Laparoscopic partial gastrectomy without lymph node dissection has been accepted worldwide for the treatment of small gastric gastrointestinal stromal tumors (GISTs). However, the role of laparoscopic surgery in the treatment of large gastric GISTs remains under debate due to the risk of tumor spillage or rupture of the tumor capsule leading to peritoneal seeding. To the best of our knowledge, one-stage laparoscopic resection for a large gastric GIST and synchronous liver metastases following preoperative imatinib therapy has not been previously reported. Here, we present our initial experience of this method of treatment. Gastric gastrointestinal stromal tumor (GIST) may be cured by sleeve resection without lymph node dissection. Laparoscopic surgery for GIST has been established as a less invasive option, provided that it is performed by experienced laparoscopic surgeons –4. HowevA 63-year-old female was admitted to the Department of General Surgery, Xuanwu Hospital, Beijing, China, complaining of melena for 5 days. Following resuscitation, gastric endoscopy was performed and a submucosal lesion in the body of the stomach was reported. A biopsy confirmed the diagnosis of gastric GIST with c-kit exon 11 mutation. Computed tomography (CT) of the abdomen showed an intramural GIST ∼15×10 cm in size with multiple liver metastases . The CT Preoperative imatinib (400 mg/day) therapy was administered to this patient. The clinical and morphologic response was monitored by physical examination and CT scan every two months  and 3. TAfter 6 months’ preoperative treatment, a partial response was achieved according to Choi criteria . LaparosPathological evaluation of the stomach specimen revealed that the structure of the mucosa was normal, and that the tumor mass was present in the muscular layer. Microscopically, although tumor cells with sharp spindles were detected in certain areas, the number had declined significantly compared with that in the biopsy specimens. Many large areas of hyaline degeneration were found with mitotic figures <5 per 50 high-power fields . The tumFour weeks following the surgery, imatimib treatment was resumed at 400 mg/day. Eleven months following the surgical resection, the imatinib treatment was ongoing, the patient was asymptomatic and there was no radiological or clinical evidence of disease recurrence.et al, GISTs with a tumor size larger than 2 cm accounted for >80% of cases (Gastric GIST is a relatively rare entity of nonepithelial, mesenchymal neoplasm with increasing rates of diagnosis due to the widespread use of upper endoscopy and endoscopic ultrasound. Gastric GIST may grow quite large without any symptoms. In the series of De Vogelaere of cases . Partialof cases –4,7,8. Hof cases  recommenof cases .In our case, surgical resection was not selected as the initial therapy for the following reasons. First, the anatomical location and the association between the giant primary tumor and the contiguous organs was not clear, with some doubt regarding the invasion to the diaphragm and splenic hilum. Extended surgery would be required without any appreciable gain in clinical benefit. Second, as multiple liver metastases were present in the different liver segments, surgery may have led to a marked loss of organ function. Third, genetic testing of biopsy specimens revealed the c-kit exon 11 mutation which indicated a notable response to imatinib therapy . GuideliOwing to the cooperation of oncologists and surgeons, our patient received curative treatment with visceral and functional preservation, maintaining a high quality of life.One-stage laparoscopic resection may be an option for large gastric GISTs with synchronous liver metastases. For advanced or unresectable GIST, preoperative imatinib treatment may be a bridge to curable disease. Minimally invasive approaches may be as safe and effective as open surgery. A greater number of cases and long-term follow-up are necessary to confirm this.


Pneumatisation of styloid process is a very rare finding and has never been reported previously. We present a unique case of a pneumatised styloid process with a cholesteatoma arising within the cavity. We describe the clinical features, associated radiological findings, and management of this lesion. Styloid process develops from the dorsal part of the second branchial arch (Reichert cartilage) at around fourth week of intrauterine life , 2. Its A 43-year-old female presented to our otolaryngology department with an 11-month history of recurrent, right-sided otalgia and foul-smelling otorrhoea. The condition had been misdiagnosed as recurrent otitis externa. Patient denied any history of hearing impairment, vertigo, tinnitus, facial nerve palsy, or a history of head trauma. Clinical examination revealed squamous debris and purulent material in a grossly eroded floor of bony meatus with the lower half of the tympanic membrane hanging in the breeze. The facial nerve function was normal and there were no other neuro-otologic signs. Examination of contralateral ear and rest of the ENT examination were unremarkable.CT scan of the temporal bone demonstrated an extensively pneumatised styloid process containing a soft tissue mass Figures . FurtherThe patient subsequently underwent a right combined approach tympanoplasty with extended posterior tympanotomy approach continuing the dissection inferiorly to the hypotympanum and down to the styloid apparatus. The lesion in the pneumatised styloid process contained a fibrous cyst wall full of cholesteatoma. This was completely excised and the cavity was obliterated with fat. Cartilage was used to reconstruct the defect in the floor of the bony external auditory canal. There were no postoperative complications and recovery was uneventful. Patient remains disease free until the last followup (14 months after surgery). The exact gestational stage at which pneumatisation of temporal bone starts is controversial; however, it is thought to start sometime between 24 and 38 weeks in utero and then continue into adult life [Anatomically the pneumatisation of temporal bone can be divided into five regions, mastoid, middle ear, perilabyrinthine, petrous apex, and accessory region. The detailed description of temporal bone pneumatisation is beyond the scope of this paper and is well described by Virapongse et al., 1985 . It is tPneumatisation of temporal bones is thought to be necessary for lightening the weight of the skull, providing a reserve volume of air (air reservoir action), insulation, reception, resonance, and acoustic dissipation of sound. Any alteration of the reservoir function of temporal bone and eustachian tube dysfunction leads to generation of negative pressure within the middle ear culminating into retraction of tympanic membrane and cholesteatoma formation , 4. Cholesteatoma most frequently involves the middle ear and mastoid but may develop anywhere within the pneumatised portions of the temporal bone including the petrous apex . The litBy the nature of the disease cholesteatoma causes destruction of the surrounding bone and local erosions which at times may be extensive . It is nDiagnosis of cholesteatoma is made clinically although radiology is helpful in difficult cases as described here. CT and MRI with their various sequences  complement the clinical examination  as in ouSurgical resection is the management of choice for both acquired and congenital cholesteatoma. The two primary goals of surgery are to remove the disease whilst managing complications and to reconstruct the middle ear. Prognosis of both congenital and acquired cholesteatoma is generally good.In summary, we present the first reported case of pneumatisation within the styloid process. Unusually, the patient in this case also developed cholesteatoma within the cavity. Pre-treatment scanning of the temporal bone and stylomastoid apparatus should always be performed before planning the surgery so as to minimize chances of leaving behind the residual disease at unusual places of pneumatisation of temporal bone including the skull base.


A 66-year-old white man presents with a 3-year history of a pruritic, burning, erythematous lesion in the left groin. It was treated in the past with topical antifungals without any improvement. Physical examination revealed a 7× 5-cm erythematous lesion in the left groin . A 3-mm What is the differential diagnosis?What other underlying conditions may be present?What are the treatment options?The patient's skin lesion punch biopsy, and subsequent treatment with wide local excision, confirmed extramammary Paget's Disease (EMPD), which is a rare, slow-growing neoplastic condition  of apocr,Extramammary Paget's disease generally presents between the ages of 50 and 80 years, and most commonly in white women. The most common presenting complaint is pruritus,2,4,Although surgical excision carries a high recurrence rate, it remains the standard therapy for EMPD.5


A 71-year-old man suddenly developed abdominal pain and vomiting on drinking soda after a meal, and visited a physician. Cervical subcutaneous and mediastinal emphysemas were observed on CT, and the patient was transferred to the emergency medical center of our hospital on the same day. Esophagography was performed at our department. A ruptured region was identified on the left side of the lower thoracic esophagus, and surgery was emergently performed employing sequential left thoracoabdominal incision. The chest wall was adhered due to inflammation, and large amounts of residual food and sloughing were present in the thoracic cavity and mediastinum. Moreover, necrotic changes were noted in the superior through inferior mediastinum. An about 2-cm rupture site was confirmed on the left side of the lower thoracic esophagus and closed by suture and filling with pediculate omentum. The presence of a tumorous lesion located mainly in the body of the stomach and lymph node enlargement were also diagnosed before surgery, for which gastric and intestinal fistulae were inserted to prepare for the second-stage surgery. The patient was admitted to an ICU after surgery. ARDS and MRSA-induced pneumonia and enteritis concomitantly developed but remitted. Curative surgery for gastric cancer was performed at 40 POD. Spontaneous rupture of the esophagus is relatively rare and that complicated by gastric caner is very rare, with only six cases being reported in Japan. Herein, we report the case. Spontaneous rupture of the esophagus is rare, and the life-saving rate is still low. We encountered a very rare case in which rupture occurred due to concomitant gastric cancer. Only 6 cases including this patient have been reported in Japan, and this is the first case report in English. The disease could be preoperatively diagnosed, and the patient could be saved despite the condition being very serious. Herein, we report the case.3/ul and CRP was 0.34 mg/dl, showing high levels of inflammatory parameters. BUN was 46 mg/dl and Cr was 4.03 mg/dl, revealing features of prerenal renal failure. PT-INR was 1.35; AT-III, 63%; and FDP, 64 μg/ml. CEA was 11.7 ng/ml, showing a high level.The patient was a 71-year-old male with a chief complaint of abdominal pain. There was no particular past medical history. The patient suddenly developed abdominal pain and vomiting on drinking soda after a meal, and visited a physician. Cervical subcutaneous and mediastinal emphysemas were observed on CT, and the patient was transferred to the emergency medical center of our hospital on the same day. On admission, the height was 168 cm; body weight, 55 kg; BP, 154/76 mmHg; HR, 93/min; respiratory rate, 35/min; and SpO2, 98% (O2 5 L mask). The patient could not hold a supine position due to respiratory distress and pain. On blood testing and chemistry on admission, WBC was 14.5 × 10On plain thoracoabdominal X-ray radiography, no apparent free air was present, but marked right pleural effusion was noted Figure .On thoracoabdominal CT, marked subcutaneous emphysema was present in the cervical region through the mediastinum, and pleural effusion was also observed Figures . MoreoveThe ruptured site was identified on the left side of the lower thoracic esophagus on esophagography and diagnosed as spontaneous rupture of the esophagus. Surgery was emergently performed in consideration of concomitant gastric cancer.The first surgery was performed employing sequential left thoracoabdominal incision. The chest wall was adhered due to inflammation, and large amounts of residual food and sloughing were present in the thoracic cavity and mediastinum Figure . Moreove1). In Japan, Yoshida et al. [In spontaneous rupture of the esophagus, a sharp rise in intraesophageal pressure due to vomiting causes full-thickness (mucosa and muscle layer) rupture. Since the disease was initially reported by Boerhaave  of Hollaa et al. reported There is a high incidence in men, accounting for 92% of all cases. The onset age peaks in the 40s, and incidences at 30-59 years of age account for 73% of all cases. The rupture site was located in the lower esophagus in 84% and left wall in 67%, and the most frequent inducer was vomiting (64%) followed by other mechanical factors, such as overeating, cough, and trauma, accounting for 19% [For treatment, surgery and conservative treatment including thoracic cavity drainage are available, but surgical treatment may be the most appropriate . FukushiIt is still controversial whether laparotomic drainage alone in the mediastinum or thoracotomic drainage should be adopted -10.In our patient, not only rupture of the esophagus but also hypertrophy of the antral stomach wall and lymph node swelling were observed on preoperative MDCT, for which sequential left thoracoabdominal incision (oblique incision) was selected. Gastric (to reduce pressure) and intestinal (for tube nutrition) fistulas were inserted, in addition to drainage, providing useful for postoperative enteric nutrition.Of cases assumed to be induced by vomiting, duodenal ulcer was the most frequent cause, and gastric ulcer, postgastrectomy disorder, and congenital obstruction are included in the causes. In this patient, in addition to the underlying vulnerability of the esophageal wall and promotion of nausea and vomiting, the pylorus was obstructed by gastric cancer as a local factor of nausea and vomiting. Under these conditions, ingestion of the meal and soda may have rapidly elevated the inner pressure and caused rupture.This is a valuable case: the 6th case of spontaneous rupture of the esophagus associated with concomitant gastric cancer in Japan -14, and Spontaneous rupture of the esophagus associated with concomitant gastric cancer are rare.To our knowledge this is the first reported case.Written informed consent was obtained from the patient for publication of this case report and accompanying images.A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.NN and CT took part in the operation. TN,MK and KK performed the literature seach and drafted the manuscript for submission. HI performed histological examination and provided photomicrographs. All authors read and approved the final manuscript.


