Gliosarcoma (GS) belongs to World Health Organization grade IV neoplasm and displaying glial and mesenchymal differentiation. Only rare cases of GS have been reported in the brachium pontis and 4th ventricle region. Here, we report a rare case of GS located on brachium pontis region and extending into the 4th ventricle with well-differentiated cartilaginous metaplasia. A 28-year-old male patient experienced intermittent headache, vomiting, and gait disorders for 3 months. Magnetic resonance imaging (MRI) showed a heterogeneous ring-enhancement lesion with weak central enhancement in left brachium pontis and 4th ventricle region. Histology revealed the GS was constituted with glial and sarcomatous elements. After immunohistochemical analysis, a diagnosis of GS with cartilaginous differentiation was then made.Symptoms of GS, including headache, aphasia, hemiparesis, cognitive decline, and seizures, mainly determined by the location. The clinical manifestation and radiologic characteristic is not significantly different from that of glioblastoma. The grade of resection is the significant factor related to prognosis of GS, and the clinical effect of adjuvant radiotherapy and chemotherapy need further study. Reporting additional cases would be of great help in better understanding of this location and pathologic type of GS. Herein, we report a case of primary GS with a cartilaginous component located on left brachium pontis region and extending into the 4th ventricle in a 28-year-old male presenting with headache, vomiting, and walking unstable.Gliosarcoma (GS) is a rare form of primary central nervous system (CNS) malignant neoplasm consisting of malignant glial cells mixed with sarcomatous components. GS belongs to World Health Organization grade IV neoplasm and displaying glial and mesenchymal differentiation according to the 2007 World Health Organization classification of tumors of the CNS.A 28-year-old male adult experienced with intermittent headache, vomiting, and walking unstable for 3 months. Magnetic resonance imaging (MRI) showed a 2.2 cm × 3.6 cm × 3.5 cm heterogeneous ring-enhancement lesion with weak or unremarkable central enhancement in left brachium pontis and 4th ventricle region. MR images showed hypointense on T1-weighted MR images and enhancement on T1 gadolinium (Gd) image  component and at least one X100 magnification field of a sarcoma histologically consistent with a malignant fibrous histiocytoma or fibrosarcoma.19GS was constituted by glial and sarcomatous element, the glial component belongs to GBM and the sarcomatous element having the features of any sarcoma type. The sarcomatous components derived from the neoplastic transformation of the hyperplastic tumor blood vessels from the past main research literature.22 Neoplastic astrocytes could also differentiate into cartilage through mucopolysaccharide deposition, which can evolve to form a chondroid matrix.24Histopathology showed features of proliferating astrocytes with marked nuclear atypia, frequent mitotic figures, pseudopalisading necrosis, tumor giant cells, and angiogenesis. The neoplastic glial cells were intensely positive for GFAP and S-100 and the sarcomatous component negative for GFAP and S-100. CD68 and EMA showed negativity in both glial and sarcomatous elements. histomorphological features along with immunohistochemistry findings suggested a diagnosis of GS. Some literatures suggested osseous or cartilaginous component in GS may be indicate high malignant degree.26 What is noteworthy is that tumor excision and adjuvant radiotherapy seem to better the prognosis.26 This survival difference depends largely on extent of resection performed, adjuvant radiotherapy, and chemotherapy administered to a limited number of patients did not improve survival in this study.27The patient tolerated surgery and was discharged 2 weeks later. Because of postoperative hyperpyrexia and economic reasons, chemotherapy and radiotherapy were not given immediately at the time of hospitalization. Two months after discharge, there was no relapse of tumor shown by MRI evaluation. Additionally, the patient began to receive 60 Gy of radiation therapy and long-term follow-up is necessary. In terms of therapies and prognosis of GS, several literatures suggest that the prognosis of GS is similar than that of GBM.In general, to the best of our knowledge the literature contains only rare case reports of GS located on brachium pontis region and extending into the 4th ventricle with well-differentiated cartilaginous metaplasia. Reporting additional cases would be of great help in better understanding of this location and pathologic type of GS.


