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PURPOSE Malignant peripheral nerve sheath tumor (MPNST) of small bowel mesentery is a rare tumor. We report a rare case of MPNST of small bowel mesentery in a patient without neurofibromatosis (NF). METHODS A 50-year-old male, with no features suggestive of NF1, presented to us with complaints of pain abdomen. Contrast-enhanced computed tomography (CECT) of the abdomen revealed a mass in the infrarenal region. On laparotomy, mass was seen to be arising from the mesentery of the jejunum. En-bloc resection of the tumor was done, and histopathological examination was suggestive of malignant peripheral nerve sheath tumor of the small bowel mesentery. RESULT Patient received adjuvant external beam radiotherapy to a dose of 50.4 Gy to the tumor bed. The patient was planned for chemotherapy but absconded and later came with recurrence. The patient finally succumbed to disease. CONCLUSION Surgery is the mainstay of treatment. Adjuvant treatment should be based on histopathological report.

COVID-19 pandemic has challenged the entire health care system to a great extent and led to the development and utilization of alternative approaches. Tele-oncology holds great potential to deliver cancer care. With the use of tele-oncology, physical distancing can be maintained. This will help the cancer patients as well as the oncologist and other supporting staff from getting exposed to the virus. However, there are many challenges for starting tele-oncology especially in resource limited settings. We hereby discuss tele-oncology its applications, methods available, tools, set up and infrastructure, benefits of tele-oncology and various patient, physician and resource-related factors in detail, especially in the setting of low- and middle-income countries.

Solitary bone metastasis to fibula in patients of lung carcinoma is a rare entity, with only four cases reported in literature. We, hereby, present a case of a 50 year-old-male who was given three cycles of chemotherapy for lung carcinoma with no distant metastasis but presented 2 months later with a fusiform, painful swelling around the knee that was clinically suspected to be inflammatory in nature but proved to be fibular metastasis on cytology. There was no evidence of skeletal metastasis on initial bone scan. He was given palliative radiotherapy for this with symptomatic relief.

The gastrointestinal tract (GIT) is a rare site of primary malignant melanoma. Most of the melanomas diagnosed in the GIT are secondary to cutaneous melanomas. Very few cases of primary melanoma of the stomach have been reported in the literature. We report a rare case of primary malignant melanoma of the stomach in a 60-year-old female patient who presented with nonspecific symptoms. Imaging revealed multiple liver metastases, and on subsequent evaluation, upper gastrointestinal endoscopy (UGIE) showed a lesion in the stomach. Biopsy from the lesion in the stomach was suggestive of melanoma. After excluding other sites of melanoma, a diagnosis of primary malignant melanoma of the stomach with liver metastasis was made. Primary malignant melanoma of the stomach is an exceedingly rare neoplasm. UGIE and biopsy remain the mainstay of diagnosis. Most of the patients present with advanced stage disease, and the prognosis remains dismal. The response to chemotherapy as well as targeted therapy is not well documented in the literature.

Fibromatosis colli or sternocleidomastoid tumor of infancy is a condition of benign proliferation of fibrous tissue within the sternocleidomastoid muscle leading to focal or diffuse enlargement of the sternocleidomastoid muscle and is often clinically associated with torticollis. Radiological imaging especially ultrasound, if performed by an expert radiologist plays an important role in differentiating this benign condition from other causes of neck masses in this age group, thereby preventing unnecessary investigations in a neonate and decreasing parent`s anxiety. We hereby, present a case report of a sternomastoid tumor in a two week old neonate diagnosed using high frequency ultrasound, signifying that every physician should be aware of this clinical entity in a neonate and refer them immediately for ultrasound.

BACKGROUND: Induction chemotherapy (ICT) in patients with head and neck cancer has been studied since a long time. The addition of taxanes to the cisplatin and 5-fluorouracil (5FU) (PF) regimen results in superior antitumor activity. We did this study to see the response and toxicity of ICT with cisplatin and ifosfamide followed by concurrent chemoradiotherapy (CRT) in locally advanced, unresectable squamous cell carcinoma of head and neck (SCCHN). AIMS: The aim of this study was to see the results of ICT using cisplatin and ifosfamide regimen in locally advanced unresectable SCCHN in terms of acute and chronic toxicity and response to treatment. MATERIALS AND METHODS: Patients with Stage III and IV, nonmetastatic SCCHN were enrolled in the study. They were given two cycles of ICT with cisplatin and ifosfamide followed by CRT. RESULTS: After ICT, the overall response rate (ORR) was 75.0% at the primary site and 70.0% at the nodal site. ORR for combined primary and nodal disease was observed to be 67.5%. The complete response (CR) and partial response (PR) for combined primary and nodal site were seen in 4 (10.0%) and 23 (57.5%) patients. Of 32 patients who received CRT after ICT, CR was 53.1% and PR was 31.3%. Mucositis, skin reaction, and pharyngeal and laryngeal toxicities were the most common but tolerable. CONCLUSION: ICT with cisplatin and ifosfamide gives comparable results to the standard paclitaxel, PF regimen. We conclude that this combination regimen for ICT is not only an economical alternative of taxol-based regimen but also well tolerated by the patients.