A 34-year-old female was admitted to Emergency Department with an abrupt onset of diffuse abdominal pain. A CT scan done prior to her transfer revealed significant dilated loops of bowel as well as multiple target signs with likely torsed bowel. The patient consented to an exploratory laparotomy. During surgery, the proximal jejunum was found to be intussuscepted, a rare finding in an adult. There was evidence of mesenteric foreshortening throughout the small bowel and multiple whitish lesions within the mesentery, both consistent with the desmoplastic response that is characteristic of carcinoid. The interest for this case report comes from the patient's surgical findings of jejunal intussusception as well as her extensive history, which includes a bowel resection with an ileocolic anastomosis for presumed ischemia and a carcinoid tumor in the stomach which had been removed endoscopically. Carcinoid disease is known for its wide array of signs, symptoms, and ubiquitous gastrointestinal involvement. Carcinoid tumors are neuroendocrine in origin. The clinical presentation of the tumor depends on its location and most tumors are asymptomatic. In patients who are symptomatic, forty percent will present with abdominal pain. This may be due to intussusception, mesenteric ischemia, or a mechanical obstruction caused by the tumor . The preA 34-year-old female was transferred from an outside hospital with a sudden onset of diffuse abdominal pain. Her history is significant for malrotation with subsequent volvulus, small bowel resection done for ischemic bowel, carcinoid tumor of the stomach, multiple gastric ulcers, and a tubular adenoma of the colon. She first presented in 2005 with suspected ischemic bowel, which was resected and repaired with an ileocolic anastomosis. Her next presentation was in January of 2013 at which time she had severe anemia with a hemoglobin of 4.2 mg/dL. An EGD revealed a carcinoid tumor in the lesser curvature of the stomach that was endoscopically removed. A polyp removed during colonoscopy was identified as a tubular adenoma. She presented again the next month and an abdominal CT revealed two lesions in the liver, a left adrenal nodule, and an umbilical hernia. The liver nodules prompted a workup carcinoid syndrome, but the urine was negative for 5-HIAA.Upon her arrival to our hospital in August of 2013, she was noted to have moderate generalized tenderness on physical exam and her last bowel movement was earlier that day. An abdominal CT scan done prior to her arrival was suggestive of new marked mechanical traction of the distal small bowel. There were also three target signs present on imaging, a classic representation of intussuscepted bowel . The patA CT scan was the primary tool for diagnosis in this patient. Multiple dilated loops of small bowel as well as target signs were present, indicating a possible obstruction as well as intussusception.A patient that presents with diffuse abdominal pain may have a perforated viscus, a bowel obstruction, or some other process that has resulted in irritation of the peritoneum. The most common cause of a bowel obstruction is adhesions from previous abdominal surgery. This was initially suspected in this patient given her surgical history. A thorough history and physical including onset and location of pain may help to rule out various causes of an acute abdomen. While often times the management and treatment of these conditions are the same, it is important to properly investigate the etiology of disease to avoid missing potential sequelae. When diagnosing a patient with potential carcinoid, it is important to remember that it may be a clinical diagnosis based on the appearance and location of the tumors throughout the GI tract, in addition to mesenteric foreshortening. In the presence of a bowel obstruction, the etiology often cannot be established prior to an exploratory laparotomy. The most common causes of bowel obstruction are adhesions and hernias, but in our case neither was determined to be the culprit ultimately.In most cases of focal carcinoid disease, surgical resection is both diagnostic and curative. However, the patient is often asymptomatic and does not present clinically until the disease has caused an obstruction of the bowel or manifestations of carcinoid syndrome indicate metastasis to the liver. The patient in our case study appeared to have multiple manifestations of disease and chronic anemia that may have been linked to her diffuse gastrointestinal involvement.The patient was discharged on postoperative day eight and was followed up as an outpatient in the clinic. She had no apparent complications from the operation and appeared to recover well. It is recommended that the patient follow up every 6 months.A patient that presents with diffuse abdominal pain may have a perforated viscus, a bowel obstruction, or some other process that has resulted in irritation of the peritoneum. The most common cause of a bowel obstruction is adhesions from previous abdominal surgery. This was initially suspected in this patient given her surgical history. A thorough history and physical including onset and location of pain may help to rule out various causes of an acute abdomen. While often times the management and treatment of these conditions are the same, it is important to properly investigate the etiology of disease to avoid missing potential sequelae. When diagnosing a patient with potential carcinoid, it is important to remember that it may be a clinical diagnosis based on the appearance and location of the tumors throughout the GI tract, in addition to mesenteric foreshortening. In the presence of a bowel obstruction, the etiology often cannot be established prior to an exploratory laparotomy. The most common causes of bowel obstruction are adhesions and hernias, but in our case neither was determined to be the culprit ultimately. Carcinoid tumors have a variety of clinical presentations, depending on their location and size. Tumors in the small bowel are hypothesized to arise from subepithelial endocrine cells and are most commonly located within sixty centimeters of the ileocecal valve. Abdominal pain is the clinical presentation in forty percent of patients and it may be linked to a mechanical bowel obstruction from the tumor itself or intussusception. Mesenteric ischemia caused by local fibrosis is also seen and is representative of the desmoplastic response that is pathognomonic for carcinoid . Other gJejunal intussusception is rare in both adults and children.Risk factors for carcinoid include family history of MEN I, African American race, tobacco use, atrophic gastritis, Zollinger-Ellison syndrome, and pernicious anemia.There is no standardized screening for carcinoid.Mesenteric foreshortening is the hallmark of carcinoid in the gastrointestinal system.Target sign on CT scan is indicative of intussusception and the need for an exploratory laparotomy.Intussusception in an adult warrants surgery to evaluate the underlying cause.


We report a case of primary extraosseous Ewing sarcoma (EES) of the lung in a four-year-old child. In the literature, there are only a few case reports of EES located in the thorax. Extraosseous Ewing sarcoma (EES) is an uncommon malignant neoplasm in which pulmonary localisation is exceptionally rare.A four-year-old girl, without any medical history, was referred to our department for a lung mass . An initAt the end of chemotherapy, after a negative search for metastasis, we performed a radical resection, which consisted of a left pneumonectomy. The pathologic examination confirmed the need for a complete resection and also confirmed the initial diagnosis. During an interdisciplinary meeting, it was decided that postoperatively the patient would receive seven courses of chemotherapy  without radiotherapy. The patient is currently receiving postoperative chemotherapy.In our case, the patient was a four-year-old girl, which was unusually young when compared with the previously reported cases –6. The mThe chemotherapy treatment was done according to the Eurowing 99 protocol and was also followed by a very aggressive surgery justified by the size, location, as well as the aggressive character of the tumour –5. AccorIntrathoracic EESs are extremely rare and complex conditions requiring a pluridisciplinary collaboration. This case highlights the importance of preoperative evaluation and strategy in aggressive tumours.The authors declare that there are no conflicts of interest that could be perceived as prejudicing the impartiality of the research reported.


