Members of the Ewing’s sarcoma family of tumor (ESFT) are malignant neoplasms and rarely observed in the adrenal gland.We report an extremely exceptional case of ESFT rising from the adrenal gland in a 57-year-old Chinese man. The patient was hospitalized with abdominal swelling for 2 months. Computed tomography (CT) scan revealed a nearly-circular mass measuring about 8.1 × 10.6 cm in the right adrenal region. The patient underwent right adrenal resection. Histopathologic examination found the tumor was composed of small round blue cells forming typical Homer-Wright rosettes in focal area. The immunohistochemical analysis confirmed the case to be ESFT, which was positive for membranous CD99 and nuclear FLI-1. The patient was scheduled for four courses of large doses of chemotherapy and died for cancer metastasis one year later after surgery.Histopathological evidence of Homer-Wright rosettes and immunohistochemical markers positivity, such as CD99 and FLI-1, are valuable factors for ESFT diagnosis, although cytogenetic analysis is considered as the gold standard. Complete surgery is the treatment of choice for ESFT and adjuvant radiotherapy and combination chemotherapy can significantly improve the survival rate of postoperative patients. The Ewing’s sarcoma family of tumor (ESFT) are rare aggressive malignancies and consist of Ewing’s sarcoma (ES) of bone, extraosseous Ewing’s, primitive neuroectodermal tumor (PNET), and Askin’s tumor , 2. ThesA 57-year-old man presented to the First Hospital of Jilin University  with the main complaint of abdominal swelling for 2 months. In addition to the mild percussion pain in the right kidney region, no other symptoms were noted during a physical examination. His past medical history was unremarkable. Computed tomography (CT) scan of the abdomen revealed a nearly-circular mass measuring about 8.1 × 10.6 cm arising from the right adrenal gland Fig. . The CT The patient underwent open surgery under general anesthesia. A 10.0 cm × 8.0 cm × 6.0 cm mass was found during laparotomy. The tumor was located above the left renal vein and the right renal vein without venous involvement. Due to firmly adhesion with the surrounding tissue, tumor dissection was difficult. Intraoperative blood loss was 800 mL and the tumor was completely removed eventually. Postoperative histopathology showed a monotonous population of small round blue cells with occasional Homer-Wright-type rosettes Fig. . The resThe patient was scheduled for adjuvant chemotherapy with adriamycin, cyclophosphamide, ifosfamide and etoposide. At his follow-up, 5 months after surgery, CT scan results demonstrated a metastatic lesion arising from the right abdominal wall. Unfortunately, the patient died for cancer metastasis one year later after surgery.ESFT rising from the adrenal gland is extremely exceptional but malignant. Patients often present with tumor compression, flank pain or mass. However, its preoperative imaging diagnosis is difficult and histopathological and genetic tools are required for an accurate diagnosis.Histopathologically, ESFT appear as immature or primitive small round blue cell tumors infiltrating the soft tissue or bone in a diffuse or lobular pattern. The tumor cells have round to oval nuclei with coarsely stippled chromatin and indistinct nucleoli. The scanty cytoplasm is pale or clear. In addition, these cells are often accompanied by hemorrhage and necrosis. ESFT are mainly represented by the existence of typical Homer-Wright-type rosette or other types of rosettes , 31.Immunohistochemical markers such as CD99, FLI-1, HNK-1 and CAV-1 are commonly expressed in ESFT and provide valuable support to the definitive diagnosis. CD99, a 32-kDa cell surface glycoprotein, is encoded by the MIC2 gene and extremely sensitive for ESFT , 14. TheAt present, cytogenetic analysis is the “gold standard” for diagnosis of ESFT. Conventional tests are valuable to make the definitive diagnosis such as Southern blot, Northern blot analyses, FISH and RT-PCR , 39, 42.ESFT is an aggressive malignancy with very poor prognosis . MultimoCooperative group studies have led to chemotherapy regimens using the same drugs , although the exact regimens differ in Europe and North America . Only 16ESFT rising from the adrenal gland is a rare clinical entity. Histopathological evidence of Homer-Wright is crucial for ESFT diagnosis. The neural markers, such as CD99, FLI-1, HNK-1 and CAV-1, may play a valuable role in the immunohistochemical diagnosis of ESFT. The definitive diagnosis of ESFT requires a combination of immunohistochemical examination, as well as histopathologic evaluation, although the “gold standard” will obviously remain cytogenetic analysis. Complete surgery is the treatment of choice for ESFT. Adjuvant chemotherapy and postoperative radiotherapy have shown significant improvements in survival. The tumor size and metastases are predictors for survival and effect prognosis obviously.


Larger insulinomas are reportedly more likely to be malignant; however, their biological behavior has not been clearly elucidated. We here report the characteristics and treatment of a giant insulinoma with local invasion and lymph node metastasis. We also review published reports concerning the clinical features of giant insulinomas and comparing their grading with that of pancreatic neuroendocrine tumors.A 71-year-old man was referred to our hospital for investigation of persistent hypoglycemia. On the current presentation, laboratory tests showed serum glucose, immunoreactive insulin, and C peptide concentrations of 45 mg/dL, 17.2 μIU/mL and 4.1 ng/mL, respectively. Dynamic magnetic resonance imaging showed a hypervascular tumor measuring 13.5 cm in the head of the pancreas. Computed tomography scanning demonstrated local invasion and lymph node involvement. He thus had Whipple’s triad, which is associated with malignant insulinoma. No distant metastases having been identified, pancreaticoduodenectomy was performed. Intraoperatively, three separate tumors were identified in the pancreatic head. Pathological examination showed all three tumors were pancreatic neuroendocrine tumors; the tumor cells in the largest mass were strongly immunoreactive for insulin. The Ki-67 index was 2–5% in most parts of the largest tumor and over 20% in the poorly differentiated areas. This tumor was classified as neuroendocrine carcinoma in accordance with the 2010 World Health Organization classification of pancreatic endocrine neoplasms. He remains free of evidence of recurrence 2 years postsurgery.A review of published reports indicated that giant insulinomas are more malignant than smaller ones, and metastatic disease is found on presentation in 56% of patients with giant insulinomas; however, we were unable to identify any correlation between grade of pancreatic neuroendocrine tumor and biological behavior of giant insulinomas.Giant insulinomas more frequently exhibit malignant behavior, such as local invasion, lymph node involvement, and liver metastasis, than smaller ones. However, there was no relationship between grade and rate of metastases or survival in this small case series. Identification of useful biological markers is necessary. Insulinomas are the commonest functioning pancreatic neuroendocrine tumor (pNET); their estimated annual incidence being one to three cases per million . ApproxiLarger insulinomas are more likely to be malignant ; howeverA 71-year-old man was referred to our hospital for investigation of hypoglycemia. Two years before, he had attended the emergency department with acute-onset loss of consciousness after a traffic accident. Hypoglycemia was identified as the cause; however, because his condition had quickly improved, he was not extensively investigated and was discharged without a definitive diagnosis. Since then, he had recurrent hypoglycemia associated with impaired consciousness. In the four preceding months, his serum glucose concentrations had been less than 67 mg/dL and he had been given intravenous glucose, which resulted in gradual resolution of symptoms.On this presentation, his serum glucose, immunoreactive insulin and C peptide concentrations were 45 mg/dL, 17.2 μIU/mL, and 4.1 ng/mL, respectively. MEN-1 syndrome was excluded by normal serum intact parathyroid hormone, calcium, and prolactin concentrations. He thus had Whipple’s triad . To confirm the diagnosis of endogenous hyperinsulinemia caused by insulinoma, the patient underwent a supervised overnight fast during which he developed symptomatic hypoglycemia with serum glucose of 50 mg/dL.Dynamic magnetic resonance imaging showed a 13.5 cm × 12 cm × 8 cm hypervascular tumor containing necrotic areas in the pancreatic head , and there was a slight male preponderance . Those patterns are similar to those of non-giant insulinomas.n = 22); involvement of both the body and tail was seen in 12 patients. Giant tumors occur more frequently in the body and tail of the pancreas because they can grow freely there without causing mass effects or mechanical obstruction [Tumor location within the pancreas was available for 42 patients. Tumors in a single segment of the pancreas had a predilection for the tail  of all cases studied; the procedures comprising distal pancreatectomy (combined with resection or ablation of hepatic metastases) in 23 (4) cases, pancreaticoduodenectomy in 3, tumor resection in 5, and total pancreatectomy in 1 case. No patient had undergone combined resection and reconstruction of the portal vein or hepatic artery. Complete resection should be performed; however, removing 90% of the tumor is acceptable. Surgery is justified for functional advanced neuroendocrine tumors, the aims being to control symptoms, improve quality of life, and extend survival rate compared with conservative treatment . Seven oThe diameter of these patient’s tumors ranged between 9 and 21 cm . Most insulinomas become symptomatic when very small, allowing early detection and prompt surgical treatment, possibly before they can metastasize. The severity of hypoglycemia varies from patient to patient and has no direct relationship with tumor burden . SugiyamTwenty-five (55.6%) of the cases of giant insulinoma reviewed had metastases at presentation, this percentage being higher than the 10% reported in most insulinoma series . At the Follow-up data were available for 41 of the published cases. Duration of follow-up of 24 cases who had no recurrences ranged between 3 months and 23 years (median 18 months). Seven cases of the 11 fatal cases were reported before 1980. The overall survival of all patients who died with disease ranged between 1 month and 13 years , whereas patients of non-giant malignant insulinoma with distant metastases had a median survival of <2 years [Since 2001, the mitotic activity, Ki-67 index, and grade have been reported for 17 patients with giant insulinomas (Table Giant insulinomas more frequently exhibit malignant behavior, such as local invasion, lymph node involvement, and liver metastasis, than smaller ones. Metastatic disease is present at surgery in 56% of cases of giant insulinoma. Unexpectedly, there was no relationship between grade of pNET and rate of metastases or survival in this small case series. Identification of useful biological markers is necessary, as is development of anti-tumor agents for liver metastases.