Squamous cell carcinoma (SCC) of the breast is very rare malignancy, and only few cases have been reported in the literature. Also, it is aggressive in nature, and the standard treatment is still debated. We share our experience of a case of primary SCC of breast which was initially misdiagnosed as adenocarcinoma on fine needle aspiration cytology but later confirmed to be pure primary squamous cell carcinoma of breast on histopathological examination.

Bouveret syndrome is gastric outlet obstruction due to gallstone. It is an extremely rare complication of cholelithiasis. We report a case of an elderly male who presented with epigastric abdominal pain, and gastric outlet obstruction due to migrated gallstone was suggested on computed tomography. He was managed with urgent 'one-stage' surgery with favorable outcome. Bouveret syndrome should be considered as a differential diagnosis, especially in elderly age group presenting with symptoms of gastric outlet obstruction or pancreatitis.

Xanthogranulomatous pyelonephritis is a rare chronic renal infection of unknown pathogenesis characterized by replacement of renal parenchyma by lipid filled macrophages frequently associated with an enlarged, non-functioning kidney and an obstructing calculus. We report a case of a 45 year old non diabetic female who presented with gradually enlarging renal mass with extensive retroperitoneal involvement and a non-functioning kidney with no evidence of obstructing stone or fat density and simulating malignancy. She was diagnosed as stage III Xanthogranulomatous pyelonephritis and managed with radical nephrectomy with favourable outcome.

Congenital longitudinal radial deficiency is a rare congenital anomaly and encompasses a spectrum ranging from mild hypoplasia to complete absence of radius. Furthermore known as radial club hand or radial dysplasia, there is variable degree of deficiency along the radial side of the limb. The authors report a case series of four cases; two cases of isolated radial club hand and two associated with other anomalies, including VACTERL syndrome. The rarity of the disease and the need to exclude other associated anomalies are emphasized.

Gastric volvulus as a cause of acute abdomen is uncommon, especially in children. This increases the likelihood of missed or delayed diagnosis leading to increased morbidity and mortality. Acute gastric volvulus is a potentially lifethreatening condition and timely diagnosis and management significantly reduces the chances of complications. We report a case of an adolescent male child, who presented to the casualty with acute abdominal pain in the epigastric region, associated with vomiting and epigastric belching and was being managed with a clinical diagnosis of acute pancreatitis. Eventration of left hemi-diaphragm was missed and gastric volvulus was not suspected in the initial differential diagnosis leading to delay in the diagnosis and management. The child was diagnosed using upper gastrointestinal barium study and was immediately operated upon with favourable outcome.

Ann Saudi Med 2012 November-December www.annsaudimed.net 1 Wilkie syndrome (or superior mesenteric artery syndrome), also known by other names such as aortomesenteric duodenal compression, Cast syndrome and chronic duodenal ileus first observed in 1842 by Rokitansky, but first reported by Wilkie.1 Wilkie syndrome is a rare condition that results from vascular compression of the third part of the duodenum, in the angle between the descending aorta and the origin of the superior mesenteric artery (SMA). There is a cushion of fat and lymphatics around the SMA and due to rapid weight loss, the protective action of fat is lost. On sagittal section of CT abdomen, the normal aortomesenteric angle ranges from 25° to Answer: Wilkie syndrome