Transverse testicular ectopia (TTE) is a rare anomaly in which both testes descend through a single inguinal canal. We report a case of yolk sac tumor in the ectopic testis of a patient with TTE. A 24-year-old man presented to our hospital with a left inguinal-mass, right cryptorchidism and elevated alpha-fetoprotein (AFP). A left herniotomy 3 years earlier demonstrated both testes in the left scrotum, one above another positionally. Four months ago, a left scrotal mass appeared and radical orchiectomy of both testes revealed testicular yolk sac tumor of the ectopic testis. An enlarging left inguinal-mass appeared 2 months ago and he was referred to our hospital. Laboratory data showed an elevation of AFP (245.5 ng/ml) and a 46 XY karyotype. He underwent bilateral retroperitoneal lymph node dissection and simultaneous left inguinal mass dissection. Histopathologic examination revealed a diagnosis of recurrent yolk sac tumor in the left inguinal mass. The retroperitoneal lymph node was not enlarged and, on histopathology, was not involved. The patient has now been followed up for 8 months without evidence of biochemical or radiological recurrence. Transverse testicular ectopia (TTE), also named crossed testicular ectopia, is a rare anomaly in which both testes descend through a single inguinal canal while the opposite inguinal canal and hemiscrotum are empty. More than 100 cases of TTE have been reported since Von Cenhossek described the first case in 1886. As withA 24-year-old man, who had fathered a child, presented to our hospital with a left inguinal-mass Figure , right cOn admission, physical examination revealed a normal constitution, with secondary sex characteristics and normal external genital development. His karyotype was 46 XY. Laboratory data showed an elevation of AFP (245.5 ng/ml). CEA and HCG levels, however, were within normal ranges. Chest x-ray and abdominal CT were normal.The patient underwent open bilateral modified retroperitoneal lymph node dissection (RPLND) and simultaneous left inguinal mass dissection at our hospital. During operation, bilateral spermatic cords were found to descend through the left internal inguinal ring Figure  and no pTTE is a rare form of testicular ectopia. The clinical presentation generally includes an inguinal hernia on one side and a contralateral or sometimes bilateral cryptorchidism . In the TTE has been classified into three types on the basis of associated anomalies: (1) associated with inguinal hernia alone (40% to 50%); 2) associated with persistent or rudimentary müllerian duct structures (30%); and (3) associated with other anomalies without müllerian remnants  (20%)[ associatPatients with TTE are at increased risk of malignant transformation, with a 5%-18% overall incidence rate, similar to the rate of cryptorchidism. There hTherapeutic approaches for TTE include transseptal orchiopexy or extraperitoneal transposition of the testis and a search for müllerian remnants and other anomalies. HoweverThis patient is classified as having a clinical stage I nonseminomatous germ cell tumor (NSGCT) of the ectopic testis. Histopathologic examination revealed a diagnosis of yolk sac tumor in the recurrent left inguinal mass. Such patients can be managed by surveillance, primary chemotherapy or nerve-sparing RPLND.. Surveillance can spare patients without metastasis from undergoing chemotherapy or RPLND. However, according to Foster's review, approximately 30% of patients with clinical stage I NSGCT have occult metastases that are difficult to identify with imaging. In addiAlthough platinum-based chemotherapy for yolk sac tumors has produced excellent survival results, its side effects are not negligible. Raynoud's phenomenon (25-30%), ototoxicity (20%), neurotoxicity (15%), and nephrotoxicity (31%) are some of the common complications that occur in survivors of testicular cancer . The advWe present a rare case of a scrotal yolk sac tumor in a patient with type 1 TTE. The diagnosis of TTE should be considered when unilateral hernia and concurrent cryptorchidism of the contralateral side are present. The malignant potential of the ectopic gonads must be recognized and long-term follow-up is mandatory. For patients who have not yet reached puberty, orchiopexy is recommended and orchiectomy is indicated only for testes that cannot be mobilized to a palpable location. For adult patients, dissection of the ectopic testis and active surveillance of the remnant is recommended. For patients with scrotal yolk sac tumor and type 1 TTE, RPLND, surveillance and adjuvant chemotherapy are all accepted treatments for long-term survival after orchiectomy.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor in Chief of this journal.The authors declare that they have no competing interests.YPZ, SLZ and DWY conceived the concept, participated in drafting the manuscript, and conducted critical review. GHS and WJX took part in the care of the patient, assembled data, and participated in writing the manuscript. All authors read and approved the final manuscript.


Parosteal osteosarcoma is a well-differentiated variant of osteosarcoma that affects the surface of the bone. The imaging pattern is very typical. We report two cases mimicking an osteochondroma, radiologically and histologically and propose an explanation.The review of 86 parosteal osteosarcomas of bone revealed this atypical pattern only once. A consultation case was received in the same time, and added to ours. Patients were 28 years old and 56 years old females. Imaging studies included two radiographs, two CTscans, one MRI examination and one bone scan and the results were compared to histology.On imaging, both lesions presented as ossified lobulated masses attached with a broad base to the underlying cortex. No radiolucent cleft separated the masses and the host bone and cortex continuity between the mass and the femur was seen, with medullary communication. The marrow of the mass had a different density and intensity compared to normal marrow. So, there were features of an osteochondroma (cortex and medullary continuity) and of a parosteal osteosarcoma (ossified marrow). Pathological assessment on the final specimen confirmed the presence of low-grade parosteal osteosarcomas, after an erroneous diagnosis of osteochondroma on the initial biopsy.Parosteal osteosarcoma can be rarely confused with osteochondroma. A radiologic-pathologic correlation is essential. Cortex continuity is the most misleading imaging feature that may occur in parosteal osteosarcomas. A knowledge of this misleading pattern will help diagnose the lesion from the beginning. TheThe study included two female patients aged 56 and 24 years old respectively. Both patients referred to our institution for evaluation of a palpable mass located at the postero-lateral aspect of the distal femur. Information regarding clinical history, physical examination and laboratory tests was recorded. Retrospective evaluation of the available imaging studies was carried out. Both cases were assessed with radiographs  and CT scans before and after contrast medium i.v. infusion. Additionally, one patient underwent a bone scan and an MRI study which included T1WSE before and after gadolinium administration and T2W FAT SAT sequences. Imaging results were compared with the initial biopsy findings and final histopathologic diagnosis.Both patients presented with focal swelling and mechanical blockage of the left knee. The duration of symptoms was 24 and 36 months for each case with no related trauma or infection during this period. Physical examination revealed two hard, immobile tender masses at the postero-lateral aspect of the distal left femur associated with flexion restriction of the joint at 90° degrees. There was no vascular or neurological deficit of the affected limp. The patients did not notice any significant enlargement of the swelling during the period of the symptoms. Routine blood tests were not significant for any pathological findings.Radiographs showed two "exophytic" mineralized masses with a sessile configuration attached to the posterior surface of the left distal femur metaphysis  . It consMacroscopically, POS presents as a dense and well-defined ossified mass attached to the underlying cortex . On histRadiographically, POS presents as a densely mineralized lobulated mass with irregular margins and attached to the subjacent cortex ,9. A chaThe differential diagnosis of POS may also include other diverse entities such as mature juxtacortical myositis ossificans, parosteal osteoma, fracture callus, Nora's lesion, periosteal osteosarcoma and/or chondrosarcoma that can be easily distinguished ,5,11. InIn conclusion, osteochondroma is the main counterpart of POS in terms of imaging differential diagnosis. Cortex continuity and in a lesser degree merge of medullary cavities, which are by definition expected in osteochondromas, may be atypically seen in POS. Awareness of these features and thorough radiologic-pathologic correlation is crucial in avoiding erroneous diagnostic considerations and treatment.The authors declare that they have no competing interests.ZP wrote the article. MA and MG reviewed the histology. PT gave the second case, and checked the manuscript. GB checked the accuracy of the surgical part, CT reviewed the manuscript. DV proposed the subject and checked the review of the cases, references and global accuracy. All authors read and approved the final manuscript.