The association of neonatal intestinal obstruction with situs inversus totalis is extremely rare with only few cases reported in the literature to date. This association poses dilemmas in management. We present two such cases (of Indian origin), and briefly discuss the pertinent literature and measures to prevent unfavorable outcome.Case 1: a 1-month-old preterm (1300 g) male neonate belonging to Hindu (Indian) ethnicity presented with recurrent bile-stained vomiting, non-passage of stools and epigastric fullness. A babygram and upper gastrointestinal contrast studies revealed situs inversus and suggested proximal jejunal obstruction with midgut volvulus. Exploration confirmed situs inversus totalis along with reverse rotation and midgut volvulus. There was a small gangrenous area in the proximal jejunal loop. A Ladd’s procedure, resection of the gangrenous jejunal loop, and jejunojejunal anastomosis was performed. Note was made of the unusual appearance of the intestines suggestive either of fibrous or fatty infiltration. Postoperatively, our patient developed septicemia and died.Case 2: a 4-day-old female neonate belonging to Hindu (Indian) ethnicity, small (1320 g) for gestation, presented with history of non-passage of meconium since birth, refusal to accept feeds, and episodes of recurrent bilious vomiting with abdominal distension. A plain radiogram revealed situs inversus and proximal jejunal obstruction. Ultrasonography of her abdomen revealed renal dysplastic changes in both her kidneys. Laparotomy confirmed multiple jejunoileal atresias with situs inversus totalis. Resection anastomoses was performed for multiple atresias. Our patient passed a few pellets of meconium stools postoperatively; feeds were started gradually on the sixth day. Our patient gradually developed oliguria and renal failure, followed by respiratory distress and generalized edema requiring ventilatory support. She died later due to multiorgan failure.Clinicians should have high index of suspicion for malrotation with midgut volvulus or intestinal atresias in neonates of situs inversus presenting with bilious vomiting. The surgical treatment should follow the same surgical principles. In situs inversus, because of transposition of viscera, midgut volvulus may occur in an anticlockwise direction, hence derotation is performed clockwise. Prognosis was poor in our series because of low birth weight, late presentation, presence of gangrenous locus in the small bowel and development of septicemia in our first case and multiorgan fibrosis/dysplasia in our second case. Early diagnosis and timely referral is paramount for favorable outcome. Situs inversus is a rare condition 1 in 8,500 people) causing mirror image positioning of thoracic and abdominal organs . Situs i00 people3, a hemoglobin level of 11.6 g, and raised C-reactive protein levels. A babygram revealed dextrocardia, liver opacity on the left side of his abdomen, a splenic shadow and stomach bubble on the right side, which was suggestive of situs inversus totalis. There were few dilated bowel loops in his upper abdomen with a paucity of distal gas shadows , male neonate belonging to Hindu (Indian) ethnicity, presented to our hospital with recurrent bile-stained vomiting, non-passage of stools and epigastric fullness for the last 2 weeks. Antenatal ultrasounds had not been done. On examination, the neonate was afebrile, moderately dehydrated with a pulse rate of 170 beats per minute and a respiratory rate of 62 breaths per minute. Chest auscultation revealed cardiac apex on the fifth right intercostal space along the midclavicular line. The abdomen was soft and distended and nasogastric aspirate was bilious. Laboratory investigations revealed a total lymphocyte count (TLC) of 16,200 mmows Fig. . Upper gows Fig. . Preoperows Fig. . There wth postoperative day revealed infiltration of the lung fields  ethnicity, full-term small (1320 g) for gestation, presented with non-passage of meconium since birth, refusal to accept feeds, and episodes of recurrent bilious vomiting with abdominal distension. There was no history of consanguinity. On examination, the neonate was hemodynamically stable, dehydrated, and mildly icteric with a pulse rate of 162 beats per minute and respiratory rate of 50 breaths per minute. There was upper abdominal distension with visible dilated bowel loops. Plain radiographs revealed dextrocardia, liver opacity on the left side of her abdomen, and a stomach bubble on the right side suggestive of situs inversus totalis. There were few dilated bowel loops in her upper abdomen with absence of distal gas shadows Fig. . Laboratlds Fig. , which wSitus inversus is a congenial anomaly with inverted position or transposition of internal organs from their normal anatomical position . It is aHeterotaxy syndrome or situs ambiguous (4 per 1 million live births) is defined as the abnormal arrangement of organs and vessels as opposed to the orderly arrangement in situs solitus or situs inversus . It has Etiologic factors implicated in situs inversus are autosomal recessive gene, maternal diabetes, cocaine abuse, retinoic acid exposure, conjoined twinning, consanguinity and familial (multiple inheritance patterns) –3, 12. TSitus inversus presenting in the neonatal period with gastrointestinal complaints is typically an abdominal emergency . The diaEchocardiography is important to evaluate CHD. It could not be performed due to resource limitations in our series. An easy way of predicting the likelihood of CHD in a neonate with a situs abnormality (excluding situs ambiguous) is to observe the laterality of stomach and cardiac apex. If the both are on the same side of the body, the risk of associated CHD is relatively low, whereas if they are on opposite sides, the risk is relatively high. Thus, severity of CHD is proportional to the failure of cardiac shift with respect to the cardiac situs within the thorax . CHD is The association of neonatal intestinal obstruction  with situs inversus abdominus is rare, but its association with situs inversus totalis is extremely rare , 9, 16.Tet al. [Gastrointestinal anomalies associated with situs inversus are duodenal atresia, annular pancreas, biliary atresia, preduodenal portal vein, diaphragmatic hernia, lung cyst, genitourinary anomalies, ear, eye, and vertebral defects . Associaet al. . This syet al. –20. AnteThe treatment of neonatal intestinal obstruction associated with situs inversus totalis should follow the same surgical principles as performed for classical cases. Appropriate incision placement is required. Elective intervention is always better tolerated than an emergency surgery . The claIn our series, unfavorable outcome in our first case was due to a combination of factors, like late presentation, low birth weight, presence of gangrenous locus in the small bowel, superimposed by septicemia. Thus, early diagnosis and timely referral along with strict asepsis is paramount for a favorable outcome. In our second case, we believe that, because of the presence of other severe anomalies, the condition was incompatible with life and further research is required to come to any final conclusion.Clinicians should have high index of suspicion for neonatal intestinal obstruction in a situs inversus patient presenting with bilious vomiting. In contrast, clinicians should look for situs anomaly when viewing plain radiographs of pediatric patients with malrotation or jejunoileal atresias. The surgical treatment should follow the same surgical principles as performed for classical cases. In situs inversus, because of the transposition of viscera, midgut volvulus may occur in an anticlockwise direction, hence derotation is performed clockwise. Prognosis was poor in our series because of low birth weight, late presentation, presence of gangrenous locus in the small bowel, and development of septicemia in our first case and multiorgan fibrosis/dysplasia in our second case. Early diagnosis and timely referral is paramount for a favorable outcome.


Glandular Odontogenic Cyst is a relatively rare cyst of odontogenic origin, which shows glandular or salivary features that are thought to indicate the pluripotentiality of odontogenic epithelium. It is seen in middle-aged adults, and commonly involves the anterior region of the jaws, especially the mandible. It shows non-specific clinico-radiographic findings which may resemble other lesions, but has characteristic histopathologic features which help in its diagnosis. This paper reports an unusual presentation of a glandular odontogenic cyst which was diagnosed in a 64-year old female in the posterior maxilla, along with a literature review of this cyst, especially the cases reported in India in the past. It was initially described by Padayachee and van Wyk in nd ducts . In 1992nd ducts . An impond ducts . This caA 64-year-old female patient reported to our out-patient department with a chief complaint of swelling over the left side of the face since two months. History revealed that the swelling was initially small in size and had gradually increased to its present size. On extra-oral examination, a diffuse swelling was seen over the left cheek region, extending from the left nasolabial fold area, posteriorly to the malar region and supero-inferiorly from 2 cms below the infra-orbital margin to 1 cm away from the left angle of mouth, measuring around 3 x 2.5 cms. The overlying skin was normal with no evidence of any discharge. On palpation, the swelling was firm in consistency, tender, non-pulsatile and non-compressible, with no local rise in temperature. Intra-oral examination revealed a diffuse swelling in the left maxilla and buccal vestibule, extending from 23 till the 26 region . The sweBased on the history, clinical and radiographic findings, a provisional diagnosis of a residual cyst was made, with a differential diagnosis of an odontogenic cyst or tumor. The lesion was treated conservatively with careful enucleation and curettage. Histopathological examination showed a cystic lining with a pseudoglandular pattern in some areas, with other areas showing a pseudostratified columnar ciliated epithelium with goblet cells, some mucous pools / crypts . The conGOC is a rare lesion with a frequency rate of only 0.012% to 1.3% of all the jaw cysts . Till daHistologically, GOC shows certain characteristic features that were subdivided by Kaplan et al into major and minor criteria based on the frequency of occurrence of each feature in the previously reported cases . At leasGOC is an uncommon cystic lesion, with less than 200 cases reported world-wide till date. This cyst shows non-specific clinico-radiographic characteristics which may resemble a wide-spectrum of lesions. A final diagnosis can only be obtained after a careful histopathologic examination and a long-term follow-up is required to rule out recurrences.


Lymphangiomas are uncommon benign lesions of lymphatic vessels very rarely affecting the spleen. Isolated involvement of the spleen in adult patients is rarely reported.We report a case of a 40-year-old Arabic woman who presented with a 25-cm abdominal mass, fatigue, and anemia evolving for 6 months. Her physical examination revealed anemic syndrome and an enormous splenomegaly extending beyond the umbilical area. An abdominal computed tomographic scan showed a 25-cm splenic mass with multiple hypodense nodules without enhancement after contrast injection. A surgical total splenectomy was performed. Histopathological analysis led to the diagnosis of cystic splenic lymphangioma. The patient’s postoperative course was uneventful, and she was discharged from the hospital.Isolated splenic lymphangioma in adult patients is very rare. The preoperative diagnosis is challenging because imaging techniques are not specific. Pathological analysis of the resected specimen is the only effective way to render the definitive diagnosis. Splenic lymphangiomas have a benign course after complete surgical resection. Splenic0%; they terature –3. The dterature , 4, 5. T% and axiterature , 2. Adeqterature –3. In th3, and a white blood cell count of 6110/mm3. The results of other tests were within normal limits. A thoracoabdominal CT scan was obtained, which revealed a splenic mass of 25 cm in larger diameter with regular contours. This mass was made up of many hypodense nodules with variable size and showed no enhancement after contrast injection. The mass was limited to the spleen and pushed the stomach without invasion. No other mass was detected elsewhere. The radiologist suggested the diagnosis of a splenic lymphoma. The result of a bone marrow biopsy was normal at histopathological examination. A surgical total splenectomy was performed.A 40-year-old Arabic woman was referred to our hospital for evaluation of a huge splenomegaly causing abdominal pain and shortness of breath. She reported progressive abdominal distension evolving for 6 months, with a recent onset of dyspnea and asthenia. She had consulted a doctor who had prescribed her an abdominal computed tomographic (CT) scan, which revealed a huge splenomegaly of around 25 cm. Her medical history was unremarkable. A physical examination revealed skin pallor and a significant abdominal distension with giant splenomegaly that extended beyond the umbilical area. The patient had no other organomegaly, adenopathy, or skin lesions. Laboratory explorations revealed a microcytic hypochromic anemia with 7.1 g/dl hemoglobin, thrombocytopenia with 98,000 platelets/mmMacroscopic analysis of the resected spleen showed a giant spleen mass measuring 27 × 23 × 7 cm replacing almost the entire normal parenchyma, with a small residual normal parenchyma  are the main histological differential diagnoses [In the vast majority of the reported cases, a diagnostic splenectomy was performed, and the pathological examination provided the definitive diagnosis , 9–12. Tiagnoses –16.Hemangiomas are vascular tumors showing dilated vessels lined by endothelial cells whose lumina are extensively filled with red blood cells, in contrast to amorphous proteinaceous material seen in lymphangiomas. Also, D2-40 immunostaining is negative in endothelial cells of hemangiomas, even though myoepithelial cells are positive for this marker . MesotheSplenic lymphangiomas have a benign course, even though rare cases with sarcomatous transformation have been reported . CompletLymphangiomas are rare benign tumors that often present with multiorgan involvement in children. The occurrence of lymphangiomas as isolated lesions of the spleen in adult patients is extremely uncommon. The preoperative diagnosis in this context is misleading, and splenectomy for therapeutic and diagnostic purposes is the only appropriate management.