A color doppler ultrasound scan confirmed fluid and gas around liver and hilus lienis while the gallbladder cannot be detected. During Computed Tomography (CT) guided puncture positioning technology and setting a three-channel tube, about 400 ml of foul smell hazel turbid liquid was drained out. He was diagnosed as gallbladder perforation and he was underwent conservative treatment consist of drainage, banning diet, total parenteral nutrition and intravenous antibiotics. Then he recovered well within the subsequent 10 days and was discharged.A middle-aged man presented 1 day after being discharged from hospital with completing the first course of postoperative chemotherapy. He suffered a sudden persistent high fever and chills. It was noted that he had a history of a total gastrectomy (with D2 lymphadenectomy) 1 month ago. His admission bloods revealed total bilirubin was 142.2umol/L , indirect bilirubin of 107.6umol/L and white cell count of 20.05×10 Perforation of the gallbladder is a rare but life-threatening condition, which usually requires immediate surgical intervention , 2. SomeA 48-year-old man was presented to hospital because of persistent high fever and chills on the first day after completing the first course of chemotherapy. He has a history of a total gastrectomy with D2 lymphadenectomy 1 months ago and one course of postoperative chemotherapy which consisted of Oxaliplatin and S-1 because of gastric cancer. He has no liver or biliary disease history.At the time of admission, his temperature was up to 40 degree and blood pressure was 120/80mmHg. The heart rate and respiratory rate were 86/min and 22/min, respectively. The breath sound of lungs was clear. The whole skin appeared mild jaundice and sclera moderate yellow dye. There was no enlargement phenomenon of superficial lymph nodes. Abdominal examination revealed no pain or tightness. The liver and spleen was unpalpable. Murphy syndrome was negative. Other examination was unremarkable.9/L. His hepatitis virus examination was negative and renal function, Electrolyte was normal.Baseline investigations revealed a aspertate aminotransferase of 97U/L, and the total bilirubin was 142.2umol/L, indirect bilirubin of 107.6umol/L, white cell count of 20.05×10An urgent color doppler ultrasound of his upper abdominal was performed which demonstrated right upper abdominal structures disorders as evidenced by the strong echo gas. Parenchyma of Right liver lobe was uneven and the gallbladder cannot be detected. There was a small amount effusion around liver and hilus lienis Fig. .Fig. 1CoBoth for confirm diagnosis and further treatment, he underwent an emergency CT guided puncture positioning technology and setting a three-channel tube Fig. .Then aboConservative treatment consist of banning diet, total parenteral nutrition and intravenous antibiotics was complemented at the time of admission while records the drainage quantity everyday.His temperature dropped to 37 degrees,and the jaundice was significantly improved by the first day of conservative treatment. His drainage quantity and liver function tests were noted to be persistently elevated Table . HoweverReview normal every 4 weeks and he had begun the second course of chemotherapy which consisted of oxaliplatin and fluorouracil  500mg iv day1, 5-FU 2500mg civ day 2–3).Perforation of the gallbladder is one of the rare but severe complications of acute calculous cholecystitis, like empyema, gallstone ileus, cholecystoenteric fistula and emphysematous cholecystits . Acute aIt is relatively rare that the gallbladder perforation developed without inflammation, cancer, foreign, stone, or body trauma which makes our study more unique and innovative. Uncommonly, gallbladder perforation may be related to the obstruction of bile duct or the destruction of the gallbladder wall, such as acute cholecystitis, gallstones, parasites, abdominal surgery, gallbladder vascular compression or obstruction. Chemotherapy can lead to gallbladder injury, but, to our knowledge there is no report of postoperative chemotherapy-induced gallbladder perforation.There is a study suggest that the biliary stasis is an important contributing factor in postoperative gallbladder complications . BiliaryIt has been reported that the risk of suffering from acute postoperative acalculous cholecystitis was 3.1 % higher in patient with radical gastrectomy compared with patient with simple gastrectomy .A more suggestive cause of postoperative gallbladder perforation may be that the duodenum was excluded from the passage of large volumes of contents, resulting in possible stasis of bile and/or bacterial over growth within its lumen promoting bile inspissation,  which inIn this case, Gallbladder motility-related hormonal reduce, enteric nervous system function decline and pancreatic fistula may also play important roles in the gallbladder perforation.In this case, there was no stone shown in the patients with color Doppler ultrasound examination. The following several surgical morbidity factors should be considered.It’s worth noting that the gallbladder perforation occured on the first day after completing the first course of chemotherapy. Chemo-therapic cholecystitis cases are not rare. In this gallbladder perforation case, chemotherapy-induced gall bladder injury is an important contributing factor.In addition to the symptoms and signs, clinical picture and imaging is the first choice method for diagnosis of the disease. CT and ultrasonography are useful in making the diagnosis. Gallbladder wall thickening, peric-holecystic fluid collection, and a streaky omentum or mesentery is common findings of gallbladder perforation. Detection on imaging such as CT scans or color doppler ultrasound can be difficult in small perforations and the findings are usually non-specific that is, pericholecystic fluid/abscess and gallbladder cannot be detected. The presence of extra-luminal gallstones would be a suggestive sign, but it was not seen in this case . In the This case report is the first presentation of gallbladder perforation associated with operation and chemotherapy. The progression of gallbladder perforation may be mainly facilitated by operation, timing, scheme and dose should be more cautious when postoperative chemotherapy need to be implemented.