortisol level. Computed tomography revealed a right adrenal mass. She underwent right adrenalectomy. Histopathological examination of the resected adrenal gland showed adrenocortical adenoma. Her postoperative evaluation showed a normal testosterone level. Conclusion Adrenocortical neoplasms though rare in children are well documented in Beckwith-Wiedemann syndrome patients. So tumor surveillance protocol should be employed, even in a resource-limited setting for early tumor detection and a better outcome.


2019 ◽  
Vol 3 (2) ◽  
pp. 130-136
Author(s):  
Arif Fadillah ◽  
Andi Friadi

Background : Malignant ovarian tumor are still the number one cause of death and the second most  of incidents for gynecological malignant tumors. The principles of management of ovarian cancer are the same as the principles of handling other malignant diseases, for the treatment of primary lesions operatively and the handling of potential sites of tumor metastases with chemotherapy. Histopathological examination is still considered a gold standard for diagnosis and definitive treatment of malignant ovarian tumors. If histopathologic results are obtained, then the patient will be planned to undergo postoperative chemotherapy. The mismatch between the preoperative and intraoperative clinical features, and the results of postoperative histopathological examination is a problem in managing cases of ovarian malignant tumors.Objective : Report a case of residive ovarian carcinoma with no appearance of malignancy marker in the previous postoperative histopathological examination.Method : Case reportCase : We report the case of a 45-year-old woman with a history of two previous laparotomy. The first operation was carried out in February 2014 on the indication of an extra ovarian cyst, resulting in the impression of a "Follicular Cysts" based on histopathological examination. The second operation was performed in March 2015, performed surgical staging tumors with an indication of malignant ovarian tumors with clinical metastases, but from histopathological examination it was found that "Cystadenoma Ovarii Muscinosum Multiloculare" and "no visible signs of malignancy", so the patient was not managed with postoperative chemotherapy. In April 2019 patients came with complaints of new mass growth, from CT-Scan and USG investigations there was a suspicious impression of a residive ovarian tumor with metastases into the omentum and massive ascites. On May 16, 2019 an optimal debulking was performed with the findings of residive mass, ascites, and mass metastases in the intra operative peritoneum. From the results of histopathological examination, it was found that "Muscinous Carcinoma with metastases to the umbilicus, peritoneum, and ascitic fluidKeywords: Malignant residive ovarian tumor, mucinous ovarian carcinoma


2020 ◽  
Vol 11 (5) ◽  
pp. 54-60
Author(s):  
Apurba Mandal ◽  
Shibram Chattopadhyay ◽  
Sushanta Mondal ◽  
Arunava Biswas

Background: Adnexal mass is a common presentation in today’s gynecological practice. The incidence of ovarian cancer is increasing day by day and diagnosis is often difficult to be made pre operatively with inadequate surgical exploration is a regular occurrence. Aims and Objectives: To assess and validate the importance of RMI-3 score as pre-operative diagnostic tool of differentiating benign from malignant adnexal mass for starting first line therapy of ovarian cancer and to find out the incidences of ovarian malignancy among study population. Material and Methods: The study was conducted in the Department of Gynecology and Obstetrics on (n=115) patients attending GOPD and indoor with adnexal mass fulfilling the inclusion and exclusion criteria using purposive sampling technique. All the selected cases underwent ultrasonography and serum CA- 125 level estimation necessary for calculating RMI score. A score of >200 was taken as suggestive of malignancy and confirmatory diagnosis was performed by histopathological examination obtained from staging laparotomy of adnexal mass. The individual scores were then correlated with final outcomes with statistical analyses. Results: The study revealed benign ovarian tumors are more under 50 years (78.46%) and patients with normal BMI are diagnosed with maximum of malignancy (n = 28). History of tubal ligation carried less risk of malignancy (p<0.0001). Histologically malignant tumors found mostly in 71.4% postmenopausal group whereas 94.1% benign pathology were present in perimenopausal group and there is no association found between parity and histopathology (p=0.058). Bilateral (p=0.013), multilocular (p=0.000) tumors with solid areas (p<0.0001) and thick papillary projections (p<0.0001) had statistically significant association with malignant lesions. RMI score (>200) had more efficacy than serum CA-125 level (>46) in differentiating malignant lesions from benign one in terms of specificity (96% vs 83.87%) and positive predictive value (95% vs 79.17%). Conclusions: RMI-3 score is a simple, reliable and effective tool in differentiating benign from malignant adnexal masses thereby help in quick referral and management of cases with increase chances of survival of the patients.


