Malignant peripheral nerve sheath tumor of the cervical vagus nerve in a neurofibromatosis type 1 patient - An unusual presentation

Malignant peripheral nerve sheath tumors (MPNST’S) of the head and neck comprise 2% to 6% of head and neck sarcomas. These tumors may arise as sporadic variants or in patients with neurofibromatosis (NF). Development of these MPNST’s is one of the serious complications of neurofibromatosis type 1(NF1). To our knowledge there are only two reported cases of MPNST’s arising in the cervical vagal nerve, occurring in NF1 patients. We present here an NF1 patient who developed an MPNST of the cervical vagus nerve and presented only with a cervical swelling and hoarseness

Adenoid Cystic Carcinoma of External Auditory Canal

Adenoid cystic carcinoma is extremely rare tumour that accounts for approximately 5% of primary malignancy of external auditory canal. These tumours are related with a high risk of recurrences and significant morbidities from surgical management and adjuvant radiotherapy. Despite the aggressive management for these tumours, many patients succumb to distant metastasis, making overall prognosis of these tumours poor. Although ACC of EAC has been reported in 5th decade, but its occurrence in young patient is very rare. We report a rare case of ACC in a young 22 years old female, who presented with ear canal mass and ear pain. Biopsy suggested mass to be ACC. Patient underwent wide local excision followed by adjuvant radiotherapy

Malignant Melanoma of Nose and Paranasal Sinuses: 2 Case Reports

Malignant melanoma is one of the rare and highly aggressive diseases of the sinonasal cavity. High index of suspicion is required for diagnosis as the patient usually presents with non specific signs and symptoms. In the natural course of the disease, higher rate of loco regional recurrences and distant metastasis are seen making the overall prognosis of disease very poor. In reviewing the various treatment modalities used in the past, surgical resection of the tumour with postoperative radiotherapy is preferred one. Advances in surgery, radiotherapy and chemotherapy don’t have any impact on improved survival, which remains poor in this disease. We report two cases of malignant melanoma, which were treated at our institute

Aggressive Fibromatosis in Neck.

Aggressive fibromatosis (AF) is a locally aggressive infiltrative low-grade benign tumor that accounts for approximately less than 3% of all soft tissue tumors. In the head and neck region this tumor tends to be more aggressive and associated with significant morbidity. Aggressive surgery is a viable management option and may be successfully used as a single modality treatment, or in combination with radiotherapy. We report a rare case of AF in a 38 year old female, who presented with a painless mass over the left supraclavicular fossa, extending inferiorly into the thoracic inlet, which was excised successfully in toto with the help of cardiothoracic vascular surgeon (CTVS)

Vulval lymphangiectasia: 2 case reports

Vulval lymphangiectasia is a rare cutaneous condition of lymphatic channels in deep dermal and subcutaneous layers. It can be congenital condition or might develop secondary to tuberculosis, surgery, radiotherapy, crohn’s disease etc. Very few cases have been reported in the literature so far. Diagnosis is mainly clinical aided with histopathology. Various treatment modalities are available for this condition. Surgical treatment is the most commonly used method to treat vulvar lymphangiectasia and has offered promising results. In this report, we present 2 cases of vulval lymphangiectasia- one congenital case and other acquired after tuberculosis

Nodular Fasciitis of Neck in Childhood.

Nodular fasciitis, is a benign, pseudo sarcomatous proliferative lesion of the soft tissue, which is frequently misinterpreted as sarcoma, both clinically and microscopically. It is a reactive lesion composed of fibroblasts/myofibroblasts and most commonly found in extremities and trunk. NF has been described in the head and neck region in 10-20% of cases. Many pathologists do not consider NF in the differential diagnosis of soft tissue masses arising in the Head neck region. NF that occurs in otherwise healthy individuals usually presents with a history of rapid growth, and is commonly found in the upper extremities and on the chest and trunk. The importance of otolaryngologists being aware of the existence of this entity in this area of the body is stressed. It has a confirmed perfectly benign clinical course, and simple excision, as tissue-sparing as possible, is the treatment of choice. A case of NF over the neck in a 05-year-old female not associated with trauma who presented with a localized mass over her left neck is presented

ATTENUATION OF IRON OVERLOAD AND EFFECT OF ANTIOXIDANTS SUPPLEMENTATION ON OXIDATIVE STRESS IN HOMOZYGOUS β-THALASSEMIA

It has been projected that ferritin and iron yoke in homozygous thalassemic children is coupled with the enhanced free radical formation and blemished in antioxidative defense coordination. Aim: The purpose of the current study was to analyze the consequence of serum iron, erythrocyte catalase, and erythrocyte superoxide dismutase (ESOD) in patients with beta-thalassemia major. Method: 60 beta-thalassemia major patients were studied before and after supplementation of A – Z antioxidants for 20 weeks, and status were compared with 60 age and sex-matched healthy normal.    Serum Iron estimation was carried out by Ramsay’s Dipyridyl Method. Estimation of erythrocyte superoxide dismutase was done by Kajari Das Method. The levels of Catalase concentration in erythrocytes were analyzed by the Goth method.  All the objectives mentioned above were run by using a UV visible Spectrophotometer (Systronix). Results: A marked enhancement was seen in the intensity of serum iron, and superoxide dismutase (p<0.001) with parallel decline was observed in the level of erythrocyte catalase (p<0.001) in homozygous thalassemia patients when compared with healthy subjects. After 20 weeks of regular supplementation of antioxidants A-Z syrup, which consists of multimineral multivitamins, the concentration of catalase was increased whereas iron and ESOD (p<0.001) were reduced significantly when compared with normal and baselines thalassemic patients. Conclusion: Due to multiple blood transfusions, beta-thalassemia major children are at advanced risk due to secondary iron surplus and intense oxidative stress. Such kind of circumstances may be handled with supplementation of antioxidants A-Z syrup with their regular treatment. &nbsp

ATTENUATION OF IRON OVERLOAD AND EFFECT OF ANTIOXIDANTS SUPPLEMENTATION ON OXIDATIVE STRESS IN HOMOZYGOUS β-THALASSEMIA

It has been projected that ferritin and iron yoke in homozygous thalassemic children is coupled with the enhanced free radical formation and blemished in antioxidative defense coordination. Aim: The purpose of the current study was to analyze the consequence of serum iron, erythrocyte catalase, and erythrocyte superoxide dismutase (ESOD) in patients with beta-thalassemia major. Method: 60 beta-thalassemia major patients were studied before and after supplementation of A – Z antioxidants for 20 weeks, and status were compared with 60 age and sex-matched healthy normal.    Serum Iron estimation was carried out by Ramsay’s Dipyridyl Method. Estimation of erythrocyte superoxide dismutase was done by Kajari Das Method. The levels of Catalase concentration in erythrocytes were analyzed by the Goth method.  All the objectives mentioned above were run by using a UV visible Spectrophotometer (Systronix). Results: A marked enhancement was seen in the intensity of serum iron, and superoxide dismutase (p<0.001) with parallel decline was observed in the level of erythrocyte catalase (p<0.001) in homozygous thalassemia patients when compared with healthy subjects. After 20 weeks of regular supplementation of antioxidants A-Z syrup, which consists of multimineral multivitamins, the concentration of catalase was increased whereas iron and ESOD (p<0.001) were reduced significantly when compared with normal and baselines thalassemic patients. Conclusion: Due to multiple blood transfusions, beta-thalassemia major children are at advanced risk due to secondary iron surplus and intense oxidative stress. Such kind of circumstances may be handled with supplementation of antioxidants A-Z syrup with their regular treatment. &nbsp