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Fetus in fetu (FIF) is a rare congenital anomaly. The most common site at which FIF occurs is the retroperitoneum. The mechanisms underlying the development of FIF have not been fully elucidated. The monozygotic twin theory postulates that FIF results from the unequal division of the totipotent cells of the blastocyst. However, the monozygotic twin theory does not explain all cases of FIF.Herein, we describe the clinical characteristics of a 20-day-old infant with scrotal sac swelling. Ultrasonography and computed tomography revealed the presence of a mass consistent with a FIF rather than a teratoma. Surgical removal and a subsequent pathological evaluation demonstrated that the anencephalic fetus exhibited limb buds adjacent to a palpable vertebral column, supporting the diagnosis of FIF. The infant had an uneventful recovery and was discharged on the fifth postoperative day. In the present report, the pathogenesis, presentation, diagnosis, and management of FIF, as well as new concepts emerging in this area of research, are discussed.Although the majority of cases of FIF may be diagnosed preoperatively, FIF should be distinguished from teratoma because the latter has substantial malignant potential. The recommended treatment for FIF is complete resection. To confirm the diagnosis of FIF, pathological examination, karyotyping, serologic marker assessment, and DNA restriction site mapping should be performed after removing the mass. Although FIF is thought to be a benign disorder, follow-up is necessary as a precaution against malignant recurrence, which has been described once. A limited number of cases of FIF have been detected prenatally as cystic intra-abdominal masses growing inside the fetus.2 The oldest patient with documented FIF was a 47-year-old man.3 To distinguish a FIF from a teratoma, Gunaydin et al4 described a FIF as a mass containing a vertebral axis with organs and limbs arranged around it. Interestingly, even on pathological examination, approximately 9% of FIF cases in the literature reviewed by Gonzalez-Crussi5 lacked a vertebral column. Gonzalez-Crussi5 subsequently stated that the diagnosis of FIF should be applied to any fetiform structure exhibiting significant organogenesis and the presence of an axial skeleton. The majority of FIFs occur in the retroperitoneal space. Scrotal FIF is extraordinarily rare. We describe a scrotal FIF preoperatively diagnosed and successfully operated.Fetus in fetu (FIF) is a rare congenital anomaly, with an approximate incidence of 1 in 500,000 live births.A 20-day-old infant was hospitalized for scrotal swelling following birth. The boy was born full term via a normal vaginal delivery, with a birth weight of 3250 g. There was no history of twinning or teratomas in either parents’ families. He had an enlarged scrotum, which contained a 4.5-cm firm mass. His complete blood count and kidney–liver function test results were within the respective reference ranges. A coagulation profile test showed the bleeding time, prothrombin time, activated partial thromboplastin time, fibrinogen level, and fibrin split products to be normal. Urinalysis values were normal. His β-human chorionic gonadotropin (β-HCG) and serum carcinoembryonic antigen (CEA) levels were also within the respective reference ranges. His serum α-fetoprotein (AFP) level was 1200 ng/mL. Ultrasonography of the scrotal sac demonstrated a well-defined fetiform mass measuring 3.5 × 2.0 cm, surrounded by a fluid-filled sac containing bony elements resembling a vertebral axis , the embryos may develop separate placentas (chorion) and separate sacs (amnion). These embryos are dichorionic, diamniotic twins. On approximately the fifth day of the development, the blastocysts implant into the uterine endometrium; however, if one of the blastocysts derived from the conceptus implants into the other blastocyst instead of the endometrium, or if one blastocystis enclosed by the other implant close together, the subsequent development of such an inclusion body may result in FIF. From an embryological point of view, the existence of avertebral column in a fetiform mass may reflect its derivation from the primitive streak. The formation of the primitive streak normally begins during the third week of development in conjunction with gastrulation, which results in notochord formation and the subsequent development of the vertebral column and segmental axis. Therefore, a FIF may initially develop in a manner similar to that of normal fetal development. Interestingly, studies of genetic markers and genotyping suggest that host infants and their fetiform masses are genetically identical, which supports the monozygotic twin theory.21The pathogenesis of FIF remains controversial. Most investigators agree with the monozygotic twin theory, which postulates that FIF results from the unequal division of the totipotent cells of a blastocyst. According to the monozygotic theory, a FIF is a diamniotic, monochorionic, monozygotic twin that becomes incorporated into the body of a host twin following the anastomosis of the vitelline circulation.22 In other words, FIFs and teratomas may share an underlying pathogenetic mechanism. Proponents of the teratoma theory argue that FIFs may lack a spinal column and that teratomas may be highly differentiated and organogenic. Highly differentiated teratomas may contain a variety of organs, such as well-formed limbs, teeth, intestinal loops, a spinal cord, and brain-like tissue.8 FIFs may be found at many of the sites at which teratomas are located, including the retroperitoneum and ovaries. FIFs may be associated with teratomas, and retroperitoneal teratoma formation has been described following FIF removal.23 As there are many similarities between the 2 entities at the histological level, attaining a full understanding of the true nature of a FIF is an essential step toward improving treatment efficiency in the setting of this rare disease.As mentioned previously, the monozygotic twin theory does not explain all cases of FIF, and the theory surrounding a possible relationship between FIF and a highly differentiated teratoma remains controversial. Some investigators have hypothesized that a FIF represents a well-differentiated and highly organized teratoma.24 Eighty-nine percent of FIF lesions have been noted before 18 months of age.6 Most FIFs are located within the retroperitoneal space25; additional sites include the