Intracranial chondromas of the dural convexity are exceedingly rare with less than 30 reported in the literature to date. We report a massive intradural convexity chondroma in a patient initially thought to have a frontal gait neurodegenerative disorder. This large tumor required a complex, piecemeal surgical resection due to the dense, fibrous nature of the tumor and the proximity of crucial structures. The patient had complete resolution of her preoperative symptoms after surgical excision. Intracranial chondromas are benign, slow-growing neoplasms of mature hyaline cartilage. They occur intracranially in rare cases with an estimated incidence of 0.2% to 0.5% of all intracranial neoplasms . Most coHere, we present a rare case of a massive intradural convexity chondroma in a patient thought to have a frontal gait neurodegenerative disorder. We also discuss the relevant literature on this rare intracranial tumor. To our knowledge, this is the first report of a dural convexity chondroma that manifested as lower body Parkinsonism  .PresentationA 54-year-old, right-handed female presented with progressive difficulty ambulating, worsening cognition, and left hand tremor. She had been using a front wheeled walker to ambulate for two years. At the point of presentation to our clinic, she was wheelchair-bound and unable to ambulate. She was referred to the movement disorder clinic, where her gait was described as primarily lower body Parkinsonism with akinesia, shuffling gait, and frequent gait freezing. With the exception of a small left hand tremor, she had no upper extremity symptoms. She was suspected to have a frontal gait neurodegenerative disorder possibly due to vascular parkinsonism or normal pressure hydrocephalus. As a part of her initial workup, she received a magnetic resonance imaging (MRI) scan with contrast, which showed a large (8.5 x 5.5 x 4.5 cm), heterogeneously contrast-enhancing lesion Figure , 1B. ThiSurgeryA trapdoor incision was made on the patient's left side and crossed the midline. This was necessary given the large extent of the tumor, including to the contralateral side past the SSS Figure . Prior tHistopathologyGross inspection revealed a firm-to-hard, white, homogenous, smooth-surfaced tissue. Histologic sections showed a homogeneous, cytologically-bland cartilaginous neoplasm. No mitoses were appreciated. Occasional binucleated chondrocytes and rare chondrocytes with larger nuclei were seen Figure , 3C, 3D.The pathogenesis of intracranial chondromas remains controversial. Proposed theories include metaplasia of meningeal fibroblasts or perivascular mesenchymal cells, the growth of heterotrophic chondrocytes that develop in the meninges during early ontogenesis, or the cartilaginous activation of meningeal connective tissue after trauma or inflammation .Similar to meningiomas, intradural chondromas appear dural-based, extramedullary, and well-circumscribed masses. Although rare, they differ from a meningioma on imaging by the absence of a dural tail and heterogeneous contrast enhancement . DependiIt is important to distinguish chondromas from other connective tissue tumors that can involve the CNS, such as chordomas and chondrosarcomas that display similar MRI imaging features (hypodense on T1 and hyperdense on T2 MRI imaging) . ChordomLess than 30 of these rare clinical entities have been reported in the literature. The most common presentations in previously reported cases of dural convexity chondromas tend to be headaches and seizures. Based on our review of previous cases of dural convexity chondromas presented by Abeloos, et al., we were only able to identify two likely instances of presentations with impaired ambulation -6. HardyIf small and asymptomatic, intradural chondromas can be observed for interval growth with subsequent MRI brain imaging. Although the circumscribed nature of most intracranial chondromas facilitates surgical resection, as the tumors grow they frequently engulf surrounding brain vasculature that must be preserved. In addition, the large size and fibrous nature of these lesions complicate their surgical resection, particularly when adjacent to the eloquent cortex, as in the case of our patient. Following maximal safe surgical resection, postoperative MRI brain should be obtained at three months as a new baseline. Repeat MRIs would be at the discretion of the treating physician, and we have elected for yearly scans for the first couple years. Radiation therapy is not recommended in cases of residual tumor or inoperable tumors as it is thought that this increases the risk of malignant transformation .Dural convexity chondromas are rare, histologically benign intracranial lesions. Intracranial chondromas may be difficult to distinguish radiographically from meningiomas but generally lack a dural tail and are heterogeneously enhancing. Although surgically curable, these lesions present technical challenges, given that they are frequently large at the time of presentation and involve the eloquent cortex and crucial vascular anatomy.


Leiomyomas are benign tumors observed mainly in adult women. The retroperitoneum is a rare location for leiomyomas; almost 100 cases have been reported. Because retroperitoneal leiomyomas are paucisymptomatic and the tumor size at diagnosis is relatively large, surgical management is challenging. Regular follow-up is required because recurrence and malignant sarcomatous transformation have been described in a few cases.We report a case of a 52-year-old North African woman with a 22-cm retroperitoneal leiomyoma. A preoperative embolization was performed 2 days before surgery. The clinical, therapeutic, and evolutive aspects of this rare entity are discussed.Despite its benignity, retroperitoneal leiomyoma is a challenging diagnostic, therapeutic, and evolutive condition. Surgeons must consider mainly the tumor’s vascularization. Regular follow-up is mandatory because malignant transformation cannot be excluded. Retroperitoneal leiomyoma is a rare and benign condition. It is infrequently observed among primary retroperitoneal neoplasms. The diagnostic approach is challenging because retroperitoneal leiomyomas may be confused with renal tumors. Tumor vascularization must be defined and considered before surgery because it usually arises from the aorta and the renal artery. In this report, we describe a case of a patient with a retroperitoneal leiomyoma requiring preoperative arterial embolization. The diagnostic, therapeutic, and evolutive aspects are discussed.A 52-year-old North African woman consulted our department with a surgical emergency for recent left flank pain associated with a palpable tender solid mass. The patient is a married mother of three children and working as a secretary in public administration. She did not have any past medical history. She delivered two of her children by cesarean section. She complained of chronic constipation of 6 months’ duration. She was apyretic and did not complain of vomiting or recent disturbance in bowel motility. She had no urinary or gynecological disorders. She did not present with sensory or motor focal deficits or visual or speech disorders. The palpable mass was located in the left flank and the iliac fossa, with a posterior dorsal extension and regular margins. Deep palpation of the mass caused tenderness. The patient had no jaundice, and her urine and feces were not discolored.3. Her hemoglobin level was 13.5 g/dl. Her platelet count was normal. Her C-reactive protein concentration was 11 mg/L. Her CA-125 and carcinoembryonic antigen levels were within normal range.The patient’s blood test results were normal. Her liver and kidney function was sufficient. Her white blood cell count was 7000/mmAbdominal ultrasonography revealed a voluminous left-sided heterogeneous tissue abdominopelvic mass with a mass effect on the left kidney. Doppler imaging showed that the tumor was nonvascularized.On an abdominopelvic computed tomographic (CT) scan, the mass measured 16 × 17 × 22 cm. It was well-limited, heterogeneous, and hypervascularized. The vascularization seemed to arise exclusively from the renal artery. The mass effect involved the left kidney and its hilum, the left ureter, the left colon, and the inferior mesenteric vascular axis. The renal vein and the inferior vena cava were permeable (Fig. Angiography confirmed the CT findings because the tumor vessels emerged exclusively from the renal artery. Embolization of the renal artery was performed using a transfemoral approach, which led to total disappearance of the tumor vascularization Fig. . The patAfter 2 days of opioid analgesia, the patient underwent surgery. A midline incision allowed exploration of the abdominal cavity. The results of the operative exploration were similar to the scan findings. The left kidney was pushed to the median line. The mass seemed to be firmly adherent to the kidney and its hilum. The dissection plane was not obviously identifiable. The tumor had a smaller size after embolization, but it was not dissociable from the kidney. Its manipulation was not particularly hemorrhagic. The first step was the identification of the ureter and clamping of the renal artery and the vein. A thorough dissection of the tumor from adjacent structures was performed, allowing a one-piece resection of the mass with the left kidney Fig. .Fig. 3OpThe patient’s postoperative course was uneventful. She was discharged on the fourth postoperative day.Histological examination of the tumor demonstrated it to be a benign mesenchymal proliferation involving regular smooth muscle cells grown in a hypervascularized stroma. Focal hyalinized spots were observed. The mitotic index was not high. No fat cells or necrotic lesions were present Fig. . The tumThe patient was followed regularly for 2 years. The results of her clinical examination were normal. Her renal function was conserved. An abdominal CT scan ruled out any local or distant recurrence.We report a new case of a 52-year-old woman with a retroperitoneal leiomyoma. The tumor measured 22 cm, and the CT scan aspects were confused with a voluminous renal tumor. Vascularization was exclusively from the renal artery, which is rarely observed in retroperitoneal leiomyomas. Preoperative embolization of the renal artery 2 days before surgery was performed safely and allowed decrease of the tumor’s size. The tumor was not dissociable from the kidney, and the operative specimen included the retroperitoneal mass and the kidney in one piece. Immunohistochemical markers were distinctive to ensure diagnostic accuracy.Leiomyomas are developed from smooth muscle cells and are rarely located in the retroperitoneum. To the best of our knowledge, around a hundred of cases have been described in the English literature; 40 of them contain sufficient data for study .Retroperitoneal smooth muscle cell tumors are often diagnosed fortuitously, and symptoms are related to compression of adjacent structures. The mean size at diagnosis is 12 cm . Predictet al. described the eventuality of the development of embryonic vessels in remnant musculature. This hypothesis is supported by the previous case reports describing pulmonary and heart locations [The pathogenesis of retroperitoneal leiomyomas is still unclear. The role of gonadotropic hormones seems to be influential. The uterine location, the one most often described, occurs mainly during the genital activity period. Up to 40% of retroperitoneal cases are associated with synchronous or previously operated uterine myomas . This thocations .The diagnosis is rarely established preoperatively . SymptomUltrasound and CT scan aspects are not evocative. The tumor is usually described as a voluminous, heterogeneous, hypervascularized mass . PreoperDifferential diagnoses concern other spindle cell tumors, such as leiomyosarcomas and stromal tumors. Other rare diagnoses, such as malignant peripheral nerve sheath tumor, inflammatory myofibroblastic tumor, and pleomorphic sarcoma, have been described . ImmunohTumor biomarkers, especially the CA-125 and carcinoembryonic antigens, may be elevated in patients with huge tumors. Preoperative documented elevation of these antigens can be helpful in further follow-up .Surgery is the only curative option. Open surgery was the option chosen in most of the previously reported retroperitoneal leiomyoma cases. A laparoscopic approach was described in only two cases . The difRetroperitoneal leiomyoma is a challenging diagnostic and therapeutic situation. Histological diagnosis is usually postoperative because radiological features are not conclusive of benignity. Surgeons must consider the vascularization of the tumor because it may arise from the renal artery in some cases. Thorough clinical and radiological follow-up is required because of the potential malignant transformation.