Congenital adrenal hyperplasia (CAH) is a rare congenital disorder, which in cases of female genotype may result in virilization. Specific enzyme deficiencies in adrenocorticoid hormones biosynthetic pathway lead to excess androgen production causing virilization. Classic type presents early in infant life as salt losing or simple virilizing type, whereas non classic form presents late at puberty or in adult life. Depending on the type of classic CAH, type of adrenocorticoid deficiency, extent of virilization & genotype, surgical corrective procedures, glucocorticoid & mineralocorticoid replacement therapy are the mainstay of management. We present here a case of classic congenital adrenal hyperplasia of simple virilizing type, which presented later in childhood. Birth of neonate is immediately accompanied by assignment of gender based on features of external genitalia. Gender assignment becomes a dilemma if external genitalia do not conform to the normal male or female appearance. Ambiguous genitalia, the congenital condition in which the external genitalia do not conform to either sex, give rise to extreme anxiety & concern to the parents.  Early but accurate & appropriate diagnostic evaluation is required in such cases. Key feature in physical examination is the presence of gonads in labioscrotal folds or inguinal canal. In the absence of palpable gonads, possibility of female pseudohermaphrodite is considered whereas in case of palpable gonads diagnostic evaluation is directed to male pseudohermaphrodite. 1,2 Virilized females with 46 XX karyotype have ambiguous external genitalia but normal internal female genitalia. During first seven weeks of gestation external genitalia of male & female genotypes are identical. In the absence of testosterone & dihydrotestosterone (DHT) external genitalia appear phenotypically female. DHT, the converted form of testosterone under the action of enzyme 5α reductase, acts on urogenital sinus & genital analgae to affect male differentiation of external genitalia. 5 α reductase is present in urogenital analgae in both male & female fetuses. Exposure of abnormal androgen levels cause masculinization of female external genitalia. Absence of antimullerian hormone leads to normal female internal genital organs. Any posterior labial fusion (defined as a ratio of the distance from anus to fourchette/anus to base of clitoris >0.5) or clitoromegaly  constitutes ambiguous genitalia and is indicative of a virilizing process. Among the variety of causes of ambiguous genitalia, congenital adrenal hyperplasia (CAH) is the commonest reported cause in the presence of 46XX karyotype. We report here a case of ambiguous genitalia resulting from congenital adrenal hyperplasia presenting later in childhood.  A 7 & 1/2 years female child was brought in outpatients clinic with history of ambiguous genitalia since birth. She had five siblings including an elder sister. Mother’s history did not reveal intake of antiepileptic drugs, progestogens or other drug treatment. There was no history of genital ambiguity in parents or any of the family members. Child was noted to have abnormal genitalia at the time of her birth at home, but medical help was never sought for that. She was in a good state of health with weight 30 kg & height 137 cm. Blood pressure was 100/60 mm of Hg.3,4 There was no abnormality on systemic examination. On laboratory investigations, serum Na was 143meq/l, potassium 4meq/l. Examination revealed no breast development, no axillary hair. On genital examination there were a few pubic hairs, clitoromegaly i.e. stretched phallus size 5cm, labia majora & minora normally developed no fusion abnormality, normal introitus, gonads not palpable, single uretheral opening below the base of phallus. These findings were consistent with Prader stage 1 for ambiguous genitalia. Ultrasound pelvis revealed uterus normal size for age, 3.5 cm in length & 2.1cm in transverse diameter, central midline echo, right ovary 2.3 X1.4 cm, left ovary 2.1 X1.4cm. karyotype was 46XX.  On the basis of raised 17αhydroxyprogesterone, testosterone, & Dehydraepiandrostanedione Sulphate (DHEAS) & decreased cortisol response to Adrenocortictropic Hormone (ACTH) stimulation in these results, alongwith normal karyotype & clitoromegaly in the presence of normal introitus, labia & female reproductive organs, classic congenital adrenal hyperplasia due to 21 hydroxylase deficiency was diagnosed. Her management comprised surgical correction of enlarged phallus by clitoroplasty with dorsal neurovascular bundle preservation & glucocorticoid replacement therapy. The operation was performed by the team of plastic surgeon & Gynaecologist. A traction suture of 3/0 poplygalactin, was placed in the glans of clitoris. Circumferential incision was given over base of the glans. Two longitudinal incisions were made lateral to dorsal neurovascular bundle. Corpra cavernosa visualized in entire extent. Excision of a portion of corpoeal tissue with wedge resection of glans was done, preserving neurovascular bundle. Conserved portion of corpora folded & sutured to pubic symphisis. Reconstruction of skin of labia minora & hood of clitoris was done. Postoperatively she recovered well with no complications. She was also started on replacement therapy with hydrocortisone.5 Whereas 11β OHD accounts for 5-8% cases.4,5 21α OHD is a cytochrome P450 enzyme located in endoplasmic reticulum. There are 2 genes encoding for 21 hydroxylase, the structural gene encoding p450c21 (CYP21) & pseudogene CYP21p, both located on chromosome 6p21.3.4 Incidence of 21α OH deficiency varies between 1 in 5000 to 1 in 15000 based on neonatal screening programmes.5 A hospital based study from Karachi reported diagnosis of congenital adrenal hyperplasia in 41.44% cases of ambiguous genitalia.6 It also showed that consanguinity of parents was identified in 52% of these cases. Incidence of CAH is reported as 1 in 15000 to 16000 births. These are a group of monogenic autosomal recessive disorders characterized by impaired cortisol synthesis & abnormal adrenal hormonal profile. These disorders are caused by deficiency of enzymes necessary for the synthesis of adrenal corticosteroids. Spectrum of severity of this condition ranges from severe salt losing type due to aldosterone & cortisol deficiency to a milder form with mild androgen excess. Important steps in the biosynthesis of corticosteroids involve conversion of pregnenolone & 17 hydroxy pregnenolone to progesterone & 17 hydroxy progesterone respectively, mediated by enzyme 3 β hydroxyl steroid dehydrogenase (3β HSD). These compounds in turn are converted to Deoxycorticosterone  & 11 deoxycorisol through the action of 21α hydroxylase [21α OHD] Final conversion of each of these precursors to Corticosterone & cortisol respectively is mediated through 11 β Hydroxylase [11β OHD]. Enzymes necessary for these steps in biosynthesis, if deficient lead to diversion of this pathway away from corticosterone & Cortisol synthesis to androstanedione & testosterone synthesis. Cortisol is the only adrenal hormone able to stimulate biofeedback loop. Corticosteroids deficiency leads to raised adrenocorticotrophic hormone (ACTH) which stimulates adrenal cortex to produce more androgenic hormones leading to features of masculinization  of female fetus resulting in ambiguous genitalia. Thus deficiency of any of the three mentioned enzymes may lead to congenital adrenal hyperplasia leading to virilization. Most common enzyme deficiency in causing CAH is 21α OHD, accounting for 90% cases of CAH.3 11β OHD deficiency is the second commonest cause of classic CAH. It is the hypertensive form of CAH, as absence of 11β OHD leads to accumulation of 11 deoxycorticosterone. Though aldosterone levels are decreased but salt retaining properties of 11 deoxycorticosterone result in hypertension. According to time of presentation of androgenization, CAH can be of classic or non classic type. The classic type, presents early in life as salt losing type or simple virilizing type, depending on presence of both cortisol & 11 deoxycorticosterone (DOC) deficiency or sole deficiency of cortisol. Affected females are born with enlarged clitoris, fused labioscrotal folds & a urogenital sinus which may become a phallic urethera. Affected females can present a range of virilization of external genitalia from mild clitoromegaly & posterior fusion to complete labioscrotal fusion & urogenital sinus which may open on perineum or shaft of phallus. 21α OHD deficiency leading to failure of conversion of progesterone to 11-deoxycorticosterone may result in aldosterone deficiency which is called “salt losing type” of CAH. It accounts for 75% cases of classic CAH, in which neonate develops dehydration, hypotension & hyponatremia between 7-28 days of life. In “non salt losing” type there is lesser degree of masculinization as compared to salt losing type,4,5 Non classic type of CAH presents later in childhood or near puberty & is characterized by normal production of cortisol & aldosterone with excess production of androgens. It is always caused by 21α OHD & only rarely due to other causes. Non classic CAH is common with an incidence of 1 in 100 to 1 in 300.7 Diagnosis of 21α OHD is made with raised serum 17 hydroxyprogesterone. In 11β OHD deficiency, 11 deoxycorticosterone level is raised whereas in 3β HSD deficiency, serum 17 α hydoxypregnenolone & DHEAS are raised.8 Surgery is tailored according to amount of virilization to best suit the gender role. Early diagnosis & management are likely to yield best possible outcome. For clitoral reduction & recession major corrective surgery is ideally suitable at 3 to 6 months of age.8 Surgical techniques of preservation of neurovascular bundle & glans as well as corporeal preservation including recession dismembered clitoroplasty are increasingly being favoured.8,9 Our case was classic type of CAH but instead of infancy she presented late in childhood despite the abnormality being identified at birth. There are local & Indian reports of CAH presenting in later childhood despite being recognized at birth.10,11 Management of cases of ambiguous genitalia depends on location of gonads, adequacy of phallus, size & location of vaginal orifice; if present need to be assessed. Management of CAH is a complex issue due to delayed help seeking & lack of neonatal screening programmes in our set up. Accurate diagnosis of this rare disorder requires specialized endocrinological assays, which are not easily available at public sector set up for poor patients. Expert genital reconstructive surgery tailored to best suit the gender role are prerequisite for proper management so as to enable social & psychological adjustment of affected patients.


Schwannomas may arise from any peripheral nerve containing Schwann cells. However, sural nerve schwannoma is extremely rare. In this study, a case of solitary schwannoma originating from the sural nerve in a 42-year-old male is presented. Physical examination revealed a 3-cm, elastic-hard, mobile, non-tender mass, while neurovascular examinations, including Tinel’s sign, were normal. Magnetic resonance imaging revealed an oval-shaped subcutaneous mass with iso-signal intensity relative to skeletal muscle on T1-weighted sequences. T2-weighted spectral presaturation with inversion recovery sequences showed higher signal intensity peripherally and lower signal intensity centrally, representing a target sign. Contrast-enhanced T1-weighted sequences demonstrated a marked central enhancement of the mass. The tumor was completely enucleated using an intracapsular technique. Histological examination confirmed the diagnosis of a schwannoma, consisting mainly of Antoni A tissue. The patient had no evidence of local recurrence and no neurological deficit at the final follow-up. Although rare, schwannoma should be considered in the differential diagnosis of a well-defined, oval, subcutaneous mass in the posterior aspect of the lower leg. Schwannomas are benign peripheral nerve sheath tumors composed exclusively of Schwann cells. The peak incidence occurs in the fourth to the sixth decades of life, with no gender predilection. Patients usually present with a slowly growing mass in the head, neck and the flexor surfaces of the upper and lower extremities . Pain anThe histological hallmark of schwannoma is the alternating pattern of Antoni A and B areas. The relative levels of these two components vary, and they may blend imperceptibly or change abruptly. Antoni A areas are highly cellular and demonstrate nuclear palisading and Verocay bodies. Antoni B areas are less cellular and far less orderly. A number of schwannomas have thick-walled vessels with fibrinoid and hyaline changes in the vessel walls .Sural nerve schwannoma is exceptionally rare. This study presents an unusual case of solitary schwannoma originating from the sural nerve in a middle-aged man. Written informed consent for publication was obtained from the patient.A 42-year-old male patient was referred to Fukuoka University Hospital  with a >30-year history of a slowly growing, painless mass in the posterolateral aspect of the left distal lower leg. Physical examination revealed a 3-cm, elastic-hard, mobile, non-tender mass, while neurovascular examinations, including Tinel’s sign, were normal. The patient’s past medical history included nothing of note. Magnetic resonance imaging (MRI) demonstrated an oval-shaped subcutaneous mass. The mass showed iso-signal intensity relative to adjacent muscle on T1-weighted sequences , and higThe surgery was performed under general anesthesia with tourniquet control and loupe magnification. The tumor and the proximal and distal portions of the affected nerve were exposed , and theThe postoperative course was uneventful. At two months of follow-up, the patient had no evidence of recurrence and no neurological deficit.et al uptake by schwannomas (The radiographic features of schwannomas are non-specific. On MRI, the lesions usually demonstrate iso- or low signal intensity relative to skeletal muscle on T1-weighted images and high signal intensity on T2-weighted images. A fascicular appearance and a thin hyperintense rim may be observed on T2-weighted images . Furtherwannomas –11. ThesIn the present case, the possible differential diagnosis included neurofibroma and benign perivascular tumors, such as angioleiomyoma. Neurofibromas may assume one of three growth patterns: Solitary, diffuse or plexiform. Solitary neurofibromas usually arise in a cutaneous nerve of the dermis or subcutis. In the majority of patients, solitary neurofibromas present as a slowly growing, painless mass. Histologically, solitary neurofibromas consist of a mixture of Schwann cells, perineurial cells and fibroblasts in a matrix of wavy collagenous fibers. In contrast to schwannomas, the cells are loosely arranged and diffusely infiltrate the involved nerve . On MRI,Angioleiomyomas are relatively common soft-tissue tumors that occur in the subcutaneous tissues of the extremities, particularly the lower leg . Pain isIn conclusion, a rare example of solitary schwannoma originating from the sural nerve in a middle-aged male patient has been described. Clinicians should consider schwannoma as a possible diagnosis for a well-defined, oval, subcutaneous mass in the posterior aspect of the lower leg.