cerebral ventricles,26 mouth,27 neck,28 adrenal gland,29 liver,30 scrotum,31 pelvis,32 and mediastinum.33 As FIF is a benign disorder and has been described at various sites ranging from the cranial cavity to the scrotal sac, FIF lacks specific symptoms. Most FIFs present as asymptomatic, slow-growing abdominal masses. The symptoms of FIF are usually related to mass effect and include abdominal distension, feeding difficulties, emesis, jaundice, and dyspnea, which are caused by the compression of adjacent organs and tissues. If an FIF develops in the cranial cavity, neurologic symptoms may be observed.Most cases of FIF involve a single parasitic twin. However, >1 parasitic twin may be observed in the host body, although such occurrences are rare. To the best of our knowledge, the maximum number of FIFs documented previously is 11.34 Both ultrasonography and plain radiography may be useful in identifying these features. Different organs may be observed within a FIF, including limbs (86.0%) and a vertebral column (84.3%). Other organ systems include the central nervous system, gastrointestinal tract, blood vessels, and genitourinary tract. A FIF may be differentiated from a teratoma by the presence of vertebral organization with the limb buds and the other organ systems.To diagnose FIF, one of the following characteristics must be present: a mass enclosed within a distinct sac, either partially or completed covered by skin, with grossly recognizable anatomic features attached to the host via a pedicle containing a small number of relatively large blood vessels.35 to make a prenatal diagnosis of FIF. Ultrasonography may reveal the presence of calcific structures, but it may also result in misdiagnosis. Calcifications within the fetal abdomen may be caused by a teratoma, meconium peritonitis, a neuroblastoma, viral infections, and adrenal hemorrhage.36 Careful scrutiny of the calcifications, as well as magnetic resonance imaging (MRI), may enable clinicians to make a correct diagnosis. When fetal ultrasonography cannot distinguish between a cyst and a solid mass, fetal MRI may be useful.Ultrasonography may be helpful in the diagnosis of FIF as early as 21 weeks of gestation. It was first used by Nicolini et al37Ultrasonography and x-ray imaging may also aid in diagnosing FIF, as both modalities may help clinicians to visualize various aspects of the fetal skeleton, including the vertebral column. CT and MRI may allow for a more accurate diagnosis and may help define the relationship between the FIF and any adjacent intra-abdominal structures Figure . In patiComplete blood counts and kidney–liver function tests are frequently performed, and the results are usually within reported reference ranges. To distinguish a FIF from a malignant teratoma, both serum β-HCG and serum AFP levels should be tested. Serum AFP is an important indicator of the level of the malignancy of the teratoma, and elevated serum β-HCG levels may reflect either the presence of chorionic tissues within the mass or teratoma recurrence. Testing serum CEA levels may help clinicians and researchers to exclude specific types of abdominal tumors. The results of the serological examinations mentioned above may be helpful in making a preoperative diagnosis of FIF.38 described a fetiform teratoma with a highly developed axial skeleton that was distinguished from a FIF on the basis of zygosity.Despite the above-mentioned tests, making a definitive diagnosis preoperatively is often difficult. Postoperative pathological examinations, genetic marker assessment, and genotyping should be undertaken to confirm the diagnosis. Under gross pathological examination, various well-differentiated organs may be observed, including the vertebral column, limbs with digits, and other organs. Approximately 15.7% of FIF cases lack a vertebral column; however, such cases must have the aforementioned characteristics to be consistent with a diagnosis of FIF. Even if one mass with a well-differentiated axial skeleton is confirmed, the diagnosis of FIF may not be 100% certain. Kuno et al7 They are embryonal tumors that develop abnormally from the 3 primitive blastoderm cell layers. They may contain parts of or each of the 3 germ cell layers, with different degrees of maturity. The tissue types most often observed in highly differentiated teratomas are epithelium, hair, cerebral tissue, neurocytes, cartilage, bone, and teeth; however, teratomas lack an axial skeleton. However, because some FIFs lack a spinal column, some investigators have suggested that FIFs area type of highly differentiated teratoma.22 Making the diagnosis may be difficult in these cases because of the similarities in the entities’ clinical and radiological features, as well as in their appearances on histological examination. The most common sites are the sacrococcyx, anterior mediastinum, testicles, ovaries, and retroperitoneum, which are also sites at which a FIF may be observed. The incidence of teratomas is approximately 1/4000 live births,39 which is much higher than that of FIF. Approximately 7% of teratomas are malignant, whereas FIF is a benign disease. Although the pathology results may be similar between FIF and teratomas,15 karyotyping, serologic marker assessment, and DNA restriction site mapping may confirm that the mechanism underlying the development of FIF is different than that underlying the development of a teratoma. Regarding fetiform teratomas, which may have a spinal column, the most common location is the ovaries. This entity may be differentiated from a FIF via zygosity, as most fetiform teratomas are homozygous, and FIFs are heterozygous. Additionally, fetiform teratomas are most commonly found in women of reproductive age, whereas FIF is most often observed during infancy.40Teratomas are solid tumors that are common in infancy and are composed of various tissues foreign to the sites at which they arise.36 The differential appearances of these diseases on imaging studies are presented in Table Meconium peritonitis is the result of sterile chemical peritonitis, which results from an intrauterine bowel perforation and the subsequent exudation of meconium into the peritoneal cavity. The most common radiographic finding in newborns with meconium peritonitis is calcification, which is visualized as a classic eggshell calcification. Other x-ray imaging findings include ascites and