Myeloid sarcoma is a tumor mass of immature myeloid or granulocytic cells that affects extramedullary anatomic sites, including uncommonly the oral cavity. A 24-year-old female was referred for evaluation of a fast growing painful gingival swelling lasting 2 weeks, associated with fever, fatigue, and cervical lymphadenopathy. Intraoral examination showed a bluish swelling on the right posterior lower gingiva exhibiting necrotic surface. Incisional biopsy of the gingival lesion displayed diffuse infiltration of undifferentiated tumor cells with granulocytic appearance, strongly immunopositive for CD99, myeloperoxidase and Ki-67 (60%), and negative for CD20, CD3, CD34 and TdT. Blood tests presented a severe pancytopenia, and genetic analysis confirmed the diagnosis of acute promyelocytic leukemia. The final diagnosis was of oral myeloid sarcoma associated with acute promyelocytic leukemia with t. The patient was submitted to chemotherapy but died of the disease one month later. The clinicopathologic and immunohistochemical features of the present case are compared with the 89 cases of oral myeloid sarcoma previously reported in the English-language literature. Key words:Myeloid sarcoma, chloroma, granulocytic sarcoma, gingiva, oral, acute promyelocytic leukemia, acute myeloid leukemia.  Myeloid sarcoma (MS), also known as granulocytic sarcoma or chloroma, is a tumor mass of immature myeloid cells that usually occurs in an extramedullary site or bone of male patients in the sixth decade of life . MS has The microscopical features of MS include the presence of immature myeloblasts within a dense inflammatory background, which are better identified after careful histological and immunohistochemical evaluation -5. Some Oral involvement by MS is uncommon. To the best of our knowledge, only 89 cases of oral MS have been published in the English-language literature so far, and only four of them were associated with acute promyelocytic leukemia -15. HereA 24-year-old female was referred by a general dentist for evaluation of a fast growing gingival swelling that had been present for 2 weeks. The patient reported a 3-weeks history of fever and fatigue. Physical examination revealed cervical lymphadenopathy, and intraoral examination showed discrete areas of clotted blood within the gingival sulcus of some teeth, and a 3 cm painful brownish swelling with necrotic and bleeding surface localized in the right posterior lower gingiva Fig. . RadiogrThe gingival specimen showed a diffuse connective tissue infiltration by poorly differentiated blast-like cells intermingled with chronic inflammatory infiltrate. Tumor cells were large, round to oval, with mild to moderately basophilic cytoplasm containing granules, and round to folded nuclei with fine chromatin. Occasional mitotic figures were found Fig. . By immuMyeloid sarcoma (MS) is an extramedullary solid tumor composed of myeloblasts or immature cells of granulocytic lineage and erythroid precursors ,3. FirstMS is mainly found in the bone and soft tissues, but it may affect virtually any site of the body such as the skin, lymph nodes, orbit and eye, oral cavity, bronchi, pericardium, peritoneum, gastrointestinal tract, kidney, reproductive organs, breast, and bladder -4. ConsiMicroscopically, oral MS exhibits variable numbers of primitive, poorly differentiated cells with granular cytoplasm, round to oval nuclei with well-defined membrane and prominent nucleoli, intermingled with reactive inflammatory infiltrate. The tumor cells show different stages of myeloid differentiation, including the eosinophilic myelocytes and blastic cells with minimal granulocytic differentiation . The micMS may precede, coexist with, or follow a presentation of acute myeloid leukemia (AML), but might also result from an acute blastic transformation of myelodysplastic syndromes or myeloproliferative neoplasms . In factApproximately 90% of patients with APL have shown complete remission of disease after the advent of ATRA and anthracycline-based therapeutic regimens . HoweverIn summary, oral MS is uncommon, with clinical and microscopical features that may mimic inflammatory lesions or other malignant tumors. Careful morphological and immunohistochemical analyses, correlating with clinical data are necessary to establish the diagnosis of oral MS. Clinicians and oral pathologists should consider MS when evaluating gingival swellings and ulcerations in patients with clinical findings suggestive of hematological abnormalities.


A 43-year-old female presented at the accident and emergency department of Princess Marina Hospital, Gaborone, Botswana. She reported a deep dull aching pain of two years duration in the right iliac fossa that has been progressively becoming worse. Ultrasound revealed a large sausage like cystic mass extending from the pelvis up to the medial aspect of the ascending colon. CT scan showed a large sausage like cystic mass extending from the pelvis up to the hepatic flexure of the colon with the cecum displaced. No metastatic features were seen. We made an impression of appendiceal mucocele. A semi-elective laparotomy was scheduled. Intraoperative findings: a giant intact cystic distended appendix with involved base, displacing the cecum cranially. A right hemicolectomy was performed. The histopathological results revealed a low-grade appendicular mucinous neoplasm with no lymph node involvement. The surgical margins were free. The patient recovered uneventfully. Rokitansky first described Appendiceal mucocele in 1842. It is a mucinous distention of the appendix, which can be of neoplastic or non-neoplastic origin , 4. It cWe present a case of a 43-year-old female who presented at the accident and emergency department of Princess Marina Hospital, Gaborone, Botswana. She was referred from a peripheral hospital. She reported a deep dull aching pain in the right iliac fossa that has been progressively becoming worse. She had no associated vomiting or change in bowel habits. She reported no weight loss. The pain was of two years duration and has been in and out of hospital on antibiotics and analgesia. She was referred to Princess Marina Hospital after having been found to have a large sausage like cystic mass extending from the pelvis up to the medial aspect of the ascending colon on ultrasound. Its origins were unclear. There was no free fluid seen on ultrasound. The patient was otherwise afebrile and hemodynamically stable. She had normal full blood count, urea and electrolytes, liver function tests and pregnancy was negative. She was admitted to the ward on analgesia and an abdomino-pelvic CT scan was performed the next day, which showed a sausage like cystic mass extending from the pelvis up to the hepatic flexure of the colon with the cecum displaced and ante-flexed over the ascending colon. There were no liver lesions or lymphadenopathy noted. The length of the mass was 17.27 cm and the maximum diameter was 5.28cm . There wX50 : low power viewing): The appendiceal wall shows a flattened and misplaced epithelium (on top) as well as marked fibrosis surrounding a focus of dystrophic calcification X50 : low powX100 and x400 (: medium and high power viewing): The appendiceal wall shows an intestinal type covering epithelium (right side) comprising of crowded columnar cells with basal, hyperchromatic nuclei and large amounts of apical mucin as well as minimal dysplastic changes. Marked fibrosis beneath the epithelial lining is readily apparent. *Fibrosis and chronic inflammation are secondary changes due to luminal compression in a long-standing disease.and x400 : medium Preoperative diagnosis of appendiceal mucocele is very important. Contrast-enhanced CT imaging is most commonly used modality for preoperative diagnosis. It informs the choice of procedure and avoids complications. Preoperative colonoscopy may reveal a pathognomonic volcano sign that describes a mass obstructing the appendiceal opening with a central crater that produces mucin . HoweverAppendiceal mucocele is a rare condition. The clinical presentation is often non-specific and the clinician should have appendiceal mucocele in mind in patients presenting with long-term right lower quadrant pain, adnexal masses and acute appendicitis picture. Often, the diagnosis is made incidentally during imaging or surgical procedure. Radiological imaging and careful analysis is critical as it informs choice of procedure and management. Surgical resection is potentially curative and rupture of the mucocele should be avoided as it may lead to pseudomyxoma peritonei, a condition with high morbidity and mortality. Exercise diligence when choosing surgical approach and procedure.The authors declare no competing interests.