Duplication of the lower urinary tract is a very rare congenital anomaly which is diagnosed either at birth or during early childhood. These rare malformations are most of the times accompanied by other concomitant anomalies and are therefore diagnosed immediately after birth. In some even rarer cases there are no concomitant anomalies and symptoms thus leading to a diagnosis later in childhood. This is the first case in the literature of complete bladder duplication in the coronal plane with concomitant duplication of the urethra and no other associated anomalies in a 52-year-old male who remained asymptomatic and therefore undiagnosed for more than 5 decades. A 52-year-old male was admitted to the urology department due to sudden purulent urethral secretion. He reported a painless progressively developing palpable mass anterior to the pubic symphysis which he first noticed 18 months prior to his admittance. He also reported that the mass had subsided after the appearance of the purulent exudate. The patient had no lower urinary tract symptoms. Clinical examination revealed a palpable painless mass anterior to the pubic symphysis, and after close inspection a second epispadic urethral meatus was observed. The patient was nonfebrile with normal blood count and biochemistry. The urine culture was sterile.A contrast-enhanced CT was performed that revealed a low-density 7 cm wide cystic mass anterior to the pubic symphysis, in the anatomic region of the suspensory ligament of the penis and in direct contact with the bladder . ConsistThe patient underwent an operation for removal of the mass. In front of the bladder and in direct contact with it, a large cystic mass was found . The masThe duplication of the lower urinary tract refers to the duplication of the bladder or/and the urethra. Abrahamson was the first to suggest a classification for bladder duplication . He clasDepending on the position of the accessory bladder in relation to the normal bladder, bladder duplication is also classified as sagittal or coronal. Bladder duplication in the sagittal plane is more common with the two bladders side by side, each receiving urine from one ureter . DuplicaOur case has several characteristics that make it unique. To our knowledge this is the 6th case of complete bladder duplication in the coronal plane with no other associated anomalies reported in literature. These cases are so uncommon because usually such malformations are accompanied by other congenital anomalies, urologic or not. Due to the common embryologic origin of lower urinary tract and lower intestines, gastrointestinal anomalies such as lower GI duplication, anorectal atresia, imperforate anus, and others are common. Other anomalies of the genital system are also present in numerous similar cases such as duplication of external genitalia like diphallus and duplication of the glans or bifid scrotum. Finally there are reports of several cases with concomitant vertebral anomalies like duplicate spine and spina bifida. In a review of all cases with bladder duplication  by Coker et al. , only 6 The most unique characteristic of our case is the age of our patient. The supernumerary bladder remained asymptomatic for more than 5 decades until an infection, probably due to the bladders' colonization from bacteria through the epispadic urethra, occurred and led to symptomatic disease. Another factor that contributed to the late diagnosis was the fact that the epispadic urethral meatus was very close to the normal meatus thus making it almost impossible to see. This proves that if left untreated at early childhood, this type of malformation can remain asymptomatic for many decades although there is always the possibility of becoming symptomatic probably due to infection.


Osteochondroma is a disease of growing bone and thus typically presents in younger patients. It has rarely been described in middle-aged and elderly patients. Data on the occurrence of osteochondroma show that the reported incidence of costal osteochondroma is very low. Moreover, costal osteochondroma arising at the costovertebral junction with neural foraminal extension and spinal cord compression is extremely rare.This study reports the case of a 58-year-old patient with a solitary osteochondroma of the 12th rib with intraspinal extension and spinal cord compression. The clinical history, plain radiographs, computed tomography (CT), magnetic resonance imaging, and pathologic findings of the reported patient have been reviewed. The relevant medical literature has also been reviewed. The patient was treated with surgery for complete tumour excision to avoid tumour recurrence. After surgery, the patient's symptoms improved. An additional CT scan obtained at 1 year after surgery did not show any evidence of recurrence.This patient is the oldest patient reported to have this rare form of costal osteochondroma. The age of the patient and the erosion of the adjacent bones raised clinical suspicion of malignancy; therefore, surgical management involved complete tumour excision with thoracolumbar fixation and fusion. Osteochondromas are the most common benign tumours of the bone and may present as either a solitary lesion or as multiple lesions ,2. SolitWe report a case that is unusual in many aspects such as the site of tumour origin within the rib; associated myelopathic symptoms; occurrence in a middle-aged patient; and the erosion and fusion of the vertebral pedicle, facet, and body due to tumour extension. Extension of the tumour necessitated total pediculectomy of T12, total facetectomy of T12-L1, and partial unilateral vertebrectomy of T12 with thoracolumbar fixation and fusion.A 58-year-old man presented with a 3-month history of right lower extremity weakness and numbness. He also reported progressive gait disturbance of 1-month duration. He denied bowel and bladder incontinence and sexual dysfunction. There was no medical or family history of neurologic dysfunction or bony tumour. Neurologic examination showed profound weakness, decreased pinprick sensation, and hyperreflexia of his right lower extremity. Right ankle clonus and positive Babinski response were also elicited.Plain radiographs of the lower thoracic and lumbar spine regions showed an exophytic bony mass on the right side of T12-L1 Figure . The difA posterior midline skin incision was used to expose the posterior elements of T11 to L1, and the exposure was extended to both ribs. After dissection, the large outer fragment of the multilobulated osseous mass was completely exposed Figure . This frPathologic examination of the tissues of the lesion showed proliferated hyaline cartilage but atypical changes indicative of myxoid degeneration were not observed. The histologic examination results indicated the strong possibility of osteochondroma, but low-grade chondrosarcoma could not be excluded because of the age of the patient and the erosion of adjacent bones Figure . After sThe reported incidence of costal osteochondroma is very low ,4. HowevOsteochondroma is a disease of growing bone, and thus typically presents in younger patients . Tumour Although the location of the bony portion of the osteochondroma can be reliably determined using multiplanar CT reconstruction, the exact size of the tumour may be underestimated because the cartilage cap of the tumour is not detectable by CT ,4. MR imMalignant transformation, usually into a chondrosarcoma, occurs in approximately 1% of solitary osteochondromas and 10% of HMEs ,12. A suThe type of osteochondroma described in this case, which arose at the costovertebral junction with neural foraminal extension and spinal cord compression, is extremely rare. Only 7 such cases have been previously reported in the English-language literature. This is the first report of this form of rare costal osteochondroma occurring in a middle-aged patient and requiring surgical management involving complete tumour excision with thoracolumbar fixation and fusion. Malignancy remained a possibility throughout because of the patient's age and erosion of adjacent bones.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.JH Shim provided major contributions to writing and editing the manuscript. CK Park supervised the writing of the manuscript. SH Shin contributed to analysis of the data. HS Jeong interpreted the radiologic results. JH Hwang contributed to conception and design of the data. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2474/13/57/prepub