2019 ◽  
Vol 65 (3) ◽  
pp. 321-329
Author(s):  
David Zaridze ◽  
Anush Mukeriya

Smoking not only increases the risk of the development of malignant tumors (MT), but affects the disease prognosis, mortality and survivability of cancer patients. The link between the smoking of cancer patients and increased risk of death by all diseases and oncological causes has been established. Mortality increases with the growth of the smoking intensity, i.e. the number of cigarettes, smoked per day. Smoking is associated with the worst general and oncological survivability. The statistically trend-line between the smoking intensity and survivability was observed: each additional unit of cigarette consumption (pack/year) leads to the Overall Survival Reduction by 1% (p = 0.002). The link between smoking and the risk of developing second primary tumors has been confirmed. Smoking increases the likelihood of side effects of the antitumor therapy both drug therapy and radiation therapy and reduces the treatment efficacy. The smoking cessation leads to a significant improvement in the prognosis of a cancer patient. Scientific data on the negative effect of smoking on the prognosis of cancer patients have a major clinical importance. The treatment program for cancer patients should include science-based methods for the smoking cessation. The latter is fundamentally important, taking into account that the smoking frequency among cancer patients is much higher than in the population.


2021 ◽  
pp. 1-7
Author(s):  
Mohammad Al-Wiswasy ◽  
Mahmoud Al-Balas ◽  
Raith Al-Saffar ◽  
Hamzeh Al-Balas

INTRODUCTION: Primary breast sarcoma (PBS) was first described in 1887 as a rare heterogeneous neoplasm arising from the mesenchymal tissue of the mammary gland accounting for less than 5% of all body soft-tissue sarcomas and less than 0.1% of all malignant tumors of the breast. CASE PRESENTATION: A 31-year old lady presented with left breast mass which she felt four years before during which the mass increased in size from 1 to 6 cm in the largest diameter, diagnosed clinically as a benign fibroadenoma without any further cytological or histopathological confirmation. Histopathological examination of the excised breast mass reveals undifferentiated, primary stromal sarcoma of the breast (PSSB), which was followed by mastectomy three weeks later with reconstructive breast surgery with a total duration of follow-up of 3 years thereafter. This is the first case of PSSB reported in Jordan. DISCUSSION/CONCLUSION: PSSB is the generic term given to malignant breast tumors thought to arise from the specialized mesenchymal stroma of the breast but lacking an epithelial component with a phylloides pattern. PSSB is difficult to diagnose preoperatively due to its rarity and inadequate imaging methods to establish an exact diagnosis. The histology of the patient mass may be the leading factor for the management of these tumors. Even in very young patients, a progressively growing breast mass should alert the clinician to investigate for malignancy and verify the results by biopsy. Surgery with adequate resection margins represents the only potentially curative modality with prognostic significance. Adjuvant chemotherapy and radiotherapy are not very beneficial. The prognosis is dismal for patients with lymph node involvement and the size of the tumor has a lesser bearing on the outcome.


2021 ◽  
Vol 29 (1) ◽  
pp. 230949902199399
Author(s):  
Taketsugu Fujibuchi ◽  
Hiroshi Imai ◽  
Joji Miyawaki ◽  
Teruki Kidani ◽  
Hiroshi Kiyomatsu ◽  
...  

