Askin's Tumor in the Chest Wall-a Rare Clinical Entity and Review of Literature.

Askin tumors are the rare malignancy of neuroectodermal origin of the thoracic wall. Its prevalence is more in younger age group who present with vague symptoms leading to delayed diagnosis. We hereby present a case report of complex management of large chest wall tumor in a young boy and review the literature of this entity.

Management of a Rare Challenging Case of Duodenal Ampullary Lipoma.

Duodenal lipoma is a very rare entity with limited case reports present in literature. But duodenal ampullary lipomas are even more rare in nature. Owing to the recent advances in endoscopy and modern imaging techniques, more cases are being diagnosed and treated. However, challenge lies in performing a less invasive and least morbid procedures to treat them surgically in such complex location of tumour. To study the diagnosis and treatment of duodenal ampullary lipoma in a young male patient and challenges faced during surgical management. A 15-year-old young boy presented to us with complaints of intermittent upper gastrointestinal bleed and jaundice since last 2 months. At admission, his serum haemoglobin was 3 g% for which he was transfused 3 units of packed blood cells for optimization. On further evaluation, CT scan abdomen revealed 71 × 49 mm large heterogeneous mass in D3 segment of duodenum causing duodeno-duodenal intussusception involving D1 and D2 segment along with ampullary region with mass being the lead point. There was compression of CBD with dilatation measuring 11 mm in diameter and mild IHBR dilatation. UGIE revealed narrowing at D1-D2 junction due to polypoidal lesion with overlying smooth mucosa with no active bleeding point identified. His blood parameters were normal except for low haemoglobin (before blood transfusion) and total serum bilirubin of 2.3 mg/dl.He was optimized for surgery and underwent exploratory laparotomy with duodenotomy at D2 with mass excision of 7 × 5 cm sessile polyp with base over ampulla followed by plastic repair of sphincter of Oddi (pancreas preserving procedure). He was started on oral liquids on POD 3 and was discharged on normal diet by POD 7 with an uneventful recovery. Result of histopathological report revealed, on gross cut section, the presence of mass of 7 × 5 × 3 cm size with smooth mucosa and fibrofatty tissue. On microscopic examination, diagnosis of submucosal lipomatous polyp was made. Our case report indicated duodenal ampullary lipoma is extremely rare entity. The symptoms are nonspecific and CT scan abdomen is the first investigation of choice for diagnosis. The treatment depends on the patient's condition as well as the size and position of the tumour. In our case report, the tumour base was exactly at the level of ampulla where we performed complex procedure of local excision of mass with sphincteroplasty avoiding major Whipple procedure for such benign condition. It provided rapid postoperative recovery to the patient.

Intraoral Basal Cell Carcinoma of Soft Palate: A Diagnostic Dilemma and Review of Literature.

Basal cell carcinoma is the most common cancer worldwide. Most of basal cell carcinoma can be detected in the early stages and are generally well controlled with local resection. Despite the high incidence of BCC, intramucosal BCC is a very rare clinical entity. We hereby present a rare case report of pigmented BCC on soft palate and review the literature of this entity.

Maxillary Fibromatosis: A Rare Clinical Report and Its Review of Literature.

Fibromatosis is a rare locally aggressive benign tumour which arises from the musculoaponeurotic structures throughout the body. In the oral and maxillofacial region, It has been described under a variety of synonyms, including 'extra articular desmoids', 'desmoids tumours', 'grade-1 fibrosarcomas','non metastasizing fibrosarcoma'and 'aggressive fibromatosis'. The pecularity of this entity in paranasal sinuses is that it is rare in this location and are locally aggressive with higher rates of recurrence in a relatively restricted area.The purpose of this study is to present a rare case report and reviewing the literature of this entity.

A Rare Case of Angiosarcoma of Tibia in a Young Age-a Case Report.