Pituitary adenomas are associated with significant morbidity. The usual symptoms on presentation are of endocrine dysfunction and mass effects. A 31-year-old African female presented with headache, irregular menses, blurring of vision in the right eye and complete loss of vision in the left eye for 1 year. She had coarse facial features, enlarged hands and feet. Her right eye had temporal hemianopia with decreased visual acuity and her left eye had no perception of light. Investigations revealed an elevated fasting blood sugar and an elevated prolactin and growth hormone level. A CT scan and MRI done showed a hemorrhagic pituitary macroadenoma. She was put on bromocriptine, ocreotide, analgesics and insulin. Thereafter, she underwent transphenoidal surgery, where near total resection of the tumor was achieved. Patient is doing well post-operatively. This case highlights the importance of the use of a high clinical index of suspicion and radiological findings in diagnosis. Pituitary adenomas are the most common tumors of the sellar region. They are associated with significant morbidity, especially if they present with pituitary apoplexy. The usual symptoms on presentation are of endocrine dysfunction and mass effects. The documented endocrine dysfunction in this case was elevated prolactin levels and growth hormone levels resulting in acromegaly. On occasion, there is development of impaired glucose tolerance or secondary diabetes mellitus as was in this case. This occurs in only about 20% of acromegaly cases.A 31-year-old African female presented to the Accident and Emergency Department of Kenyatta National Hospital with the chief complaints of headache, irregular menses, blurring of vision in the right eye and complete loss of vision in the left eye for 1 year. She was well prior to the onset of symptoms. The headache started first followed by gradual loss of vision in the left eye which progressed to complete loss of vision in that eye. This was then followed by progressive decline of vision in the right eye. The headache and decline in vision has progressively worsened over the one year period, but she reported temporary relief when on her medication. Her medication included bromocriptine and ocreotide. She also had associated symptoms of fever and night sweats. She did not complain of any nausea or vomiting. Her last menses were 2 months ago. There was no other significant history. She does not smoke or drink alcohol and has no past medical history or family history of hypertension or diabetes mellitus.On examination, her vital signs were within normal. Of note, were her coarse facial features, enlarged hands and feet with sausage shaped fingers. Ocular exam of the right eye showed temporal hemianopia with decreased visual acuity. Examination of the left eye showed no perception of light. There were no other findings of significance.Investigations revealed an elevated prolactin level (Prolactin 1629uIU/ml), an elevated growth hormone level (Growth hormone 70.30ug/L) and an elevated fasting blood sugar (23.3mmol/L). Cortisol levels, thyroid hormone levels, thyroid stimulating hormone levels, urea, creatinine and electrolytes were all normal. HIV serology result was negative. Full blood count showed no evidence of infection. A CT scan and an MRI done both showed a suprasellar tumor with a cystic area suggestive of a hemorrhagic pituitary macroadenoma She was put on bromocriptine, ocreotide, analgesics and insulin, and once her blood sugars were controlled, she underwent transphenoidal surgery. Intra-operatively, after opening the dura of the sella turcica, brown (motor oil like) hematoma was encountered and evacuated. Tumor was brownish purple and soft in consistency. Near total resection of the tumor was achieved. Patient is doing well post-operatively.Pituitary adenomas are the most common tumors of the sellar region. They arise from epithelial pituitary cells and account for 10-15% of all intracranial tumors. Tumors exceeding 10 mm are defined as macroadenomas .In USA, pituitary tumors are found on autopsy in as many as 25% of unselected cases. The annual incidence of pituitary neoplasms varies from 1-7 cases per 100,000 population based on neurosurgical series. Morbidity results from mass effects, hormonal imbalance , and patient comorbidities. Significant morbidity is also associated with treatment of these tumors. No racial predilection exists for pituitary macroadenomas and there is an equal distribution of pituitary tumors between men and women. Corticotropinomas are an exception, occurring mainly in women, with a female-to-male ratio of 4:1. Tumors affect individuals of all ages, but incidence increases with age, peaking between the third and sixth decades of life [The usual symptoms on presentation are of endocrine dysfunction and mass effects but some patients may be asymptomatic. Symptoms of endocrine dysfunction depend on hormones involved. Hyperprolactinemia presents with hypogonadism, infertility, amenorrhea, and galactorrhea. Corticotropin excess presents with cushing disease and corticotropin suppression presents with glucocorticoid insufficiency. Thyrotropin excess presents with secondary hyperthyroidism and inadequate thyrotropin presents with secondary hypothyroidism . Excess Gonadotropinomas most often are asymptomatic but rarely, they can lead to testicular enlargement in men and ovarian hyperstimulation in women. Deficiency of gonadotropins presents with hypogonadism and infertility. Pituitary apoplexy is characterized by sudden onset of headache, visual symptoms, altered mental status and hormone dysfunction due to acute hemorrhage or infarction of the pituitary tumor. Mass effects of the macroadenoma may present with visual deficits, headache, elevated intracranial pressure, or intracranial hemorrhage .The diagnosis of a pituitary adenoma is based on clinical and radiological findings and is then confirmed by characteristic histological findings. The evaluation of a patient with a probable pituitary adenoma consists of: imaging, endocrine assessment, ophthalmology assessment and histology. On CT scan imaging, macroadenomas have variable appearances. Most are isoattenuating relative to the cortex on nonenhanced CT scans and show moderate enhancement on enhanced scans. Calcification is rare 1-8%). Necrosis, cyst formation, and hemorrhage may result in lesions of mixed attenuation. CT also shows bony changes and the mass lesion, with expansion of the sella turcica with or without erosion or depression of the sellar floor toward the sphenoid sinus. Secreting adenomas protrude or expand into the cavernous sinus more often than do nonsecreting macroadenomas . In summ-8%. NecrThe goal of treatment is complete cure. When this is not attainable, reducing tumor mass, restoring hormone function, and restoring normal vision are attempted using medications, surgery, and radiation. Pituitary macroadenomas often require surgical intervention for cure. The exceptions to this rule are the macroprolactinomas, which usually have an excellent response to medical therapy. Prolactin-secreting macroadenomas respond to dopaminergic agonists. The most frequently employed medications include bromocriptine, cabergoline, and, previously, pergolide. Quinagolide is an alternative with fewer adverse effects than bromocriptine. Prolactin-secreting macroadenomas are so responsive to medical therapy that surgery and radiation often are not used in treatment. Growth hormone-secreting tumors should be treated surgically, often followed by radiation therapy. Medical treatment is used after surgery to suppress growth hormone secretion, awaiting the occurrence of the effects of radiotherapy. Octreotide is the treatment of choice. A long-acting formulation administered monthly is now available. Somatostatin must be administered as a continuous infusion, while shorter-acting octreotide is administered tid-qid. Growth hormone receptor antagonists have been another addition to the treatment of acromegaly. Dopamine agonists also may be used but are not as effective as octreotide (approximately 30% of somatotropinomas respond) .Corticotropin-secreting pituitary tumors are treated using surgery and radiation therapy . Medical therapy is reserved for patients whose therapy fails, those who decline other therapy, and those who cannot be treated otherwise. Medical therapy is divided into centrally acting agents that reduce corticotropin release and peripherally acting agents that reduce cortisol secretion or block cortisol action. Centrally acting medications (unfortunately effective in very rare occasions only) include bromocriptine, valproic acid, and cyproheptadine. Peripherally acting agents include ketoconazole, mitotane, and metyrapone. Use of such medications should be in combination with radiotherapy. Gonadotropin-secreting macroadenomas are treated surgically, followed by radiation. Medical therapy is reserved for those patients who decline definitive treatment. Bromocriptine or octreotide may be used. LH-releasing hormone antagonists may decrease hormone levels but do not affect the tumor size. Nonsecretory macroadenomas are treated surgically. If surgery is contraindicated, octreotide or bromocriptine may be tried. Thyrotropin-secreting tumors are treated surgically, followed by radiation therapy. Octreotide is quite effective in such tumors and can be used as adjuvant therapy .Traditional radiotherapy using external beam radiation is used to complement surgery in inoperable cases or in patients declining surgery. The major drawbacks include delayed onset of action and high incidence of panhypopituitarism. Recent studies show the benefits of radiation . RadiosuPituitary macroadenomas often require surgical extirpation for cure. Transsphenoidal surgery is the approach of choice –9. Only In a study of 13 patients, Elhateer et al reported on the effectiveness of fractionated stereotactic radiation therapy (FSRT) in the treatment of macroadenomas. Based on an observational follow-up study  of 30 patients with pituitary macroadenomas (10 patients with functioning adenomas and 20 with nonfunctioning lesions) that were refractory to conventional surgical and/or medical treatment, Schalin-Jäntti et al also found FSRT to be a beneficial adjuvant therapy for these tumors.We present a case of a pituitary macroadenoma presenting with pituitary apoplexy, acromegaly and secondary diabetes mellitus. This case sparks an interesting discussion on the varied clinical presentation and management options. It also highlights the importance of the use of a high clinical index of suspicion and radiological findings in diagnosis.


Fused teeth may cause aesthetic, spacing, periodontal, eruption, and caries problems. The present case report describes a 7-year-old boy patient with a chief complaint of unerupted maxillary incisor. Radiographic examination indicated a fused tooth which had two fused roots but two independent root canals. A complex management of a fused tooth is really difficult to standardize. In this case an orthodontic, endodontic, and surgical treatment  allowed the tooth to be retained until 18 years following intervention. Maintenance of the root and alveolar bone in young adults at least until full skeletal maturation should be the main treatment objective. Dentition development is a very complex process either in the primary or in the permanent tooth. Some authors have proposed a model of genetic network regulation in tooth formation; it seems obvious that most human congenital malformations and dental defects are caused by mutations in developmental regulatory genes. There would be a molecular basis of dental defects .Fusion and gemination are considered abnormalities in tooth development. It is often difficult to differentiate between gemination and fusion and it was common to refer to these anomalies as “double teeth,” “double formations,” “joined teeth,” “fused teeth” or “dental twinning” –5.In gemination the subdivision of the tooth bud is incomplete, giving rise to two dental units, the width of which in the mesiodistal dimension can be twice the dimensions of a single dental unit usually sharing a single root, pulp chamber and root canal. This bifid tooth is considered as a single tooth , 7.By contrast, in fusion the originally separate tooth buds unite at the crown level (enamel) or at the crown and root levels (enamel and dentine), yielding a single large tooth during the odontogenesis, when the crown is not yet mineralized , 9.Both anomalies occur more frequently in the primary dentition, particularly in the canine-incisor region, involving maxillary central and lateral incisors and mandibular lateral incisors and canines . The incThe aetiology of fusion is still unknown, but the influence of pressure or physical forces producing close contact between two developing teeth was reported as a possible cause. Gemination can be interpreted as an attempt of a supernumerary tooth to form . Others This case report describes the treatment of a fused maxillary central incisor with a talon cusp using a multidisciplinary approach to manage and restore function and aesthetic appearance.In 1995, a 7-year-old boy was referred to Sant Joan de Déu Hospital complaining of unerupted maxillary right central incisor -tooth 11-. There was no significant past medical history nor family history of dental anomalies. The patient was in the first phase of mixed dentition stage. After radiographic examination, the preliminary diagnosis was a supernumerary tooth. Some months later, the incisor was present in the arch after a spontaneous eruption and the total number of teeth was normal. However, morphologically this central incisor showed macrodontia as a result of a fusion with a supernumerary tooth with a talon cusp . At thatThe molar relationship was a half unit Class II bilaterally. Moreover left side presented a severe osteodental discrepancy which made difficult the right lateral incisor eruption -tooth 12-.At the age of 10, the treatment plan was explained to the patient and his family. The main aim was to reduce mesiodistal tooth size—hemisection—in order to allow lateral incisor eruption. As there was a high risk of pulp exposure during odontosection, due to its fused roots, previously root canal treatment was performed.Under general anaesthesia a full-thickness buccal flap was reflected. After examining the outline and the position of the roots, the fused tooth was extracted and separated using a high-speed bur with water spray longitudinally throughout the root conjunction line. During this process gutta-percha was exposed and that moment it was decided to cover the root canal material with silver amalgam to avoid any filtration. The tooth's remaining portion was replanted into the socket and splinted to adjacent teeth with an orthodontic appliance. No orthodontic force was applied to it for 30 days .At the age of 13 orthodontic treatment for Class II malocclusion was initiated using an extraoral headgear and multibrackets (24 months). At the end of orthodontics a dental cosmetic treatment of these central and lateral incisors was indicated . At the Eleven years later, at the age of 28, the patient was attended at Dental Hospital in the University of Barcelona and his complain was a progressive pathological migration of the right central incisor in buccal direction. Clinical exploration confirmed the buccal migration, a grey discoloration, an incremented probing depth (11 mm) at the interproximal area with right lateral incisor, some pus exudation from this periodontal pocket, and no dental vitality of this lateral incisor . HoweverPeriapical radiograph showcased an apical image involving lateral right incisor and a severe vertical bone defect between right central and right lateral incisor, but the alveolar mesial bone level was preserved . Oral SuJust as important is the identification of the possible anatomic variations and different anomalies present in all tooth groups. One of these anomalies causing major difficulties for diagnosis and treatment is tooth fusion, which may be commonly confused with tooth gemination.Although the aetiology of these anomalies is still unknown, it is believed that some physical force or pressure/trauma causes the contact of developing teeth. Genetic predisposition and racial traits were also reported as contributing factors , 12.Clinically a fusion of a regular tooth and a supernumerary tooth may result in crowding, protrusion, or the impactation of an adjacent tooth owing to insufficient arch length . SeveralDens evaginatus is the malformation of a tooth characterized by the presence of an accessory cusp. Talon cusp refers to the same condition but it is manifested on anterior teeth [or teeth , 15, 16.or teeth , 17.Clearly, a careful clinical and radiographic examination is beneficial for optimal treatment planning. However, Computerized tomography (CT) has the potential to visualize the topography of the root canals, offers new perspectives for dental imaging for special clinical cases, and may confirm the exact path of the root canal –19.Several treatment methods are described in the literature with respect to the different types and morphologic variations of fused teeth . Case reThe treatment of fused teeth may be complex and contain various treatment protocols –24. The The success of intentional replantation, estimated as the tooth retention rate, is reported to be above 67% and up to 93% and seems to be linked to three factors: previously existing endodontics pathosis and chronic infection, the length of extraoral treatment, and type of splinting. The time from extraction to replantation and the preservation and handling methods of the tooth are probably crucial for maintaining vitality of the periodontal ligament .In our case, with the aim to avoid cracks on root and apical surface after tooth hemisection, the root planning was done with multifluted burs. At the end of this procedure, root must present a plane and smooth aspect, without degrees or irregularities that could act as irritants and stimulated root dentin resorption during periapical healing. Currently, the root-end cavity would be filled with Mineral Trioxide Aggregate (MTA). MTA is a biocompatible dental material which allows the proliferation of periodontal ligament cells, promotes an adequate sealing, and reduces apical leakage –30. HoweMaintenance of the tooth and the alveolar bone is nevertheless crucial in growing patients: both from the psychological point of view as well as for hard and soft tissue maintenance, which, in case of an unsuccessful replantation because of resorption and/or root ankylosis, may facilitate implant therapy in adulthood , 26. In This case report provides, after 18 years, relevant information to the clinicians because it has a considerable follow-up. Additionally it illustrates that the treatment of a double tooth may be really difficult to standardize. Preservation of teeth during the age of development, even with uncertain prognosis, appears crucial for maintenance of the anatomy of the alveolar process for eventual implant therapy in adulthood.