Dear Editor, Hydranencephaly is a rare congenital abnormality characterized by absence and replacement of the cerebral hemispheres by a large cerebrospinal fluid pool. It is thought to be caused by occlusion of bilateral internal carotid arteries in the fetal life mainly during the second trimester due to a variety of causes.[1,2] It is one of the recognized forms of brain malformations which is usually associated with intrauterine fetal demise and is therefore rarely seen in postnatal life.[3] It is a rare entity with a reported incidence of less than 1 per 10,000 live births.[4] A 4-month-old preterm infant was brought by his parents to the pediatric outpatient department with complaints of recent onset of fever and progressively enlarging head size. The fever was of moderate degree and was not associated with chills and rigors. According to the parents, the size of the baby's head was normal at the time of birth but it started increasing after one week of life. On examination, the child was lethargic with increased occipito-frontal circumference and there was hypotonia in all the four limbs. There was no associated cranio-facial dysmorphism or limb deformity. The mother also reported that the eyes of the child showed roving movement. The primitive reflexes, such as grasp and suction, were intact. He responded only to sound and painful stimuli and a lack of awareness to the surroundings was noted. The fundus examination of the child was normal. The mother was neither diabetic nor hypertensive; she was not a smoker and had never ingested alcohol. There was no history of radiation exposure or febrile illness and exanthematous skin rash during pregnancy. She did not undergo any ultrasound examination during the antenatal period. There was no history of consanguinity or similar complaints in the other siblings. After admission, he was managed with a working diagnosis of complicated tubercular meningitis. Mantoux test and CSF examination were normal. The baby was then referred for computed tomography (CT) of the head. Axial CT of the head showed complete absence of B/L cerebral hemispheres with no cortical mantle and their replacement by fluid [Figure 1]. However, entire falx was preserved excluding holoprosencephaly. Also thalamus, pons, medulla, and cerebellar hemispheres were relatively preserved [Figure 2], all in keeping with the diagnosis of hydranencephaly. Somatosensory evoked potentials (SSEP) showed complete absence of the cortical activity with preservation of waves of brainstem origin. Electroencephalogram revealed a flat pattern. The visual evoked potential was absent bilaterally. The parents of the baby were counseled for poor prognosis of the condition and the minimal risk of recurrence in subsequent pregnancies. They refused any active surgical intervention. Figure 1 Non-contrast axial CT image shows (a) complete destruction of bilateral cerebral hemispheres with no evidence of cortical mantle with preserved midbrain and (b)falx cerebri Figure 2 Non-contrast axial CT image shows relatively normal cerebellar hemispheres, brainstem, and bilateral thalami Hydranencephaly is a rare encephalopathy that occurs in-utero and presents rarely in postnatal period. The posterior fossa structures as well as parts of the temporal and occipital lobe supplied by posterior cerebral arteries are usually preserved.[1,5] However, cases with cerebellar hypoplasia and occlusion of bilateral posterior cerebral arteries have been reported.[1] This rare disorder usually occurs after ventricles have already formed; hence, the falx is preserved. Hydranencephaly is associated with various congenital anomalies, including arthrogryposis, renal aplastic dysplasia, poly-valvular heart defect, trisomy 13 and Fowler syndrome.[6] Most common etiologies described for hydranencephaly are occlusion of the supra-clinoid segment of bilateral internal carotid arteries,[1] or an extreme form of leukomalacia formed by confluence of multiple cystic cavities. Other etiologies include fetal hypoxia due to maternal exposure to carbon monoxide or butane gas resulting in massive tissue necrosis with cavitation and resorption of necrotized tissue and necrotizing vasculitis or local destruction of the brain tissue secondary to intrauterine infections, e.g. congenital toxoplasmosis, cytomegalovirus and herpes simplex.[4,7] Hydranencephaly can be diagnosed using ultrasound (ante- and postnatal), Magnetic Resonance Imaging (ante- and postnatal) and CT. The radiological features include almost complete absence of cortical mantle. The cranium is completely filled with membranous sac containing cerebrospinal fluid. The skull may be normal-sized or enlarged as was seen in the present case. The falx cerebri and tentorium cerebelli are usually intact. Thalamus, hypothalamus and choroid plexus are preserved and cerebellum is intact.[1,4] The electrophysiological studies confirm the imaging findings. Prolonged survival upto 22 years has been reported in literature with significant associated morbidity.[3,8] Ventriculo-peritoneal shunting has been tried with limited success because of frequent need for shunt revision or removal and associated poor prognosis for psychomotor development.[9] Recently, endoscopic choroid plexus coagulation is being explored as an alternative to shunting.[10] The condition needs to be differentiated from severe obstructive hydrocephalus and severe open lip schizencephaly, however, in both these conditions, the cortical mantle is only reduced in thickness. Alobar holoprosencephaly can be differentiated by the presence of falx and frequent coexisting midline facial abnormalities. Thus, hydranencephaly should also be considered in the differential diagnosis of an infant presenting with enlarging head size, especially in children with psychomotor retardation.