Angiosarcoma of bone is very rare entity, accounting for less than 1% of all primary bone sarcomas with associated poor prognosis. We hereby present a case report of angiosarcoma of tibia in a young boy and reviewed its literature and management of the disease. A 21-year young male presented to us with complaints of pain and swelling over left lower leg since last 3 months. On evaluation, MRI lower leg revealed multiloculated lesion of size 32×36×52 mm showing multiple fluid-fluid levels which was hyperintense on T2 images in left distal tibial metaphysis showing endosteal scalloping with cortical destruction along lateral border and abuts the physis. Image-guided biopsy was done. PET CT revealed no evidence of metastasis. Histopathology report revealed tumor involving bone and soft tissue comprising of plump ovoid to epitheloid/spindled cells in nests and focal fascicles with marked nuclear atypia, prominent nucleoli and mitotic figures with vascular pattern with thick walled vessels, many haemosiderophages. Differentials of telangiectatic osteosarcoma/adamantioma was made. On further IHC studies, tumor cells were found positive for SATB2, CD31, ERG while negative for CK, P40, DESMIN, MYOGENIN, TLE-1, S-100. Diagnosis of angiosarcoma of tibia was confirmed. Decision to give neo-adjuvant chemotherapy with doxorubin/Ifosfamide-based regimen was made followed by later with limb salvage surgery. He received 5 cycles of chemotherapy 3 weekly regimen which he tolerated well. Repeat MRI leg showed partial reduction in tumor size but there was presence of pathological fracture seen with some extraosseous component of tumor. Limb salvage surgery was not possible and he underwent below knee amputation. His postoperative recovery was eventful. Final histopathology confirmed diagnosis of angiosarcoma tibia. Case was discussed in multidisciplinary tumor board and he was started on alternate chemotherapy with Injection docetaxel and gemcitabine-based regimen. He had received 3 cycles of this regimen till now and is disease free from last 3 months. Angiosarcoma of bone is a very rare entity; the literature is limited regarding treatment and outcome of patients with this tumor. Most information is currently available from case reports, and treatments are based on guidelines for other types of primary bone sarcomas. We hereby present a case report of angiosarcoma of tibia in a young boy and reviewed its literature, diagnostic dilemmas associated with it and management of the disease after discussing in multidisciplinary board. Role of chemotherapy, surgery, and their sequencing is not well defined. Prospective trials are required to manage this rare entity.

Long-Term Complete Response with Single-Agent Capecitabine in Her-2-neu-Positive Metastatic Breast Cancer-A Rare Case Report.

The standard treatment approach for metastatic breast cancer with HR- and Her-2-neu + disease is trastuzumab with systemic therapy. But in patients having severe cardiac dysfunction, trastuzumab is avoided. Various combination regimens are available in that setting, but no study has shown better efficacy of capecitabine monotherapy in this setting. We hereby present a case report of using capecitabine monotherapy in first-line setting, and the patient had complete resolution of lung metastasis from the last 2 years. A 64-year-old postmenopausal lady with a known case of breast carcinoma in the left side diagnosed in the year 2016 with hormone receptor-positive, Her2-negative disease completed chemoradiation and on is aromatase inhibitor from the last 5 years. She complained of breathlessness and fatigue lasting for 1 month in July 2021. On evaluation, chest CT scan revealed multiple bilateral lung metastases along with 3×3 cm right-sided breast lump with no metastasis elsewhere in the body. Core needle biopsy of breast lump and CT-guided left lung nodule biopsy were performed which revealed infiltrating carcinoma with immunohistochemical markers showing tumor cells positive for Her-2-neu with hormone receptor-negative disease. PET-CT scan was done which revealed FDG avidity in bilateral lung fields and right breast lump with no disease elsewhere. Her echocardiography showed ejection fraction of 40% owing to which injection trastuzumab was deferred and the plan to start tablet capecitabine 1000 mg twice BD days 1-14 cycles every 21 days was made. She showed improvement in symptoms with PET-CT scan revealing resolution of lung metastasis from the last 2 years. Trastuzumab in combination with pertuzumab and taxane is the standard of care for metastatic breast carcinoma with hormone receptor-negative and Her2-positive disease. But in patients who are elderly, frail with severe cardiac dysfunction, trastuzumab-based regimen is contraindicated. No study demonstrated efficacy of capecitabine monotherapy in this subset of disease. More prospective studies are required to identify patients who can benefit from capecitabine monotherapy in first-line setting in this subset of disease. Also capecitabine usage has several advantages mainly low cost and availability in oral form, and patients can be followed up on OPD basis.

Platysmal Myocutaneous Flap for Intra Oral Defects:Is It an Underutilized Option? Our Experience of 10 Cases.

Various methods of reconstruction are available for reconstructing oral cancer defects, but all of them have their inherent drawbacks. The superiorly based platysma myocutaneous flap is a common reconstruction option for intra-oral defects following oral cancer resections. We present our results of using platysmal flaps in reconstructing intraoral defects following oral cavity cancer resection in our 7 patients along with resection of three cases of premalignant conditions of oral cavity. All the patients were males of age ranging from 24 to 42 years and diagnosed as squamous cell carcinoma of oral cavity stage I and II were included in this study.Of 10 patients eight had no postoperative complications. One patient developed partial skin loss of neck donor site, which was managed conservatively. Other patient had complete flap loss which healed with secondary intention. We recommend this flap as incision is always away from the face, the scars are hidden beneath collars and the patients are very comfortable with good cosmetics and functional outcomes.\.

Use of Temporalis Muscle and Temporoparietal Fasciocutaneous Flap (TPPF) for Orbital Exentration Defects: Our Experience of 10 Cases.