Purpose: The spectrum of diagnoses and clinical features of hand tumors differ from those of tumors in other body parts. However, only a few reports have comprehensively referenced the diagnosis and clinical features of hand tumors. This study aimed to elucidate the diagnostic distribution and the clinical features of hand tumors undergone surgery in our institute. Patients and methods: A total of 235 lesions in 186 patients diagnosed with hand tumors between 1978 and 2020 were reviewed. Age at surgery, gender, chief complaint, tumor location, and pathological diagnosis were analyzed. Results: There were 121 benign bone tumors, 98 benign soft tissue tumors, and 16 malignant tumors. Chondroma and tenosynovial giant cell tumor were common benign bone and soft tissue tumors at the proximal phalanx of the ring finger and the palm, respectively. Meanwhile, chondrosarcoma and synovial sarcoma were common malignant tumors at the dorsal part of the hand. Local pain and painless mass were the chief complaints in patients with benign bone and soft tissue tumors, respectively. Most patients with malignant tumors were referred after unplanned resection. When patients were classified into two categories by tumor size according to maximal diameter, tumors larger than 19 mm had a significantly higher risk of malignant ( p = 0.031) despite being smaller than other tumors in different body parts. Conclusion: When a tumor malignancy is suspected, the patient should be referred to a specialist to avoid unplanned resection or delayed diagnosis due to misdiagnosis. Knowing the distribution and clinical features should help in diagnosing hand tumors.


2019 ◽  
Vol 47 (03) ◽  
pp. 192-195
Author(s):  
Julia Schoiswohl ◽  
Bianca Lambacher ◽  
Andrea Klang ◽  
Reinhild Krametter-Frötscher

AbstractFibrosarcomas are malignant tumors of the connective tissue, which are characterized by proliferation of fibroblasts. Most of these tumors are localized subcutaneously and cause different symptoms depending on their location. The case report describes a dermal tumor on the outside of the pinna in a 7-year-old female Cashmere goat. The tumor was surgically removed and histopathological examination revealed a low grade fibrosarcoma. Although tumors are rare in ruminants because of the animals’ short lifespan (age of slaughtering in most cases < 24 months) in modern production processes, they should be considered as a differential diagnosis.


2017 ◽  
Vol 41 (S1) ◽  
pp. S406-S406
Author(s):  
M. Bhadar ◽  
S. Asghar ◽  
Z. Mukhtar

IntroductionSomatic symptoms in depression are not uncommon. There is increased sicidality, poor prognosis, and increased risk of relapse. Neurological and l muscloskeletal symptoms can be explained on basis of increased muscular tension in the body.MethodsAn internet search was made using key words muscular tension, SSRIs depression, anxiety, somatic symptoms.ResultsNinety-eight percent patients reported at least one of somatic symptoms. Forty-five percent reported six somatic symptom. GIT symptoms in 67% patients. Fatigue in 78% % of patients. Weakness in body parts 45% and headache in 43% to 65%. Chest pain more common in male. Patient over 40 showed pain in limbs or joints. Number of symptoms was directly related to severity of depression.DiscussionIncreased muscular tension is one component of mix anxiety and depression. Main nerves and their branches pass in between muscular bellies. When there is increased muscular tone, it puts extra compression on major nerves and their branches. This will cause dysesthesia in body parts. Stretching of muscles produce pain due to hypoxia. This produces headache, strain neck and backache pain in chest. Fatigue is result of over consumption of energy due to hypertonia. One of their side effect is of SSRIs is muscular hypertonia which will not be very helpful for these symptoms. Drugs like tricyclics, SNRI can have better results when used alone, gabapentine, pregabline tinazidine used as adjunct can alleviate symptoms. Non-pharmacological treatment includes massage, relaxation technique, and warm water therapy.ConclusionSomatic symptoms of mix anxiety and depression should be important consideration in its treatment.Disclosure of interestThe authors have not supplied their declaration of competing interest.