Sarcoidosis is a multisystemic disorder of unknown cause that affects almost every tissue in the body. Colon is an extremely rare location of this disease. Clinical presentation, endoscopic appearances, and radiologic findings are not specific and may mimic much other affection. We report the case of a 64-year-old woman with inactive pulmonary sarcoidosis who presented alternating constipation and diarrhea. Colonoscopy revealed a stenotic tumor in the ascending colon. Histology failed to determine the nature of the lesion. Radiologic findings are those of a long stenotic tumor of the ascending colon associated with a multiple satellite lymphadenopathy. Endoscopic and radiologic descriptions are highly suggestive of a malignancy. The patient underwent a laparotomy, and a right hemicolectomy was performed. Examination of the resected specimen showed follicular structure with central epitheloid and giant cells and surrounding fibroblasts. These findings made the diagnosis of colonic sarcoidosis. The nonspecificity of the endoscopic and radiological signs of gastrointestinal sarcoidosis and the extreme rarity of colonic location make the preoperative diagnosis unlikely. The diagnosis will be then made only on histological examination of surgical specimens. We describe, through this observation, the results of paraclinical investigations that can suggest diagnosis and perhaps avoid surgery.  Sarcoidosis is an inflammatory disease with unknown etiology. It is characterized by noncaseating granulomas in the absence of other autoimmune processes, infectious diseases, or foreign agents . It primA 64-year-old woman, with a 4-year case history of known pulmonary and cutaneous sarcoidosis, was admitted for abdominal pain, bloating and weight loss of nine-month duration.The diagnosis of sarcoidosis was made based on the histological findings obtained from a transbronchial lung biopsy specimen. She was treated with Prednisone. She has been in remission since two years, and she was observed without medication. Pulmonary disease remained inactive.The patient had developed diarrhea four months previously, which had seriously increased and often interrupted by episodes of obstinate constipation.Physical examination revealed tenderness of the right lower quadrant with a mal-limited deep mass. Pelvic exam was normal. Pulmonary and cutaneous exam was normal.Total colonoscopy revealed a stenotic tumor in the ascending colon .Biopsies from the tumor were taken, and histology of the specimens showed no evidence of neoplastic lesion, but an inflammatory infiltration of the submucosa which was rich of eosinophil cells.Contrast enema showed a long irregular stenotic lesion of the ascending colon .Abdominal ultrasound showed a 4 × 5 cm hypoechoic tumor in the ascending colon with no distant metastatic lesions.Abdominal computed tomography (CT) revealed a long stenotic tumor of the ascending colon extended 8 cm of length reducing notably the colonic lumen and associated with an infiltration of the adjacent fat tissues . There wThe laboratory data revealed white cell count of 10200, hemoglobin 12.5 g/dL, normal liver tests, and a normal level of serum electrolytes. The carcinoembryonic antigen level was 3.6 ng/mL.Relying on the endoscopic view and the scanographic appearance of the colonic lesion, and despite the short outcome of histology, the diagnosis of malignancy was made.The patient underwent an exploratory laparotomy, which revealed constricting lesion of 8 cm in the midascending colon with numerous adjacent lymphadenopathy. A carcinologic right hemicolectomy was performed.Examination of the resected specimen revealed a thickening wall of the mildascending colon causing a long stenosis. The mucosa was dotted with a multiple superficial ulcerations. In microscopic exam, the submucosa showed follicular structure with central epitheloid, giant cells, and surrounding fibroblasts . There i The subsequent evolution was favorable. The patient was discharged six days later.Sarcoidosis is a multisystemic disorder of unknown cause that is characterized by the formation of immune granulomas in involved organs.Sarcoidosis may affect almost every tissue in the body, but it possesses a predilection for certain structures so that a disease pattern is presented. The lungs and the lymphatic system are the main affected sites –8.Gastrointestinal tract is involved in less than 1% , 6, 8, 9For this location, literature is scanty. In a systemic revue of literature, Beniwal found 10 cases of colonic sarcoidosis from 1966 to 2003 [Clinical presentation is not specific, but the symptoms are those of a colonic disease. Indeed, patients were suffering from intermittent diarrhea, constipation, hematochezia, abdominal pain, distention, vomiting, and weight loss , 5–7, 10Endoscopic appearances are variable but not suggestive of the diagnosis, and prior diagnosis of malignancy creates more diagnostic uncertainty.It includes aphthous erosions or ulcers, friable mucosa or small punctuate bleeding sites miming colitis, plaque-like lesions, fold thickening, focal nodularity, or segmental narrowing, elevated lesions mimicking submucosal tumors –3, 7, 11However, histological evidence of sarcoid granules has been found in apparently normal mucosa , 3, 12 sIn biopsy specimens, the presence of noncaseating granulomas containing multinucleated giant cells with little inflammation or crypt abscess formation is the pathologic hallmark of this disease , 7, 8. TThe utility of serum angiotensin-converting enzyme (SACE) level in differentiating sarcoidosis from Crohn's disease has been reported .Serum ACE is thought to be produced by the epithelioid cells within sarcoid granulomas. It is elevated in approximately 60% of patients with sarcoidosis, and its level is thought to reflect whole-body granuloma mass and disease activity , 8.Mentions of colonic sarcoidosis and corresponding radiologic descriptions are few. CT may show segmental or a symmetric wall thickening with preserved wall stratification . These fIn case of appearance pseudotumor, if biopsy specimens fail to demonstrate the diagnosis of colonic sarcoidosis, surgical treatment with conventional surgical oncologic principle must be proposed for fear of carcinoma .If not and if sarcoidosis is symptomatic, it can be treated with systemic corticosteroids , 4.Colon is an uncommon site of gastrointestinal sarcoidosis. It should always be suspected for the tumor developing in colon in patients with the history of sarcoidosis especially when sarcoidosis lesions were found in several organs. The diagnosis is difficult to be made because of their high similarities in appearance with malignant tumor. The diagnosis should be suspected with compatible history patients if histology failed to show neoplastic proliferation. In these cases, only the presence of noncaseating granulomas will make the diagnosis. If not, patients should be treated as having a carcinoma.