Dear Editor, Gall stones are of common occurrence and frequently cause acute abdominal pain and are managed by cholecystectomy. Cholecystectomy, whether open or closed, is among the most commonly performed surgeries worldwide. The cause of cholelithiasis is infrequently investigated properly. We report a case of a middle-aged female patient who presented for routine laparoscopic cholecystectomy and pre-operative chest X-ray which lead to the diagnosis of hemolytic anemia as the cause of gall stones with compensatory extramedullary hematopoiesis (EMH). A middle-aged female patient presented to the surgery out-patient department for routine laparoscopic cholecystectomy. She recently had an episode of acute epigastric pain and was diagnosed as cholecystitis with cholelithiasis on ultrasound abdomen. During the pre-anesthetic work-up for cholecystectomy, she was referred to the radiology department for routine chest X-ray. Her routine investigations were all normal except for mild anemia (Hb –9.8 gm/dl). X-ray of the chest Postero-anterior and Lateral view showed well-defined mediastinal masses manifesting as increased retro-cardiac paravertebral soft-tissue density [Figure 1a] and as spine sign on Lateral view [Figure 1b]. Detailed history for any chest complaints was negative except for mild dyspnea and occasional cough. There was no family history of any hematological disorder. The patient was advised computed tomography (CT) of the thorax for evaluation of retro-cardiac opacity. Contrast-enhanced CT showed multiple, well-defined, lobulated, bilateral paravertebral masses with no evidence of calcification or fat density within masses and mild homogenous enhancement [Figure ​[Figure2a2a and ​andb].b]. There was no evidence of underlying bone expansion or destruction or extension into neural foramina. The lesions displaced adjacent lung field suggesting extra-parenchymal location of the lesions. Additional significant findings seen on CT in this patient were presence of gall stones, hepatomegaly, and spleenomegaly [Figure ​[Figure2c2c and ​andd].d]. Based on these radiological findings of bilateral posterior mediastinal masses; Lymphoma, Multiple Myeloma, Metastasis, EMH and Pott's spine were important differentials considered in this patient. Further hematological work-up of the patient revealed – Hemoglobin 9.8 gm/dl, hematocrit 35.6%, mean red cell volume 102 fl with increased reticulocyte count, i.e., 8%. The blood film contained about 28% spherocytes with markedly increased erythrocyte osmotic fragility on osmotic fragility lysis test, thereby suggesting the diagnosis of hereditary spherocytosis (HS). There was no evidence of any clinically palpable lymphadenopathy or retroperitoneal lymph nodes on ultrasound or CT. CT-guided fine-needle aspiration cytology of the paravertebral mass was performed which showed aggregates of erythroid and myeloid precursors with usual morphology along with megakaryocytes suggesting extramedullary hematopoiesis. Therefore, the diagnosis of HS with compensatory EMH was confirmed. Figure 1 X-ray Chest Postero-anterior and Lateral view shows well defined mediastinal masses manifesting as increased retro cardiac paravertebral soft tissue density (a) and as spine sign on Lateral view (b) Figure 2 Contrast enhanced computed tomography mediastinal window shows bilateral posterior mediastinal masses displacing adjacent lung field with no evidence of calcification or fat density within the masses (a and b). Gall stones, hepatomegaly and spleenomegaly ... HS is the most common red cell membrane disorder with an autosomal dominant or, less commonly, an autosomal recessive pattern of inheritance. The peripheral blood smears show spherocytes with an increase in osmotic fragility. The pattern of expression of this disease is highly variable. It varies from clinically asymptomatic condition to a severe, life-threatening presentation.[1,2] The cause of EMH in HS, or for that matter in all hemolytic anemia, is a compensatory mechanism in response to destruction of red blood cells leading to the production of myeloid and erythroid elements at ectopic sites. Other conditions that predispose to EMH include myelofibrosis, polycythemia rubra vera, and replacement of bone marrow by carcinoma or lymphoma. EMH commonly occurs in spleen, liver, lymph nodes, and mediastinum and less commonly in skull, kidney, or retroperitoneum.[1–4] Intrathoracic EMH is a relatively rare entity, commonly seen in the posterior mediastinum, and are frequently asymptomatic. They are frequently identified incidentally, as in our case. Rarely, they can produce symptoms of spinal cord compression if they extend to an epidural location.[5,6] The investigation of choice for EMH is magnetic resonance imaging which shows an isointense mass with a high signal intensity rim on T1-weighted images and a homogenous hyper intensity on T2-weighted images.[3,6] Treatment of patients with EMH is considered only if complications occur. Treatment options are surgery, radiation therapy, blood transfusions, or their various combinations.[6] Relatively small doses of radiotherapy are quite effective as EMH masses are highly radiosensitive.[6,7] Surgical removal of spleen is considered the treatment of choice for patients with EMH due to HS because it eliminates the main site of red cell destruction.[8] This case highlights the importance of pre-operative chest X-ray in a patient having symptomatic gallstones and an un-investigated mild anemia which lead us to the diagnosis of HS with EMH. The routine pre-operative chest X-rays, however, still remains a debated issue and probably unjustified if we do it in all the cases.[9]