Orbital defects represent difficulties in head and neck reconstruction owing to 3-dimensional complexity of the socket with aim to restore form and function. Recommended methods of reconstruction include mucosal and skin grafts free microvascular myofascial or fasciocutaneous flaps. However, most frequently, reconstruction of orbital defects calls for measures somewhere in between. The temporoparietal fascia flap (TPFF) fits well as it provides thin, pliable coverage of defects with dependable blood supply, tolerance for a large degree of rotation and minimal donor site morbidity. We hereby present our experience and results of 10 cases using TPPF and temporalis muscle post orbital exentration defects.Ten patients (7 male and 3 females) age ranging from 25 to 64 years underwent reconstruction of orbital exenteration defects using TPPF and temporalis muscle from June 2019 to June 2020 in our department. The primary disease was squamous cell carcinoma (SCC) of orbital structures (anterior compartment) in all 10 patients. All cases were clinically N0 and M0.All patients had successful transfer of TPFF grafts and temporalis muscle transfer without flap compromise. TPPF was used in all 10 patients while Temporalis muscle flap was used to fill orbital socket in our 9 patients. Temporoparietal fascial flap showed viable option for subtle orbital and malar contour defect. All patients had intact dura with residual orbital cavity after resection reconstructed with TPPF and temporalis muscle without any additional flap usage. No frontal paralysis or orbital fistula was seen but local recurrence occurred in one patient in follow up and managed with RT. The TPFF is one of the most reliable and versatile regional flaps in the head and neck for orbital reconstruction.

Unusual Presentation of Tonsillar Spindle Cell Neoplasm: A Case Report.

Spindle cell neoplasm of the tonsil are rare (Minami et al. in Am J Otolaryngol 29(2):123-125, 2008) and can be difficult to diagnose due to their non-specific clinical presentation and histological characteristics (Su et al. in J Chin Med Assoc 69(10):478-483, 2006). Differential diagnoses include lymphoma and squamous cell carcinoma (Hyams in Clin Otolaryngol Allied Sci 3(2):117-126, 1978). Oropharyngeal spindle cell neoplasms were more likely to occur in the tongue base and tonsil (58%) (Gerry et al. in Ann Otol Rhinol Laryngol 123(8):576-583, 2014). In this article, we report a case of tonsillar spindle cell neoplasm which is extremely rare.

Management of Giant Cell Tumor of the Hyoid bone- A rare Clinical Entity with Review of Literature.

BACKGROUND: Giant cell tumors (GCTs) are typically found in the long bones but can also occur in the head and neck region. GCT of the larynx is a rare entity with only 42 reported cases in the international literature. Furthermore, to the best of our knowledge this is the largest laryngeal GCT reported in the literature to date. GCT of the larynx can present with dysphonia, dyspnea, and/or dysphagia and should be considered in the differential diagnosis of a neck mass. PATIENT AND METHODS: A38-year-old man presented with complaints of mass in the neck with dysphonia and dysphagia. On clinical examination,there was huge spherical swelling extending from submental region till sternal notch with overlying normal skin. Computed tomography (CT) neck revealed a 20 × 15.7 cm mass centered on the hyoid bone, which was further diagnosed by histopathology as giant cell tumour on biopsy. The patient was counselled on treatment options and it was decided to proceed with a surgical approach. The patient consented to and successfully underwent en-bloc excision of mass with hyoid bone. Histopathology report revealed GCT of Hyoid bone.Currently the patient has no evidence of disease at 25 months follow-up, has an optimal voice, and is on regular follow -up with us. DISCUSSION AND CONCLUSION: GCTs of the larynx have a good prognosis and can be treated successfully through complete resection of the tumor, negating the need for adjunctive therapy such as radiation, chemotherapy or denosumab therapy.

"Flap of Hope: a Pectoralis Major Muscle Solution for Tracheal Resection Closure": Case Report.