pneumoperitoneum, which may also be observed in FIF. The calcifications associated with FIF, which most often resemble bony structures, are often used to distinguish a FIF from meconium peritonitis.Although FIF is thought to be a benign disorder, the recommended treatment for FIF is surgical excision to relieve obstruction, prevent further compression, and minimize the risk of hemorrhage. Significant blood loss during surgery is common; therefore, packing the abdomen may be helpful in achieving hemostasis for cases involving either the abdomen or the retroperitoneum. The resection of all parts of the mass is necessary because the diagnosis of FIF cannot be confirmed until a pathological analysis has been performed. Additionally, removing the entire mass may also decrease the risk of local malignant recurrence.41 The indicators for postoperative follow-up include serum levels of β-HCG and AFP, which are suggestive of the development of malignant teratoma. Although some cases of FIF are associated with high CEA levels,14 there is no evidence of a correlation between FIF and CEA. Either CT or MRI should also be performed at 3, 6, 12, and 24 months following therapy.The prognosis of most cases of FIF is good, although the prognosis of intracranial FIF is poor. Only 2 of the 18 patients with an intracranial FIF have survived, 1 of whom has only minimal head control and suffers from spasticity in the lower extremities.FIF is a rare entity that typically presents during infancy and early childhood. Current imaging modalities enable more accurate diagnosis and define the relation of the FIF with the other structures before surgery. FIF should be differentiated from a teratoma because of the malignant potential of the latter. Although the prognosis of FIF is generally good, cases of malignant degeneration have been described. Clinical and iconographic follow-up are therefore encouraged.


Hemangiomas are relatively common benign proliferative lesion of vascular tissue origin. They are often present at birth and may become more apparent throughout life. They are seen on facial skin, tongue, lips, buccal mucosa and palate as well as muscles. Hemangiomas occur more common in females than males. This case report presents a case of capillary hemangioma in maxillary anterior region in a 10-year-old boy.How to cite this article: Satish V, Bhat M, Maganur PC, Shah P, Biradar V. Capillary Hemangioma in Maxillary Anterior Region: A Case Report. Int J Clin Pediatr Dent 2014;7(2):144-147. Incidence of occurrence is seen in female than male.2 Eighty percent of hemangioma occurs as single lesion, but 20% of affected patients will have multiple tumors.2 They appear as pale macules with thread-like telangiectasia over the skin and mucous membrane.1 They are soft and painless. They are described clinically as a soft tissue mass, smooth or lobulated sessile or pedunculated with variable size. They may be smooth or irregular bulbous in outline.5 This article describes the case of a 10-year-old boy reported with a growth arising from attached gingiva in the maxillary anterior region.Hemangioma is relatively common benign proliferative lesion of vascular tissue origin, which may be present either at birth or may arise during early childhood.A male patient aged 10 years reported with a painless swelling in upper left anterior region from past 3 months. Soft tissue overgrowth was seen in relation to 22 and 63 involving attached gingiva . HistoryExtraoral examination including the lymph nodes was insignificant. Intraoral examination revealed a localized gingival growth which was pinkish white in color, arising from the attached gingiva of deciduous maxillary canine and maxillary 1st molar but was not interfering the occlusion. Tooth no. 64 was having proximal caries. There was grade II mobility with respect to 63 and 64. The oral hygiene was reasonably good.A provisional diagnosis of hemangioma was made due to its clinical appearance and its growth. Preoperative hematological test revealed all findings to be normal. The lesion was excised under local anesthesia  and 3. PSingle mass of tissue measuring 0.8 × 0.5 × 0.1 cm, pinkish white in color, firm in consistency, roughed surface and irregular in shape.The H&E stained soft tissue section shows lobulated cellular growth which is separated by fibrillar connective tissue. These lobules contain plump proliferating endothelial cells, combination of numerous well and poorly canalized blood vessels which are lined by endothelial cells.The overlying epithelium is parakeratotic stratified squamous type. The intervening connective tissue stroma is fibrillar composed of loose bundles of collagen fibers. It is sparsely infltrated with chronic inflammatory cells predominantly lymphocytes and plasma cells  to 9.On the basis of clinical and histopathological findings capillary hemangioma was made.6 Although the exact etiology is unknown, some authors believe that this lesion is not true lesion, but rather a developmental anomaly or hamartoma.7The word hemangioma comes from Greek word, hema – ‘blood’, angeio – ‘vessel’, oma – ‘tumor’. This terminology has been widely used in medical and dental literature. In 80% of cases, hemangiomas occur as single lesions. More over, capillary hemangiomas have a 3:1 female to male ratio and occur more frequently among Caucasians than other racial groups.89 Vascular lesions are generally divided into two categories: Hemangioma and vascular malformations. Hemangiomas are benign vascular anomalies characterized by benign proliferation of blood vessels. There are no welldefined criteria for the diagnosis and treatment of oral capillary hemangioma.10 The term hemangioma has been commonly used to describe a large number of vasoformative tumors. They are benign, vascular tumor that can lead to disfigurement or life-threatening consequences. Hemangiomas are usually classified into capillary, cavernous and mixed hemangioma.Vascular lesions are the most common congenital abnormality.1112 Hemangiomas generally do not affect adjacent bones and they can be shallow or deep. Sometimes, they can even involve all layers of the skin and offend the muscles.13The diagnosis of hemangioma is straightforward from the history and clinical examination. Hemangiomas usually appear within a few weeks after birth and have a growth rate that exceeds the growth rate of children.14At the cellular