2021 ◽  
pp. 69-71
Author(s):  
Rifat Qureishi ◽  
M.H. Usmani ◽  
U.R. Singh ◽  
P.C. Kol

Background: Fine needle aspiration cytology (FNAC) has been used for diagnosis of salivary gland lesions for many years. Various studies in the existing literature have shown a wide range of sensitivity and diagnostic accuracy of cytologic diagnosis. FNAC is a safe, simple, cost effective, 1-4 accurate and minimal invasive procedure for the evaluation of salivary gland lesions. FNAC is not only useful in planning denitive preoperative 2,5-6 diagnosis but also can prevent unnecessary surgical intervention. Salivary gland swelling occur more commonly in 3rd decade of life with equal sex incidence. Parotid is one of the most commonly involved glands in the head & neck region swellings. FNAC appears to be highly sensitive for benign tumours and highly specic for malignant tumors and it should be the rst line of investigation in evaluating the salivary gland pathologies. 7 Early diagnosis and appropriate management carries good prognosis. Methods: Patients with suspected salivary gland enlargements, referred for FNAC, were included in this study. FNAC was performed by using the standard procedure. Cytologic diagnosis was compared with histopathologic diagnosis wherever it was available. Results: In the present study conducted in the department of pathology, Shyam Shah Medical College Rewa MP, over a period of 5 years, 152 Patients with suspected salivary gland enlargements were retrospectively appraised. The benign lesions of salivary gland were 133 (87.5%), out of which Pleomorphic adenoma was diagnosed in 75 (56.39%) cases, clinical correlation was found in 58 (77.33%) cases. 29 cases were subsequently correlated with histopathological examination 26 correctly correlated and the diagnostic accuracy of FNAC was found to be 89.65%. Inammatory lesions were proved correct in 08 out of 10 cases after histopathology. Therefore, accuracy of FNAC was 80.0%. Malignant lesions of salivary gland were found in 19 cases, out of which 15 (78.94%) cases correlated with clinical diagnosis. 13 cases were subjected to histopathology 10 correctly correlated and the diagnostic accuracy of FNAC was found to be 76.92%. Overall diagnostic accuracy of FNAC was 84.61%. Conclusion:In conclusions it can be established that FNAC is an efcient and accurate procedure with high sensitivity index, and its usefulness is enhanced due to it being a relatively easy procedure which can be carried out even on outdoor patients.


2021 ◽  
Vol 75 ◽  
pp. 292-296
Author(s):  
Patrycja Sosnowska-Sienkiewicz ◽  
Piotr Nogal ◽  
Dawid Gawron ◽  
Korneliusz Wójcik ◽  
Danuta Januszkiewicz-Lewandowska ◽  
...  

Background: The aim of this study was to evaluate the incidence and histological type of lesions affecting the ovaries and to analyze employed methods of invasive treatment. Materials&Methods: Medical records of patients who were treated surgically for ovarian tumors in the years 2015 -2019 were reviewed. The study group was comprised of 31 female patients. Results: During 5 years time, there were 31 girls in the age from 3 months to 17 years hospitalized in the department. The mean age was 11 years. Histopathological examination was performed in all of these cases. 12 patients were diagnosed with malignant lesion, 19 with benign lesion. The most commonly diagnosed malignant tumors were a dysgerminoma and a mixed germ cell tumor. In the group of benign lesions, the most frequent tumor type was mature teratoma. The first occurring symptom was abdominal pain. Some of the lesions were diagnosed accidentally during ultrasonography. The diagnostics was expanded depending on the size of the tumor, staging and clinical condition of the patient. All the patients were treated surgically, 16 of them underwent laparoscopic surgery. Torsion of the ovary or oviduct was observed in 3 cases. Chemotherapy was introduced in 8 cases as complementary treatment. Conclusions: The most commonly diagnosed tumor was mature teratoma. Ultrasonography is the most frequent method of the ovaries’ examination. Ovarian lesions are characterized by non-specific clinical symptoms, which is associated with prevalent incidental detection during ultrasonography.


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