Medullary carcinoma of the thyroid is a rare type of thyroid cancer that arises from the parafollicular cells or C-cells, which produce calcitonin. It accounts for approximately 5-10% of all thyroid cancers (Leboulleux et al. in Clin Endocrinol 61(3):299-310, 2004). The main treatment for medullary thyroid carcinoma is surgery, which involves the removal of the thyroid gland and any affected lymph nodes. In advanced cases where the cancer has spread to nearby structures such as the trachea (Gupta et al. in Indian J Surg Oncol 11(1):75-79, 2020), tracheal resection followed by reconstruction may be necessary to remove the cancer (Chernichenko et al. in Curr Opin Oncol 24(1):29-34, 2012) and restore proper breathing, closure of large tracheal defect can be done with pectoralis major myocutaneous flap (Salmerón-González et al. Plast Surg Nurs 38. 162-165, 2018). In this article, we report a case of recurrent medullary carcinoma thyroid with tracheal infiltration and tracheal resection was done, both of which is extremely rare. A 38-year-old male patient with a history of total thyroidectomy presented with recurrence was referred to our department, his previous biopsy and IHC revealed medullary carcinoma thyroid. Ga-68 DOTA PET CT scan was done which showed PET avid residual mass over right side, multiple bilateral cervical nodes, and tracheal infiltration (Fig. 1) then underwent a bronchoscopy showing involvement of the second, third, and fourth tracheal ring. Bilateral neck dissection with sleeve resection of trachea with overlying residual tumor was done and was sent for frozen which revealed positive margins and re-excision of margins was done, which lead to large defect (Fig. 2) which could not be closed primarily with a Montgomery T Tube. A de-epithelized pectoralis major myocutaneous flap used to close the tracheal defect followed by placing the Montgomery T Tube (Fig. 3).Post-operative period was uneventful. The final histopathology report showed R0 resection of tumor. T tube was removed after 4 weeks.

Latissimus Dorsi kiss Flap for Reconstruction of Large Chest Wall Defect-an Underutilized Option?

The utility of pedicled latissimus dorsi kiss flap for the reconstruction of chest wall defect is still an underutilized option. But the peculiar design and structure of the kiss flap with two semicircular flaps of equal diameter with same vascular trunk makes it amenable to cover large chest wall defects especially at places where plastic surgery facility is unavailable. We have used this flap to reconstruct large chest wall defect of size 20×18 cm in our patient operated for recurrent chest wall dermatofibrosarcoma protuberans. In the follow-up, both recipient kiss latissimus dorsi flap and donor site flap healed well without edema or extravasation.

Multiple Gastrointestinal Cancers in a Single Patient-a Rare Clinical Entity.

Multiple gastrointestinal cancers in a single patient is a rare entity. In our study, we are showing the clinical presentation and management of these patients. A fifty-nine-year-old asthenic male (already treated case of metachronous colorectal cancer in 2008 and 2011) presented with complaints of generalized weakness and fatigue. Strong family history was present with two of his first-degree relatives having diagnosed with gastrointestinal cancer at the age < 50 years with one of them having stomach carcinoma and another with GEJ tumors. On evaluation, upper GI endoscopy revealed growth at cardia and endoscopic biopsy revealed adenocarcinoma. Radiological evaluation with PET-CT scan revealed proximal stomach growth with regional lymphadenopathy. Patient was optimized for surgery and underwent D2 total gastrectomy, distal pancreatectomy and splenectomy with Roux-en-Y oesophago-jejunal anastomosis. Pathological stage revealed pT4N2M0, moderately differentiated adenocarcinoma of proximal stomach, both distal and proximal cut margins negative for tumor, LVI present with no perineural invasion, and 5/18 lymph nodes dissected were positive for malignancy. Genetic testing needs to be considered in this patient (modified Bethesda guidelines and IGCLC criteria). Familial gastric cancer are of two types: (a) hereditary diffuse gastric cancer syndrome, (b) familial intestinal type gastric cancer. Approximately 5% of patients have germ-line mutations-AD LYNCH syndrome, hereditary breast-ovarian cancer, and polyposis and non-polyposis syndrome. Once diagnosed in localized advanced stage, the best treatment is R0 resection though overall prognosis in these patients is very poor. So it is rationale to find such families with elevated risk and to do active surveillance for early diagnosis and providing prophylactic gastrectomies to them as it has proven to be beneficial in hereditary form of gastric cancer.

A clinical, physiology and pharmacology evaluation of orthostatic hypotension in the elderly.

Orthostatic hypotension is very common in the elderly. It increases morbidity and is an independant predictor of all cause mortality. It is defined as a fall in systolic blood pressure greater than 20 mm Hg or a fall in diastolic blood pressure greater than 10 mm Hg within 3 minutes of standing. Symptoms include light headedness, weakness, blurred vision, fatigue and lethargy and falls. Most patients have orthostatic hypotension due to non neurogenic causes. Drugs like antihypertensives and tricyclic antidepressants are very common causes of orthostatic hypotension. Diagnosis is based on the history and a thorough clinical examination. Based on the history and physical examination, further testing of the heart, kidneys and autonomic nervous system may be required in selected patients. Non pharmacological methods like slow position change, increased fluid and sodium intake, compression stockings and elevation of head of the bed are the key to management of orthostatic hypotension. After these methods, pharmacological treatment with fludrocortisone and midodrine should be tried. Other drugs like desmopresin acetate, xamoterol, erythropoetin and ocreotide can be used as second line agents in selected patients.