level, hemangiomas are characterized by increased birth and death rate of endothelial cells and proliferation of mastocytes during the postnatal proliferative phase in the lesion. Derived from young proliferating hemangiomas, capillary endothelium is easily grown in cell culture mediums and forms tubes. In accumulated hemangiomas, the number of mastocytes decreases and becomes equal to natural tissues. Hence, a normal hemangioma is an endothelial tumor with a very complex life cycle of cell proliferation and natural regression. Vascular malformations are the second major group of vascular lesions. In fact, they are an abnormal morphogenesis of blood or lymphatic vessels. Vascular malformations are present at birth. However, their clinical manifestations are not obvious sometimes until late infancy or even childhood.115 It is very rare to find hemangioma on attached gingiva. The same observation was seen by Mishra MB et al in a 30-year-old female patient.16 Our case describes an capillary hemangioma in a male child occurring on the attached gingiva of maxillary anterior region. Clinical findings in corelation with histopathologic findings confirm our case as capillary hemangioma.The literature suggests capillary hemangioma is observed as a relatively rare and uncommon pathologic entity in the oral cavity, but is a common soft tissue tumor of head and neck. Occurrence of capillary hemangioma with its primary location on gingiva seems extremely rare. The most frequent site of occurrence of intraoral hemangiomas are lips, tongue, buccal mucosa and palate.Regarding treatment, hemangiomas require no intervention. At an early age, spontaneous regression takes place. Only 10 to 20% require treatment, because of their size, location in their behavior. Different treatment procedures used in treating hemangiomas include microembolization, radiation, cryotherapy, sclerosing agents, surgical excision and recently Erbium lasers have been used. In the present case, the treatment compromised of complete surgical excision of the lesion. In the case discussed here, the patient is undergoing follow-up and no sign of recurrence has been seen.Any tumor, whether benign or malignant to be treated, requires early detection and diagnosis to avoid any complications at a later date. Capillary hemangiomas may be small but may cause problems in esthetics, mastication, phonetics and self-confidence in the human. There are many treatment options for hemangiomas where in surgical excision is one of the gold standard treatment regime.


Primitive neuroectodermal tumors (PNETs) and Ewing sarcoma (EWS) belong to the same family of malignant, small, round cell neoplasms of soft tissue or bone origin. EWS-PNETs that arise in the lung parenchyma involvement are extremely rare in adults. A case of a 32-year-old male presenting with chest pain and diffuse pulmonary nodules on chest X-ray and diagnosed with Ewing sarcoma-PNETs will be presented here. Ewing sarcoma family of tumors (ESFT) are mainly aggressive sarcomas of bone and are also arising in soft tissues . It is a75% of patients diagnosed with Ewing sarcoma are in the first 2 decades of their lives: the median age is 13 years and patients are rarely over 30 years of age . AccordiA 32-year-old male patient presented to the pulmonology department with the complaint of sleep apnea. Chest X-ray was performed due to the fact that the patient had a history of chest pain. Nodular opacities varying in size were observed in all lung zones on the chest X-rays . The finMicroscopically, the primitive cells were consisted solid sheets of cells divided irregular masses by fibrous strands. The tumour cells were small round/oval cells with scanty cytoplasm and uniform and uniform nuclei with dispersed chromatin. There were well developed vascular networks. Necrosis was common . ImmunohThe patient was admitted to the medical oncology clinic for chemotherapy. At the outpatient clinic, the patient underwent 6 cycles of chemotherapy consisting of vincristine, oxorubicin, cyclophosphamide, iphosphamide, and etoposide. A decrease in the sizes of the nodular lesions or radiological disappearance was observed in the postoperative 2nd month follow-up chest X-rays following regular chemotherapy . After tEwing sarcoma family of tumors are mainly aggressive sarcomas of bone and also arising in soft tissues, which share common features . Our pat EWSR1 gene translocation or amplification is the most reliable marker of PNETs, including those of pulmonary origin [In our case, although the patient had vertebral lesions, he did not complain of back pain. History of an operation following a fall from height 2 years ago may be related to the primary lesion, but data from the patient files could not be obtained. Ewing sarcoma is a vascular tumor that metastasizes to the lungs. For our patient, bone lesions are assumed to be operation-related and lung lesions are evaluated as primary lesions. Findings related to the tumor may not be observed on conventional X-rays. However, when present, it is observed as a nonspecific mass, the size of which may vary a wide range. Detection ofy origin . We did In our case, although the lesions (operation-related lesions) were also present in bones other than the lungs, biopsy was obtained from the largest lesions in the lungs, because the locations of bone lesions were not suitable for diagnostic resection. The lesions on vertebrae detected by PET scan originally thought to be related to the operation can be assumed to be Ewing sarcoma focus; however, after chemotherapy the lung lesions have shown an almost complete regression but vertebrae lesions did not change in size and detectability, which may indicate that the primary focus is not the bone. Ewing sarcoma is a malignancy that is sensitive to chemotherapy. Systemic chemotherapy is effective in reducing the local tumor size besides being effective on the microscopic and macroscopic metastasis. The treatment outcomes of patients who have metastasis are worse than patients who do not. The disease responded well to chemotherapy in our patient in spite of the diffuse lung involvement.In conclusion, ES is generally considered a childhood disease, but it can also be seen in adults. Thus, early diagnosis and effective treatment carry great importance. Our ES case diagnosed with surgical removal of the nodule had responded well to the treatment.