Spindle cell lipoma of the hypopharynx is an extremely rare entity. Here, we present the first case of this lesion originated in the cricopharyngeal region, with symptoms of chronic progressive dysphagia, which can be confused with other pathologies; endoscopic and magnetic resonance imaging (MRI) evaluation are the methods of choice for its diagnostic approach. The best therapeutic approach is endoscopic resection with rapid recovery and few complications. Long-term followup is recommended, either endoscopic or imaging, given that it can be confused with an undiagnosed liposarcoma; additionally, its long-term behavior is unknown. Lipomas are mesenchymal tumors derived from mature adipocytes, characterized by their slow growth; in pharynx and hypopharynx, they have been reported as extremely rare, being 0.6% of all benign neoplasms, with a total of 80 cases reported in that location . They arThe histological variety of spindle cell lipoma is quite uncommon; being 1.5% of all lipomas, it is a tumor derived from prelipoblastic mesenchymal cells, pathogenesis is unknown, but recently cytogenetic changes have been found characterized by monosomy or partial loss of chromosome 13 and/or 16 . We present the case of a 66-year-old male patient with a condition of progressive dysphagia of five-year evolution, who two years ago presented a protrusion of mass in the oral cavity provoked by Valsalva maneuver or intra-abdominal pressure increase. The patient was referred to our institution with diagnosis of Zenker's diverticulum. During the first consultation, the protrusion of a well-defined elongated mass was confirmed via oral cavity with the Valsalva maneuver . A bariuWith the previous findings, it was decided to bring the patient to surgery for identification and extraction of the mass. Under general anesthesia, intraoperative esophagoscopy was performed with identification of the pedunculated mass dependent on the left cricopharyngeal region and extrusion by means of an endoscopic loop; the lesion was resected from its pedicle with harmonic scalpel Figures  and 3.The histological analysis of the lesion revealed a 6 × 2.5 × 1.5 mm dependent soft-tissue, polypoid circumscribed lesion, lined by stratified squamous epithelium without dysplasia, constituted by spindle cells without atypia and mature adipose tissue, without observing atypical mitosis . The immThe patient had satisfactory postoperatory evolution, with initiation of diet on the second day and discharged on the third day, without respiratory distress, dysphagia, or any other symptomatology. Endoscopic control was conducted one week later with evidence of a healthy scarred stump. Upon control one year after the procedure no lesion recurrence was registered.Spindle cell lipoma originated from the hypopharynx is an extremely rare entity, with the finding of four cases reported in the literature in that location. We present a case of spindle cell lipoma with symptomatology of long-standing dysphagia and as single data, with protrusion of such with the Valsalva maneuver, initially confused with Zenker's diverticulum. Macroscopically, these are sessile or pediculated masses of soft textures and well circumscription, which can be confused with other benign lesions, as in the case introduced. According to existing reports, these are described as slow-growing solitary masses, which originated in the hypopharynx and can present symptoms like dysphagia, dysphonia, stridor, foreign body sensation, or long-standing history of nonspecific symptoms ; cases hAmong the histological variants of lipomas, there are angiolipoma, angiomyolipoma, pleomorphic, benign lipoblastoma, fibrolipoma, chondrolipoma, and spindle cell lipomas; this last variety is characterized by presenting abundant mature adipocytes on a collagenous matrix with spindle cells and variable vascular patterns. They are differentiated from liposarcomas by the cellular uniformity and lack of lipoblasts and of cellular pleomorphism. Spindle cells are similar to fibroblasts, with elongated nucleus, which react to CD34. The adipocytes present in the spindle cell lipoma are of mature characteristics and although classically fat cells are S100-reactive, in this histological subtype reactivity is not present . Among the diagnostic means described for the assessment of the lesion, one of the fundamental pillars is the endoscopic visualization, finding a single pediculated lesion, coated with mucosa of normal appearance, of soft texture, and well circumscription, which in the case presented is inserted in the cricopharyngeal region. Although in this case the contrasted neck CT scan did not contribute much information, cases published report that means of diagnosis may offer an approximation of the extension of the lesion and reveal if there is or is not infiltrative behavior toward deep tissue, as well as the presence of lymphadenopathy suspicious of malignity; however, upon treating a dependent soft tissue lesion, the MRI has better definition in the evaluation of soft tissue and its extension to adjacent structures and should be considered if doubts emerge regarding the lesion . Treatment of hypopharynx spindle cell lipoma consists in the radical excision of the lesion, ideally under endoscopic vision, given that because of its nature and location with this approach its complete resection is possible, with rapid recovery not exceeding three days, as shown in the case presented, without report to date of complications with this method , 9. Endo


Although hypodontia and supernumerary teeth are often considered as mutually exclusive conditions, this case report presents an unusual case of hypodontia and a supernumerary tooth occurring simultaneously. An adolescent male was referred to the local hospital department regarding upper arch crowding. Plain film radiographs confirmed the congenital absence of both lower lateral incisors in addition to an unerupted conical supernumerary tooth in the maxillary midline. This condition has been called hypo-hyperdontia and in this paper, we discuss the clinical findings and treatment planning considerations in relation to the limited number of previously reported cases. The case report raises awareness of concomitant hypo-hyperdontia and serves to highlight that concomitant anomalies should be excluded when hypodontia or supernumerary teeth are diagnosed. Hypodontia and supernumerary teeth are often considered as two extremes of the same type of dental anomaly. They most commonly occur as mutually exclusive conditions, and each represents its own challenge in terms of treatment planning. On very rare occasions, hypodontia and supernumerary teeth can coexist with the condition termed “concomitant hypodontia and hyperdontia” , “oligopAn 11-year-old, white male was referred to the Department of Child Dental Health, at the local University Dental Hospital for an orthodontic opinion from his general dental practitioner. The medical, dental, and social histories were unremarkable.The patient presented with a class I skeletal relationship with an average Frankfort-mandibular planes angle and lower facial height proportion. There was no significant asymmetry in the transverse plane. The lips were competent, and the nasolabial angle was slightly reduced. An assessment of the temporomandibular joint was unremarkable.In occlusion the patient had a mild class II division 2 incisor relationship in the mixed dentition, with an overjet of 2 mm and an increased, complete to tooth overbite . The uppThe lower arch was essentially well aligned; however, both lower permanent lateral incisors were clinically absent .The upper arch was severely crowded with a lack of space for both upper permanent canines . The patient's oral hygiene was fair with small amounts of plaque being present in the cervical margins of the posterior dentition. There was occlusal fissure caries present in the lower first permanent molars.A dental pantomogram  revealedThe clinical and radiographic findings were explained to the patient and parents. Oral hygiene instruction, diet advice, and tooth brushing instruction were given. Bite-wing radiographs followed by preventive resin restorations were planned for all four first permanent molars.Given the reasonable lower arch alignment and aesthetics despite congenitally absent lower lateral incisors, the lower arch was to be treated on a nonextraction basis. Removal of the maxillary first premolars was requested to allow spontaneous eruption of the maxillary permanent canines. There was a discussion with regard to the proximity of the upper midline supernumerary and the roots of the upper incisors. If fixed appliance therapy was to be considered, then the supernumerary would most likely require removal. As the patient and parents were apprehensive regarding a general anaesthetic, the decision was made to review the interarch relationships in a 6-month time and confirm active treatment or accept the malocclusion with regular monitoring of the upper incisor roots and supernumerary.MSX1, TGFA, and PAX9 mutations isolated for tooth agenesis [The authors have presented a case of CHH in a nonsyndromic patient. The incidence of such a condition in the general population is unknown but is likely to be very rare given the incidence in an orthodontic population has been documented as low as 0.3% . The aetagenesis . More reagenesis . The aetagenesis . Despiteagenesis . From thagenesis . In addiagenesis .Whilst hypodontia is more common amongst females and supernumerary amongst males, CHH has been reported to affect both genders equally .One case of CHH has reported that a mesiodens was found in conjunction with a missing maxillary lateral incisor . The aetFortunately in the case presented here, the diagnosis of CHH does not seem to have impacted significantly on the treatment plan. Although the supernumerary present was a classic feature of an additional tooth to the normal series, the pattern of hypodontia was not typical. Isolated incidences of congenitally absent mandibular incisors are believed to be in the region of 0.08–0.23% , and theThe body of knowledge on CHH is steadily growing. Literature reviews and case series have helped to recognise a condition which was once thought of as two opposite entities. This case report highlights a routine orthodontic referral presenting with simultaneous occurrence of hypodontia and a supernumerary tooth. Practitioners should be aware that hypodontia and supernumerary teeth may not be mutually exclusive in their presentation.


Molluscum contagiosum is a cutaneous viral infection presenting as multiple, umbilicated papules and vesicles. The cytology of molluscum contagiosum in an 11-year-old boy, which presented atypically as a solitary nodule over the right cheek, mimicking a skin adnexal tumor is reported here. Fine needle aspiration cytology plays a vital role in establishing the correct diagnosis of clinically unsuspected cases, and hence, the proper management of such lesions. The cytology of molluscum contagiosum is characterized by the presence of numerous large intracytoplasmic basophilic bodies that push the host nucleus to the periphery, giving a signet ring appearance to the superficial epidermal cells. Molluscum contagiosum is an infectious disease of human skin, characterized by the formation of multiple and discrete cutaneous epithelial nodules averaging about 2 millimetres in diameter.An unusual case of molluscum contagiosum presenting as a clinically solitary solid nodule diagnosed by fine needle aspiration cytology is reported here.An 11-year-old boy presented with a solitary nodule over the right cheek which had been there for the last six months. Local examination revealed a 8×6 mm solid, firm, non tender and immobile swelling over the right cheek, below the lower eyelid.The general physical and systemic examinations were normal. The routine investigations and the blood serology were within normal limits. Patient was immunocompetent. A skin adnexal tumor was suspected clinically.Fine needle aspiration of the right cheek nodule was done using a 24-gauge needle and 10 ml syringe, and it provided a scanty aspirate. The smears were stained with hematoxylin-eosin (H and E) and May-Grünwald-Giemsa (MGG) stains. The cytology of the smears revealed numerous benign squamous cells, with granular intracytoplasmic eosinophilic inclusion bodies, pushing the nucleus to the periphery . The cytMolluscum contagiosum was first described as a clinical entity in 1871 by Bateman.Molluscum contagiosum is a cutaneous infection produced by molluscum contagiosum virus, specific to humans, present world wide and passed by direct skin-to-skin contact. It affecMolluscum contagiosum clinically presents as small, firm, smooth surfaced, dome shaped pearly papules averaging 3-5 mm in diameter, with a central umbilication, distributed over face, trunk and extremities. Atypical presentations like cellulitis, abscesses, molluscum contagiosum with metaplastic ossification and giant molluscum contagiosum are reported.–6The characteristic cytologic feature of molluscum contagiosum is the presence of molluscum bodies, as described by Handerson and Pattersonin 1891, in the enlarged superficial cells of the epidermis. MolluscuSpecial stains like phosphotungstic acid-hematoxylin preparation and carbol-anilin-fuchsin after mordanting with potassium permanganate are used to demonstrate molluscum bodies.Clinical differential diagnoses of molluscum contagiosum include folliculitis, warts, cryptococcosis, nevi and skin adnexal tumor like syringoma, basal cell epithelioma, keratoacanthoma. A cytoloMolluscum contagiosum presenting as a solitary solid nodule is unusual. In such unsuspected cases, the cytological diagnosis of molluscum contagiosum is suggested by demonstrating the characteristic molluscum bodies in the aspirated material.