Fibroma is one of the most common soft tissue benign tumors of the oral cavity. These masses represent hyperplasias instead of true neoplasm, which develop due to irritation to the mucosal tissue resulting in proliferation of the cells. Although so common in the oral cavity, its occurrence on the palate is rare, mainly due to fewer chances of trauma. Here, we report a case of palatal fibroma in a child diagnosed on the basis of clinical, radiological, and histological features. The case represents an extremely rare occurrence as unusual trauma due to thumb sucking seemed to be the only apparent traumatic factor in the palatal region.Mishra R, Khan TS, Ajaz T, Agarwal M. Pediatric Palatal Fibroma. Int J Clin Pediatr Dent 2017; 10(1):96-98. According to Torres-Domingo et al,2 It was first reported in 1846 as fibrous polyp and polypus and is found in 1.2% of adults.34Fibromas are hyperplasias of fibrous connective tissue in response to local irritation or trauma. Tissue enlargements attributable to injury represent a hyper-plastic reaction and are collectively grouped as “reactive proliferations.” It is also known as irritational fibroma, traumatic fibroma, fibrous nodule, or fibroepithelial polyp.The diagnosis of these lesions is based mainly on his-topathological features. Most of these lesions are relatively characteristic in presentation, leaving very little doubt about the diagnosis. In certain instances, however, unusual findings may result in diagnostic uncertainty. Here, we report a rare case of palatal fibroma occurring in an 8-year-old boy where the causative irritational factor was due to a seemingly innocuous parafunctional habit of thumb sucking.An 8-year-old boy reported to the Department of Pedodontics and Preventive Dentistry at our center with a chief complaint of palatal growth in the midline region. History revealed that the problem started with ulceration in the palate about 3 years back. After a few days, he observed a small growth in the same region, which gradually enlarged during the following months. Patient also reported of thumb sucking habit till the age of 5 years. He was advised antibiotic and antifungal medication by the local physician to which there was no response.On intraoral examination, a large, smooth-surfaced, and pedunculated growth was observed (2.5 × 2 × 1.5 cm approx) in the palatal area . On palp Radiographically, no abnormality was seen. Routine blood investigations were within normal range.Incisional biopsy was done, and the tissue was sent for histopathalogic examination. The report showed orthokeratinized stratified squamous epithelium with short rete pegs encircling the connective tissue stroma with abundant collagen fiber bundles along with proliferating spindle-shaped fibroblasts, few myxomatous areas, chronic inflammatory cells infiltration, and blood vessels . OverallClinically, the soft tissue overgrowth appeared of normal mucosal color and texture, and due to the specific location, the possible differential diagnosis included salivary gland tumors, giant cell fibroma, myxoma, pyogenic granuloma, and neurofibroma. As the site of soft tissue growth was at the midline of posterior hard palate, the possibility of irritation fibroma was not considered prior to the histopathological report.Final diagnosis of irritational fibroma was made and surgical excision of lesion was done . Follow-Irritational fibroma is usually sessile, round or ovoid, nontender and may be lighter in color than the surrounding tissue due to reduced vascularity. Due to the gradual and slow growth of the lesion, the patients are generally aware of the mass.5 Contrary to the aforementioned evidence, this case presents an 8-year-old male child with the lesion. The presumed etiology of fibroma is trauma to the affected mucosa. In the case presented, although there was no direct history of trauma, a traumatic stimulus could have been inflicted due to parafunctional habit of thumb sucking or maybe due to trauma from any sharp foreign object.6 The patient did report of initial ulceration prior to the growth formation. Although the irritational fibroma can occur anywhere in the oral cavity, the most common site is the buccal mucosa along the occlusal line; other common sites are labial mucosa, tongue, and gingiva. Midline palatal fibroma at such a young age has not been reported till date in literature, except in old denture wearers, where it presents as a flat, pancake-shaped mass.The irritational fibroma has a 66% female predilection and can occur at any age, but is usually seen in the 4th to sixth decades of life.78 In contrast to the common clinical presentation, the present lesion was of a much larger dimension than normal, covering about two-thirds of the hard palate.As far as size is concerned, the lesions generally are less than 1 cm in diameter.9 Pyogenic granulomas arise from proliferation of the fibrovascular connective tissue, whereas peripheral giant cell granulomas arise from proliferation of the periosteal tissue containing osteoblasts. Periodontal ligament fibroblast proliferation gives rise to peripheral ossifying fibroma as they retain the potential to form bone and cementum.9 In our case, the absence of periodontal tissue ruled out the possibility of peripheral ossifying fibroma as a possible diagnosis.The pathological mechanism of fibroma involves hyperplasia due to trauma to the mucosal tissue resulting in proliferation of cells followed by collagen fibrillogen-esis. In the oral cavity, apart from fibroblasts, the periodontal tissues, fibrovascular connective tissues, periosteum, etc. may be the target of injury.78 Fibroma does not have any malignant potential, and recurrence is rare following total excision.Microscopically, nodular deposition of dense collagen in association with chronic inflammation, spindle-shaped fibroblast, and overlying thinning mucosa is present, which confirmed the diagnosis. Trauma-related changes, such as hyperkeratosis and ulceration may also be seen in long-standing fibromas, which were not present in our case.Fibromas, though very common lesions of the oral cavity and characteristic in presentation, may sometimes pose a diagnostic challenge. Clinicians should consider the possibility of diagnosing irritation fibroma in younger age groups and in unusual locations as palate. Detailed history regarding the lesion, precise clinical workup combined with microscopic presentation is required for diagnostic confirmation and proper management of such cases.Fibrous growths of the oral soft tissues are fairly common and include a diverse group of reactive and hyperplastic conditions. As a pedodontic, the key for management should be early education and interception of abnormal oral habits in children followed by identification of any reactive hyperplastic lesion by devising a differential diagnosis to enable precise patient evaluation and, thereon, its treatment.