Osteoid osteoma is a benign bone tumor that appears most frequently in young patients. It is more common in males and it concerns mostly the long bones of the lower limb. A 20-year-old young woman presented to our outpatient department with pain in her left elbow. The symptoms began four years ago. At first, her symptoms were attributed to ulnar neuritis, confirmed by nerve conduction studies. In the following two years, she had undergone two surgical operations for decompression of the ulnar nerve. As a result, she reported poor results, which forced her to take frequently anti-inflammatory drugs for some years. When the patient presented to us, we planned a three-phase bone scan and an elbow MRI, which revealed the lesion. Based on the image findings of osteoid osteoma, we proceeded to the surgical removal of the tumor. Since then, the patient is pain-free and has a full range of motion of the affected elbow. Osteoid osteoma usually mimics multiple pathologies in the upper limb especially joint disease posing a challenge for the physician. The diagnosis requires high index of suspicion and a prompt diagnostic and surgical management. Osteoid osteoma is a relatively common benign bone tumor that appears most frequently in young patients. Its distinct feature is night pain that is relieved by anti-inflammatory drugs. It is more common in males and it concerns mostly the long bones of the lower limb (femur and tibia). Osteoid osteomas are very rare in the elbow region and even more in the bicipital tuberosity of the radius.We therefore present a rare case of a 20-year-old woman with persisted pain in her elbow as a result of an osteoid osteoma that was falsely treated surgically as ulnar neuritis.A 20-year-old right-handed woman presented with pain on her left elbow radiating along the radial side of the forearm since the age of 14. At that time, our young patient had undergone surgical decompression of ulnar nerve at the cubital tunnel for the first time due to ulnar neuritis confirmed by nerve conduction studies. One year later, medial epicondylectomy was performed additionally. Because of poor results, nerve conduction studies were repeated and concluded that there was no ulnar nerve lesion.The young patient presented at our department four years later and she was methodically examined to investigate her chronic pain. At first, a thorough history was taken. Apart from two surgical decompression procedures of the ulnar nerve, she reported no previous elbow trauma and no previous medical history. Physical examination was carried out and revealed full range of motion of the elbow. She had negative Tinel's sign and Phalen's test and no tenderness at the epicondyles.Since there were no significant clinical findings; a radiographic examination of her left elbow was undertaken. Radiographs showed an area of dense mineralization along with surrounding reactive sclerosis at the bicipital tuberosity of the radius .To confirm the diagnosis, further imaging with a three-phase bone scan  and an MSurgical removal of the tumor was decided. The lesion was excised en bloc, under general anesthesia and tourniquet application. We used demineralized bone matrix graft to fill in the gap of the radius. There was no need to transpose the bicipital insertion in the radius.Postoperatively, no evidence of elbow instability was observed. The diagnosis of the osteoid osteoma was confirmed histologically.Since then, our patient has been pain-free and reports no need for painkillers. There was no need for physiotherapy to obtain full motion of the elbow. The normal follow-up visits for the patient were one after 6 weeks and a second after one year. Eight years after the operation, the patient visited again our outpatient department, had a full range of motion, and reported not consuming any painkillers for her elbow. She was fully satisfied by the operation and the final outcome .Osteoid osteoma is a benign bone tumor that occurs in the first three decades of life in more than 80% of the cases. It is more common in males than females and it represents about 12% of all benign bone tumors. It is more frequent in the long bones of the lower limbs (about 50% of the cases) and the spine represents the second most frequent site [In our case, the tumor was in the upper extremity of the radius. The elbow is rarely a site of the tumor –4. In soThe diversity of the symptoms and the lack of high suspicion result in a delay of the diagnosis. Especially in cases where the tumor lays close to a joint, the diagnosis is misled by pain due to arthritis and all the patients are treated for arthritic conditions. This delay in the diagnosis is supported by the literature and varies from 15 months to two years! Our patient's diagnosis was established after four years, which appears to be the longest in the literature.X-rays are quite characteristic in cases of osteoid osteomas because of the nidus. However, in cases of small bones or when the lesion is located on the bone surface, signs inflammation of the periosteum are present without the characteristic nidus and diagnosis is harder. Our young patient represents such a case, whose X-rays revealed a reactive periostitis lesion that could be mistaken for any other kind of tumor. In order to set the right diagnosis apart from the X-rays, a CT scan and a three-phase bone scan can be very useful . Even wiThe treatment consists of removal of the tumor along with the nidus. Only in very rare cases removal is not indicated when the patient is painless and there are no symptoms. Until some years ago, en bloc removal of the tumor was the only treatment available. During the last decades, however, CT-guided mini-incision surgery and CT-guided radiofrequency ablation seem to become more popular –10. EvenThe diagnosis of osteoid osteoma can be difficult. Complicated anatomy and patients' tendency to correlate their symptoms with trauma or exhaustion often misguide clinicians and lead to ineffective treatments. Doctors should have high suspicion in cases of chronic bony pain and diagnosis can be reached following thorough physical and imaging examination.