Tumors of the rostral maxilla that involve both the oral mucosa and the dermis or subdermis of the dorsolateral muzzle provide unique challenges for the oromaxillofacial surgeon. Traditionally described approaches to such lesions may involve an intraoral incision that extends and involves the upper lip to envelope the involved dermis of the dorsolateral muzzle. However, such an approach unnecessarily resects upper lip tissue resulting in a large defect that likely requires advanced skin flaps or grafts for reconstruction. Such flaps are technically challenging and introduce potential for significance postoperative complications. In this article, we provide a detailed description a combined intra- and extraoral approach that allows for composite resection of tumors of the rostral maxilla that also involve the dorsolateral muzzle. The described technique allows for excellent intraoperative visualization and provides a superior cosmetic outcome that minimizes postoperative complications. In addition, we describe our experience utilizing the technique in three clinical cases. The goal of any tumor resection is to obtain tumor-free margins while maintaining proper function and, ideally, achieving an excellent cosmetic outcome. Achievement of these goals is dependent on tumor location, invasiveness, and the complexity of the resultant wound closure. Rostral maxillary tumors that also involve the nasal bone and overlying skin of the dorsolateral muzzle often require more complicated composite resections. Composite resections are defined as en bloc resections involving adjacent tissue types . From dorsal to ventral, composite resections of the rostral and dorsolateral maxilla entail en bloc resection of skin, nasal bone, ±incisive bone, nasal mucosa and turbinates, maxilla bone, hard palate, teeth, gingiva, and oral mucosa. Because of the intra- and extraoral nature of tumors of the rostral maxilla and dorsolateral muzzle, the surgical approach is often more complicated than the more straightforward intraoral tumors that do not involve the dorsolateral muzzle. The surgical closure of the resultant wound, which often necessitates reconstruction of lip tissue, may also present additional challenges and surgical complications. In addition, postoperative function and cosmetic outcome may be less than desirable. In such situations, a combined intra- and extraoral approach to composite resections of tumors of the rostral maxilla and dorsolateral muzzle that spares the lip may be utilized. Such a technique preserves an isthmus of upper lip, which allows good to excellent cosmesis and decreased postoperative morbidity as no axial pattern flap is required for lip reconstruction. Although this technique has been very briefly described in a textbook, to the authors’ knowledge, a detailed description of the application of this technique in clinical patients has not previously been described in the peer-reviewed English language literature. Therefore, we report our experience utilizing the upper lip-sparing composite resection of rostral maxilla and dorsolateral muzzle tumors utilizing a combined extra- and intraoral approach and postoperative outcome in three dogs.The airway was secured by orotracheal intubation immediately following induction of general anesthesia. To minimize oral bacterial load and contamination, the dentition in the region of the surgical approach was scaled and polished, and an oral antiseptic rinse was applied, before surgery. Dogs were positioned in a modified dorsal recumbency position with the head positioned slightly lateral, surgical side up. The entire maxilla was clipped and prepared for aseptic surgery from the medial canthus of the eye to the nasal planum and from the ipsilateral mucocutaneous junction to at least 2 cm (dictated by tumor location) beyond the dorsal midline of the muzzle. A sterile surgical pen was used to outline surgical margins of 2 cm based on the combination of visible tumor size and osseous extent as determined from computed tomographic (CT) studies. The extraoral surgical margins were inked on the skin of the dorsolateral muzzle, and the intraoral margins were marked on the vestibular oral mucosa, gingiva, and palatal mucosa Figures  and 2. Tvia lymph node aspiration and thoracic radiography. Head CT revealed a 1.2 cm soft tissue attenuating mass along the left dorsolateral maxilla extending from the mesial aspect of the left maxillary canine tooth to the mesial aspect of the left maxillary second premolar tooth. The dorsal extent of the mass was 0.7 cm from the dorsal midline of the muzzle and ventrally involved the alveolar margin of left maxillary canine tooth. An intraoral lesion was visible from the distal left maxillary canine tooth to the left maxillary second premolar tooth  of the left rostral maxilla and dorsolateral muzzle. On presentation, the swelling had a rostral to caudal linear diameter of 3.7 cm, and suture material from the referring DVM (rDVM)-acquired biopsy was still present. Absence of metastasis was confirmed  Figures  and 10. The case was referred to the dentistry and oral surgery service for surgical treatment. A composite resection utilizing a combined intra- and extraoral approach was performed. The soft tissue resection extended from 1 cm rostral to the medial canthus of the left eye and extended to the nasal planum. From dorsal to ventral, the resection extended from the dorsal midline of the muzzle to approximately 7 cm from the mucocutaneous junction. The osseous resection extended from the mesial aspect of the left maxillary second incisor tooth to the furcation of the left maxillary second premolar tooth. The palatal extent of the resection was the median palatine raphe. Following resection, the distal root of the left maxillary second premolar tooth was extracted, and the wound was closed as described earlier.Histological evaluation of the resected tissue confirmed the diagnosis of H/L FSA and surgical margins were free of tumor with a narrow margin at the distal aspect of the resection. The dog recovered uneventfully from general anesthesia and was managed overnight in the critical care unit on a continuous rate infusion (CRI) of fentanyl (3 μg/kg/h), ketamine (5 μg/min/h), and lidocaine (25 μg/min/h). A suppository of acetaminophen/codeine (325 mg) and IV ampicillin/sulbactam (900 mg) was also administered. The dog was discharged the following day with a 125 μg fentanyl patch, oral acetaminophen/codeine (325 mg q 8 h), tramadol (5 mg/kg q 8–12 h), and amoxicillin/clavulanic acid (13.75 mg/kg q 12 h). Instructions to feed only canned or softened kibble and to avoid toys or mouth play were given to the owners. At discharge, the dog seemed comfortable and was eating and drinking well.At the time of oral surgery recheck and skin suture material removal 14 days later, the owners reported the dog was doing very well at home, eating and drinking well, was not experiencing difficulty breathing and experiencing occasional episodes of reverse sneezing. The skin and oral incisions had healed well, and no discharge, redness, swelling, or dehiscence was noted. Skin suture material was removed. At the last recheck of this patient, 18 months postoperatively, there was no evidence of recurrence, and the patient was doing well at home.via lymph node aspiration and thoracic radiography by the rDVM before referral. A 3.4 cm, firm swelling was present on the dorsolateral aspect of the left rostral muzzle. Head CT revealed an ill-defined 2.7 cm × 1.8 cm × 2.8 cm soft tissue attenuating mass in the left dorsolateral maxilla extending from the mesial aspect of the left maxillary canine tooth to the mesial aspect of the left maxillary second premolar tooth. The dorsal extent of the mass was 0.6 cm from dorsal midline of muzzle and ventrally involved the alveolar margin of left maxillary canine tooth.A 10-year-old spayed female golden retriever was referred to the medical oncology service for evaluation of a biopsy-confirmed FSA involving the left rostral maxilla and dorsolateral muzzle. Absence of metastasis had been confirmed The case was referred to the dentistry and oral surgery service for surgical treatment. A composite resection with a combined intra- and extraoral approach was performed. The soft tissue resection extended from 3 cm rostral to the medial canthus of the left eye to <1.0 cm from the nasal planum. From dorsal to ventral, the resection extended from the dorsal midline of the muzzle to approximately 7 cm from the mucocutaneous junction of the left lip. The osseous resection extended from the mesial aspect of the left maxillary third incisor tooth to the distal root of the left maxillary second premolar tooth. The palatal extent of the resection was approximately 1 cm palatal to the dental arch. Following resection, the distal root of left maxillary second premolar tooth was extracted, and the wound was closed as described earlier.Histopathological evaluation of the resected tissue confirmed the diagnosis of FSA and tumor-free margins. The dog recovered uneventfully from general anesthesia and was managed overnight in the critical care unit on an intravenous (IV) CRI of fentanyl (3 µg/kg/h), lidocaine (13 µg/min/h), and ketamine (5 µg/min/h). Ampicillin/sulbactam  was also administered. The dog was discharged the following day with a 100 mcg fentanyl patch, oral tramadol (4 mg/kg q 8–12 h), and clindamycin (5 mg/kg q 12 h). The owners were instructed to feed only canned or softened kibble and to avoid toys or mouth play. At discharge, the dog seemed comfortable and was eating and drinking well. At the time of oral surgery recheck and skin suture material removal 14 days later, there was no evidence of dehiscence, incisions were healing well, and a good cosmetic appearance was apparent. Continued follow-up was done with rDVM. The patient was euthanized 11 months after surgery for renal failure unrelated to the oromaxillofacial neoplasia. At that time, no evidence of recurrence was present.A 9-year-old neutered male Labrador retriever was referred to the medical oncology service for evaluation of a mass on the left rostral maxilla and dorsolateral muzzle. On presentation, the swelling had a diameter of 3.3 cm, round and firm. The mass could be appreciated intraoral at the apex if 204 and extraorally ~1.5 cm caudal to the nasal planum. The patient was placed under general anesthesia for head and thoracic CT as well as incisional biopsy and regional lymph node aspiration. Regional lymph node aspiration showed reactive lymphoid tissue and the thoracic CT showed atelectasis, consistent with general anesthesia, with no evidence of pulmonary metastasis or intrathoracic lymphadenopathy. Head CT revealed a 4.6 cm soft tissue attenuating mass along the left dorsolateral maxilla extending from the mesial aspect of the left maxillary canine tooth to the mesial aspect of the left maxillary second premolar tooth. The dorsal extent of the mass was 1.5 cm from the dorsal midline of the muzzle and ventrally involved the alveolar margin of the left maxillary canine tooth. Results of histological examination revealed a histologically low-grade FSA with mild bony infiltration. Based on the clinicopathologic correlation, the tumor was determined to be a histologically low-grade, biologically high-grade FSA (H/L FSA).The case was referred to the dentistry and oral surgery service for surgical treatment. A composite resection utilizing a combined intra- and extraoral approach was performed. The soft tissue resection extended from 1 cm rostral to the medial canthus of the left eye and extended into the left lateral nasal planum. From dorsal to ventral, the resection extended from the dorsal midline of the muzzle to approximately 3.5 cm from the mucocutaneous junction. The osseous resection extended from the mesial aspect of the left maxillary second incisor tooth to the furcation of the left maxillary fourth premolar tooth. The palatal extent of the resection was the median palatine raphe. Following resection, the distal root of the left maxillary fourth premolar tooth was extracted and the wound was closed as described earlier.Histological evaluation of the resected tissue confirmed the diagnosis of H/L FSA and surgical margins were free of tumor with a narrow margin at the palatal aspect of the resection. The dog recovered uneventfully from general anesthesia and was managed overnight in the critical care unit on a CRI of sufentanil (0.3 µg/kg/h) and ketamine (5 µg/min/h). A subcutaneous injection of 2.2 mg/kg carprofen (95 mg) and IV ampicillin/sulbactam (950 mg) was also administered. The dog was discharged the following day with a 150 mcg fentanyl patch, oral carprofen (93.75 mg q 12 h), tramadol (5 mg/kg q 8–12 h), and amoxicillin/clavulanic acid (13.75 mg/kg q 12 h). Instructions to feed only canned or softened kibble and to avoid toys or mouth play were given to the owners. At discharge the dog seemed comfortable and was eating and drinking well.At the time of oral surgery recheck and skin suture material removal 14 days later, the owners reported the dog was doing very well at home, eating and drinking well, was not experiencing difficulty breathing and experiencing occasional episodes of reverse sneezing. The skin and oral incisions had healed well, and no discharge, redness, swelling, or dehiscence was noted. Skin suture material was removed. At the last recheck of this patient, 3 months postoperatively, there was no evidence of recurrence, and the patient was doing well at home.Composite resections of the oromaxillofacial region may result in considerable cosmetic and functional deformity. Resections that involve the muzzle have traditionally been achieved by extending the extraoral incisions through the mucocutaneous junction of the lip and into the oral cavity, in many cases unnecessarily resecting the upper lip adjacent to the tumor . The resThe surgical approach and wound closure utilized in the three dogs of this report has been very briefly described in a veterinary surgery textbook in which it was called the “upper labial pull-down technique” . HoweverAxial pattern flaps have greater potential to reach distant wounds and have a reported overall survival of 89–100% , 8–12. HThe remaining lip tissue resulting from the approach described here, provides, in essence, a bipedicle advancement flap supplied by the preserved lateral nasal artery and the inferior labial vein. The lateral nasal artery, a branch of the infraorbital artery, courses rostrally from the infraorbital foramen in the inferior one-third of the lip. Thus, the flap is vascularized by a substantial arterial supply significantly minimizing any risk of ischemic necrosis. However, because mucosa must be separated from the flap to close the intraoral defect, the risk of ischemic necrosis cannot be fully eliminated.While proper wound healing and acquisition of a quick return to proper function is critical to clinical success, often a client’s willingness to pursue a major oromaxillofacial resection is based significantly on the possibility of achieving good postoperative cosmesis. The described technique provides excellent cosmesis Figure . The tecIn conclusion, the upper lip-sparing technique utilizing a combined extra- and intraoral approach to composite resections of rostral maxilla and dorsolateral muzzle, we detail here provides a more conservative approach that reliably allows primary closure of the resultant wound with a simple advancement flap. As a result of avoiding other local or regional flaps for lip reconstruction, this technique minimizes the typical associated complications and provides an excellent cosmetic outcome.The concept for this manuscript was primarily the responsibility of JS. Both JS and AT contributed approximately equally to the writing.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Malignant peripheral nerve tumors, a small subset of soft tissue sarcomas, provide a unique diagnostic challenge. Although they may arise from peripheral nerves or from cells associated with nerve sheaths, malignant peripheral nerve tumors often present with diverse immunohistochemical features similar to those of other tumors. These features make MPNSTs difficult to diagnose and classify. We present a case of a 26-year-old female presenting with a rapidly growing soft tissue mass. The mass was excised and immunohistological staining suggested a Ewing’s sarcoma/Primitive neuroectodermal tumor. Confirmational studies did not confirm this diagnosis and upon further review, the diagnosis was changed to a malignant peripheral nerve sheath tumor. We reviewed this case in the setting of the reported literature concerning MPNSTs with focus on the epidemiologic, diagnostic, and immunohistologic features that distinguish this tumor from other similar malignancies. Malignant peripheral nerve sheath tumors (MPNST) comprise a small subset of only 5-10% of soft tissue sarcomas . While tA 26-year-old African American female presented with a progressively enlarging left inguinal mass for three months. The mass has been more painful and now presented with multiple areas of hemorrhage. At age 17, the patient underwent an uncomplicated pregnancy and C-section. Her C-section incision was complicated with keloid formation for which she underwent two doses of radiation. She remained asymptomatic up until she noticed swelling three months prior. She did not report any other trauma and her only other medical history was significant for a tubal ligation and appendectomy. She had no family history of previous cancer and quit smoking three months prior. Her review of systems was negative except for recent fever and chills. Her vitals were stable and her physical exam was normal except for the inguinal mass and slightly palpable bilateral inguinal lymphadenopathy. The mass protruded through the skin, was very tender to palpation, and expressed multiple areas of hemorrhage. It spanned about five centimeters in diameter along the left lateral aspect of a transverse C-section incision . Her labThe patient initially underwent resection of the mass and given that a sarcoma was suspected, her wound was left open while tumor margins were obtained. The posterior tumor margin returned positive at three millimeters and a reexcision with subsequent wound closer was performed. The initial biopsy report suggested Ewing’s sarcoma/Primitive Neuroectodermal Tumor with findings concerning for melanoma. A EWSR-1 gene rearrangement and BRAF V600E mutation were then sent to confirm ES/PNET and melanoma respectively. A PET-CT revealed hypermetabolic activity with soft tissue stranding in the subcutaneous region of the anterior pelvis, bilateral pelvic and inguinal hypermetabolic lymph nodes, and a four-millimeter left apical lung nodule , 3. ThesThe patient subsequently completed two cycles of chemotherapy with cyclophosphamide, doxorubicin and vincristine alternating with ifosfamide and etoposide. By this time, the confirmatory genetic studies returned negative, thus questioning the original diagnosis. The case was then referred to another expert pathologist at Brigham and Women’s hospital in Boston who suggested MPNST. The patient underwent localized radiotherapy with follow up MRI showing minimal soft tissue stranding, resolving postsurgical change, and no definite residual or recurrent tumor. Previously identified inguinal and external iliac lymph nodes were no longer evident. On follow up she finished radiotherapy and returned back to work.On gross examination, the biopsy specimen had a shiny appearance suggesting myxoidal features along with other areas of hemorrhage . On histThe importance of correctly diagnosing a MPNST cannot be understated. While representing a small proportion of soft tissue tumors, these tumors present similarly and overlap histopathologically and histochemically with other tumors of their class. Our report provides as example of the difficulty in differentiating these tumors. An accurate diagnosis, therefore, should be based on clinical, radiographic, pathologic findings and genetic testing.Clinically and radiographically, findings may suggest a soft tissue sarcoma and if attached to a peripheral nerve, a peripheral nerve sheath tumor is the likely diagnosis. This situation, however, is not always the case, as described by Mitchell et al who report a case of an ES initially thought to be a MPNST due to its association with an L4 nerve root . FurtherGenerally speaking, CD99 and S100 have been routinely associated with ES and MPNST respectively. Making a diagnosis based solely on these stains, however, is unreliable. In a series of 16 MPNST , Zhu et al found 75% of specimens with positive S100 staining . Olsen eThe question then arises as to how to accurately reach a diagnosis in the setting of multiple overlapping stains. The most reliable ways to confirm ES are with reverse transcriptase-polymerase chain reaction (RT-PCR) and fluorescent in situ hybridization (FISH) studies demonstrating t translocation involved in expression of a EWS/FLI-1 fusion transcription factor. Mitchell et al and Mohan et al confirmed their ES diagnoses with these tests, respectively , 5.On microscopic examination, ES/PNET and MPNST also show similar patterns. MPNSTs themselves have variable differentiation, such as rhabdomyoblastic differentiation, glandular malignant differentiation, malignant triton tumor, epithelioid malignant shwannoma, and superficial epithelioid differentiation. In general, however, MPNSTs are marked by intermixed dense cellular fasicles and myxoid regions (marbleized pattern) with round or fusiform cells . There mThe treatment of choice for MPNST is surgical resection with clear margins. This may be difficult to achieve depending on the location such as in the spine. The current recommendation also includes adjuvant radiotherapy for localized control. There are also some reports of radiotherapy without surgical resection, but this is still controversial and needs further study. Chemotherapy has an even less role in MPNST. While chemotherapy is generally based on that of other soft tissue sarcomas, its efficacy is still needs further investigation . In contThe other interesting finding about radiotherapy is that it, like in our case, may also increase the risk of future MPNST. Ducatman et al also cites a small sample of 13 postradiation MPNST cases among 120 overall cases. They note a latency period averaging 16.9 years between initial radiation and tumor diagnosis [In terms of prognosis, there are a number of tumor features and characteristics to consider. While the prognosis of MPNST is generally thought to be worse with NF1, there may be conflicting evidence , 11. If There also appears to be an increase in survival (P = 0.001) and less recurrence (P = 0.0001) in patients at least 30 years old than in younger patients . Of the MPNSTs are a rare subset of soft tissue sarcomas that have clinical, radiographic, and histopatholoigic features similar to other tumors such as ES/PNET. Accurate diagnosis based on pathological and histochemical staining can be challenging yet very important due to their prognostic and treatment implications.


A 1-day-old male infant was evaluated in the neonatal intensive care unit for a “large vascular mass encircling his right hand” . UltrasoDefine congenital hemangioma and the subtypes?What are the clinical features and how are they diagnosed?What is Kaposiform hemangioendothelioma (KHE)?What are the treatment/management options?Congenital hemangiomas are rare benign vascular tumors that commonly present fully formed at birth, unlike infantile hemangiomas. The incidence of cutaneous congenital hemangiomas is unknown, and its pathogenesis remains poorly understood. Two congenital hemangioma subtypes have been most recognized: RICHs and noninvoluting congenital hemangiomas (NICHs). Recently, distinction between the two has been called into question, as not all RICHs completely involute and present as NICHs.Clinically, both RICHs and NICHs present as solitary, plaques, or exophytic lesions of varying size.KHE is a rare, locally aggressive vascular tumor that often presents in early childhood and difficult to distinguish from a congenital hemangioma. It is often found in conjunction with the coagulopathy disorder Kasabach-Merritt syndrome. The pathogenesis of KHE is poorly understood, but spindled neoplastic endothelial cells demonstrate expression of vascular markers CD31 and CD34, lymphangiogenesis markers vascular endothelial growth factor receptor (VEGFR-3), and lymphatic markers D2-40 and PROX1.6Management of congenital hemangiomas is patient specific and highly dependent on the size, location, ability to involute, and presence of complications. RICHs are self-resolving and usually expectantly managed, unless complications such as ulceration or bleeding occur. Once involution is complete, the fibrofatty residual tissue may be excised to enhance cosmesis. Contrary to RICHs, NICHs do not resolve over time but can be managed similarly if the patient is asymptomatic.


Giant Cell Reparative Granuloma (GCRG) of phalanx is uncommon. It is a benign osteolytic lesion but can be locally aggressive. GCRG has certain radiology and histological features that are similar to other giant cell lesions of the bone. We present a case report of a young patient with giant cell reparative granuloma of proximal phalanx of left third toe. The bone lesion was successfully treated surgically. GCRG has similar characteristics with other giant cell lesions especially giant cell tumor, aneurismal bone cyst and brown tumor. Thus, our report emphasizes on radioimaging and hispathological findings in this patient.Giant Cell Reparative Granuloma (GCRG) is a benign osteolytic bone lesion. It rarely affects the tubular bones of the hand and foot. In 1953, Jaffe first described GCRG which was found in a patient with mandible and maxilla lesionA 21 year-old Malay lady came to Orthopedic clinic, complaining of 2 years duration of left third toe swelling. The swelling was progressively increased in size and was associated with occasional pain for the past 1 year. There was no history of trauma or infection prior to the toe swelling. Hematological investigations were unremarkable. Diagnosis of aneurismal bone cyst was made based on radiograph findings . Patient2. Our patient presented with 2 year duration of relative painless lesion at the left third toe.The clinical presentations of GCRG in the distal phalanx are non specific. Typically patient presents with pain, swelling, tenderness and palpable mass at the phalanx. The duration of symptoms ranges from 6 weeks to 10 years3. Although MRI typically shows fluid-fluid levels within the cystic cavities of ABC, this feature is not always present. HPE was previously used to differentiate GCRG from ABC. However in recent years, a study on ABC has found similar morphology to GCRG. A term of ‘solid variant’ was added to ABC4. The solid variant of aneurysmal bone cyst and GCRG were later described as reactive and benign.Radiologically GCRG is difficult to distinguish from Aneurysmal bone cyst (ABC). Both lesions show expansile lytic lesion with multilocular appearance and thin cortex5. Complete surgical resection of the lesion reduces the recurrence rate but at the expense of lost of function. Our patient underwent surgical wide resection of bone lesion with iliac bone grafting. She recovered well after surgery. Follow up radiograph showed no recurrence of the lesion after 8 months of surgery (Conventional treatment of GCRG is enucleation and curettage. The recurrence rate of this method is estimated between 10% to 20% surgery . She wasGCRG can mimic ABC in plain radiograph. Although GCRG is rare especially in the distal phalanx, physicians should be aware of various differential diagnoses when dealing with expansile lytic bone lesion in the distal phalanx.


Bronchial papilloma and pulmonary sclerosing hemangioma (PSH) are rare tumors. The development of PSH combined with bronchial papilloma in lung is extremely rare. We herein presented a case of the co-occurrence of bronchial papilloma and PSH in a male.A 48-year-old man was referred to our department for further treatment of the productive purulent cough and fever. After bronchoscopy, the biopsy of the specimen showed a bronchial papilloma in the lumen of posterior segment of the right upper lobar bronchus. For computed tomography demonstrated a focal lesion with an air bubble in the posterior segment of the right upper lobe, a lobectomy was performed and PSH was diagnosed.The report emphasizes the fact that even though some of bronchial papilloma and PSH may have a low prevalence, nonetheless, the low prevalence of both diseases in a male has meant that it has not been possible to explore the depth of association between them.The paper reports a case of PSH in a male suffering from bronchial papilloma which is the exceptional concurrence of these two extremely infrequent events. Bronchial papilloma is a rare tumor that accounts for 0.38 % of all lung tumors and 7–8 % of all benign lung tumors. It is not uncommon to report a significant delay in making an appropriate diagnosis for the clinical presentation of this tumor is usually nonspecific. The clinical presentation ranges from cough, dyspnea at rest or with exertion, to stridor and upper airway obstruction  is predominantly identified in middle-aged women and accounts for approximately 1 % of all benign pulmonary tumors , the erythrocyte sedimentation rate (67 mm/h), the C-reactive protein (106 mg/L) and the procalcitonin (0.106 ng/ml) were normal. Cultures for bacteria, fungus, and acid-fast baclilli were negative from the sputum. Pulmonary function examination is normal including FVC (3.27 L), FEV1 (3.06 L), FEV1% FVC (93.64 %) and MVV (87.67 L/min). For chest radiography revealed features suggestive of lung abscess (Fig. A 48-year-old man visited a local hospital because of productive purulent cough for seven days and fever for three days. Aside from these, the patient’s medical history was completely uneventful. Chest radiography performed prior to admission showed a focal round lesion with thick-walled cavity in the right upper lung Fig. a. Some lThis case produces two interesting findings as following. Firstly, bronchial papilloma and PSH are rare tumors while the description of a new case enhances the understanding of these diseases. Secondly, relative to the previous literature, co-occurrence of bronchial papilloma and PSH in a middle aged male is rare.It is usually presented as an endobronchial mass in the segmental bronchi when bronchial papilloma occurs in the tracheobronchial tree, which has been diagnosed by bronchial biopsy of the specimen (Aida et al. Bronchial papilloma is seen more frequently in men and generally appears in the age range of 50–70 years. The treatment methods for bronchial papilloma, such as endoscopic resection or surgical resection, are still controversial for the pathological examination of the whole papilloma can be prevented by laser irradiation while the extent of surgical resection is also a serious consideration, because these benign tumors are mostly located at the main bronchus or orifice of the lobar bronchus (Yıldırım et al. Of particular interest, after surgical resection, the lesion of the right upper lobe in our case is a PSH proved by histopathological examination. As widely known, PSH is a rare benign tumor of the lung, which occurs predominantly in middle aged women (Guerra-Gutiérrez et al. Our immunohistochemical results have suggested that the tumor cells stained positively for TTF-1, EMA, CK7, cytokeratin and CD31 that indicate the tumor originates from the epithelium, especially from type-II pneumocytes or Clara cells, but not from endothelium and the mass was decided as a sclerosis haemangioma of lung. As noted above, bronchial papilloma and PSH have been reported respectively in the literature. To the best of our knowledge, we introduce the case of a patient with the diagnosis of bronchial papilloma and PSH.Although PSH has not been previously associated with bronchial papilloma, cases of PSH co-occurring with myomas, or with thyroid and kidney cysts have been reported in the literature, and these could indicate that PSH is yet another of the malformations that might be present in bronchial papilloma while other reports also have revealed that PSH has been found in association with Cowden syndrome or Lynch syndrome (Guerra-Gutiérrez et al. In conclusion, this is a reported case of PSH in a male suffering from bronchial papilloma which is the exceptional concurrence of these two extremely infrequent events. This case highlights the fact that even though some of these lesions may have a low prevalence, they may be included in the differential diagnosis of solitary lung lesions. Nonetheless, the low prevalence of both diseases has meant that it has not been possible to explore the depth of association between them.


Enchondroma, a subtype of chondroma, originates from the medullary cavity of the bone and produces an expansile growth pattern. Enchondroma located in the spine is rare and a few cases of large thoracic enchondroma have been reported. The authors document a rare case of large enchondroma in the thoracic spine of a 49-year-old woman, and discuss its clinical, radiological and histopathological characteristics.The patient presented with rapidly progressive and severe pain on her upper back. Magnetic resonance imaging revealed an expansile lesion at the posterior elements of T3 that was hypointense on T1-weighted images and mixed iso- to hyperintense on T2-weighted images. Administration of gadolinium-diethylenetriamine pentaacetic acid (Gd-DTPA) resulted in heterogeneous enhancement. During surgery, a large tumor of 4.2cm × 4.7cm × 2.1cm was resected along with the lamina and spinous process. Histological examination revealed that the tumor consisted of mature hyaline cartilage with typical chondrocytes, indicating that it was an enchondroma.Despite its benign-growing nature, enchondroma should be examined closely for signs of enchondromatosis and enchondrosarcoma. Complete surgical resection is the treatment of choice for immediate relief of symptoms and avoidance of recurrence. Chondroma is a slowly growing benign cartilaginous tumor, and rarely affects the spine . AccordiA 49-year-old woman presented with progressive and severe upper back pain for 1 month. Severe tenderness was detected over the T3 spinous process. There was no motor deficit, and only mild sensory loss distal to the right ankle. Plain radiographs of the thoracic spine showed a radiolucent lesion at the T3 spinous process Fig. . MagnetiExcision of the tumor in situ was performed via a posterior approach. The mass was easily separated from the surface of the dura and measured in size of 4.2cm × 4.7cm × 2.1cm. The right transverse process was then removed, followed by curettage of the pedicle to maximally eliminate the residual tissues of the tumor. During surgery, we found that the superior part of T4 lamina and spinous process was compromised Fig. . Small aEnchondroma is a slow-growing tumor and can develop at any part of the vertebra, including spinous process, lamina, pedicle, and vertebral body . NeverthEnchondroma usually occurs in a solitary fashion . The prePlain radiographs of the spine are difficult to identify an enchondroma. CT scan is of help to clearly visualize the pathology and its relation to adjacent bones . A radioGrossly, enchondroma appears as lobules of firm, mature cartilage, with regions of grittiness signifying mineralization of the matrix . HistoloSurgical excision is generally recommended as the treatment of choice for cases with local and/or neurological symptoms.  The goalWe report a rare case of large enchondroma in the thoracic spine presenting severe upper back pain in a 49-year-old woman. Despite its benign-growing nature, enchondroma should be examined closely for signs of multiple enchondromatosis and enchondrosarcoma. Complete surgical resection is the treatment of choice for immediate relief of symptoms and avoidance of recurrence.


Acquired torticollis can be the result of several different pathological mechanisms. It is generally related to trauma, tumors, and inflammatory processes of the cervical muscles, nerves, and vertebral synovia. Although upper respiratory tract and neck inflammation are common causes of acute febrile torticollis in children, diseases with as yet undefined relationships may also result in torticollis. This is the case of spinal arachnoid cyst and pneumonia. Congenital torticollis usually arises from muscular fibrosis and neurological or bony abnormalities. Acquired torticollis typically results from inflammatory processes of the cervical muscles, nerves, or vertebral synovia. Retropharyngeal abscesses and upper respiratory tract infections are the most common infections associated with torticollis , 2.Benign paroxysmal torticollis is another common cause of acquired torticollis characterized by recurrent episodes of head tilt often accompanied by vomiting, pallor, irritability, or ataxia. It usually presents in the first few months of life and resolves without complications within 1–5 years .3/mm3, neutrophils 78%, erythrocyte sedimentation rate (ESR) 55 mm/hr, and C-reactive protein (CRP) 60 mg/dL. The magnetic resonance images (MRI) of the cervical and thoracic spine, taken in out center, were reevaluated; and necrotizing pneumonia in the left lower lobe of the lung and pleural effusion were revealed  19 × 10revealed . Necrotirevealed . Teicopl3 cells/mm3 with 86% segments, ESR was 55 mm/hr, and CRP was 27 mg/dL. The chest X-ray and thoracic CT scan revealed consolidation in the middle and lower lobes of the right lung (2) was observed on the upper cervical spinal cord, markedly compressing the front of the spinal cord , with no redness or tenderness detected. All of the other systems were normal, with exception of torticollis on left side. Her initial WBC was 17 × 10ght lung . Retroph Figures . SurgeryInflammatory conditions of the upper respiratory tract and neck are common result in the unilateral muscle spasm responsible for the head posture . Less coPneumonia presenting with torticollis is exceedingly rare, but seen must often in upper lobe pneumonia. To the best of our knowledge, isolated torticollis in pneumonia without any other symptoms has not been defined. Our patient had lower lobe pneumonia with no respiratory symptoms or auscultation findings. She was diagnosed incidentally with occult left lower lobe pneumonia via thoracic spinal MRI. Pneumonia may cause neck pain, stiffness, or torticollis secondary to compensatory muscle spasm, or referred pain. We believe that the pleural inflammation and irritation of the tracheobronchial tree caused the torticollis in our patient.Spinal arachnoid cysts are less commonly in the children; they present with slowly progressive quadriparesis or neck pain. Surgical treatment is necessary for symptomatic spinal arachnoid cysts . Spinal Torticollis can be symptomatic of a life-threatening condition. Vital signs assessment, systemic examination, and neurological evaluation must be carefully and systematically performed in order to exclude serious illness, also taking into account rare conditions. Early diagnosis and treatment can prevent future complications.


Alveolar soft part sarcoma (ASPS), a rare soft tissue sarcoma in children and adolescents, carries a poor prognosis. ASPS is an aggressive tumor of controversial histogenesis that, unlike other soft tissue sarcomas, tends to metastasize to the brain. A 9-year-old boy presented to our outpatient clinic in April 2009 with a chief complaint of a large painless mass in the left thigh whose size had increased significantly over the past 10 months. After staging the tumor, we performed open biopsy; the diagnosis was ASPS and he underwent wide local excision. In the course of 4-year follow-up by clinical and imaging studies, there was no evidence of early tumor recurrence or metastasis. Complete surgical resection is the treatment of choice in patients with ASPS. Soft tissue sarcoma is a type of malignant soft tissue tumor that is usually linked to the tissue of origin. In alveolar soft part sarcomas (ASPS), the alveolar description is based on the histological pattern of the tumor. ASPS, a rare, malignant soft tissue neoplasm that affects young patients, accounts for approximately 0.5-1.0% of all soft tissue sarcomas . While iThis 9-year-old boy presented to our outpatient clinic in April 2009 with a chief complaint of a painless large mass in the left thigh. He and his parents first noticed it as a small lump that increased in size over the next year till he visited our hospital. On examination, a mass measuring about 10 cm in diameter was noted at the anteromedial aspect of his left thigh about 15 cm above the knee joint. On palpation, it was firm and immobile. The mass was neither tender nor hot and there were no changes in the overlying skin .Magnetic resonance imaging (MRI) of the left thigh, performed later in the same week, revealed a well-defined soft tissue mass, heterogeneously enhanced, measured 6.5 × 4 × 10 cm, displaced and invaded the lower vastus medialis muscle. There was no involvement or invasion of underlying bones and no evidence of a periosteal reaction . The supThese findings were suggestive of a heterogeneous soft tissue mass, possibly a sarcoma. Although a close biopsy is a realistic option, we did an open incisional biopsy, performed through a medial longitudinal incision centralized over the mass; it revealed multiple fragments of white soft tissue. Microscopic study showed sheets of malignant cells arranged in a nested pattern. They were large, polygonal with distinct cell borders and eosinophilic cytoplasm; they were separated by thin-walled vascular channels .The tumor cells contained intracellular crystals that were PAS-positive and diastase-resistant . ImmunosOn whole-body bone (Tc99m), enhanced chest, abdominal, pelvic computed tomography (CT) and brain MRI scans, there was no evidence of distant metastasis.After complete radiological and histological studies, he underwent wide local excision via an elliptical surgical incision to include the scar and tract of the earlier biopsy. Then we identified major vessels and performed a wide resection taking care not to expose the tumor. On gross examination, the tumor was a 12 × 8 × 6 cm mass with an ellipse of skin on its surface .The results of microscopic examination were similar to those of biopsy. All surgical margins were free; the closest was located 4 mm medially.The postoperative course was unremarkable. The patient was seen every 5 - 7 months for clinical and radiological follow-up by local and brain MRI, CT chest, abdominal and pelvic scans. In the course of 48 months, there have been no signs of recurrence or metastasis.ASPS, first described in 1952 by Christopherson et al [Metastasis usually occurs late in the course of the disease and involves the lungs, bones, lymph nodes and brain. Metastasis to the liver has also been reported . To ruleThere is some controversy over the pathogenesis of ASPS. While Mukai et al  providedThe histological characteristics of ASPS include organoid nests of polygonal tumor cells encompassed by a dense capillary vasculature. The cells are of uniform size and shape and contain granular, eosinophilic cytoplasm surrounding a vesicular nucleus that hosts a prominent nucleolus. The designation “alveolar” derives from the pseudo-alveolar appearance .While the role of radio- and chemotherapy is yet to be established, surgical resection with margins microscopically free of tumor cells remains the ASPS treatment of choice and represents a strong indicator of the treatment outcome . SpontanAccording to Lieberman et al  who follIn conclusion, ASPS is a rare type of sarcomas that affects primarily the lower limbs. Periodic radiologic follow-up is mandatory to detect metastasis. While the role of radio- and chemotherapy is yet to be established, complete surgical resection is the treatment of choice in patients with ASPS.