Primary pancreatic leiomyosarcomas are rare malignant neoplasms with an aggressive course and a large size. A 56-year-old woman presented with an 8-year history of abdominal pain. Multislice computed tomography revealed a large heterogeneous mass with necrotic, calcified and macroscopic fatty areas. The tumor was excised. Histopathological evaluation revealed leiomyosarcoma of the pancreas. If a patient has a large size mass with a cystic-necrotic component, pancreatic leiomyosarcoma should be considered in the differential diagnosis list after excluding other common differential diagnoses. Leiomyosarcomas are rare malignant tumors of smooth muscle origin . They coPrimary pancreatic leiomyosarcoma is a rare malignant neoplasm. It accounts for 0.1% of all malignant pancreatic cancers . Only 45A 56-year-old woman presented with an 8-year history of abdominal pain especially on the left side. She was otherwise asymptomatic, with no nausea, vomiting, weight loss, diarrhea or jaundice. Laboratory findings were normal except for the increased amylase and erythrocyte sedimentation rate (ESR) level . The patient had diabetes mellitus , hypertension and ischemic hearth diseases.Chronic pancreatitis was considered clinically and ultrasound (US) examination was requested. US revealed a large heterogeneous hypervascular mass with central necrotic areas near the anterior of the left kidney and aorta. Computed tomography (CT) showed a large heterogeneous mass with necrotic and calcified areas at the pancreatic tail . After cPrimary pancreatic leiomyosarcoma is a rare malignant neoplasm. The mean age is 45.3 14-83 years). It is not easily differentiated from other pancreatic masses and it mimicks other primary and secondary neoplasms of the pancreas . Lesions3 years. MRI characteristics of leiomyosarcomas have been described in other organs . Most leUsual therapeutic approaches are pancreatoduodenectomy if the lesion is located in the head of the pancreas, and pancreatectomy if the lesion is located in the pancreatic corpus or tail. If the patient has widespread metastasis, surgery is not suitable.Muhammad et al. demonstrated an enlarged liver containing multiple lesions including the caudate lobe suggestive of metastases and a large enhancing soft tissue mass in the region of the body of the pancreas on CT scan in their patient who was admitted with a one-month history of 16 kilograms weight loss, epigastric pain, anorexia, abdominal fullness and jaundice. After ultrasound-guided liver biopsy, histological and immunohistochemical analysis confirmed leiomyosarcoma of pancreatic origin. The patient was offered palliative chemotherapy because of the metastatic phase. Then the patient died 3 months after the initial diagnosis . Nesi etPrimary pancreatic leiomyosarcomas are rare malignant neoplasms. They show an aggressive course and have a large size. If the patient has a large size mass with a cystic-necrotic component, pancreatic leiomyosarcomas should be considered in the differential diagnosis list after excluding other common differential diagnoses.


Introduction. Aggressive angiomyxoma is a rare mesenchymal neoplasm. Although benign in the majority of the cases, these neoplasms usually present a locally infiltrative nature and high rates of recurrence. Due to its rarity, misdiagnosis is a common problem. Case Presentation. We present one case of aggressive angiomyxoma in a 25-year-old pregnant woman. The patient presented with a large vaginal mass that was interpreted as a vaginal cyst. We performed surgical resection of the neoplasm and the correct diagnosis was only achieved after histological examination. With this case, we highlight the importance of considering this diagnosis in patients with genital and perineal masses of unknown origin and the impact of a correct preoperative diagnosis in patient's management and follow-up. Conclusion. Although aggressive angiomyxoma is rare, it should be considered in differential diagnosis of pelviperineal masses in young women. Its positivity to estrogen and progesterone receptors can justify enlargement and recurrence during pregnancy, although few cases are reported. Early recognition demands high index of suspicion for both gynaecologists and pathologists. Wide surgical excision with tumor free margins is the basis of curative treatment. Adjuvant therapy may be necessary for residual or recurrent tumors. Long-term follow-up is recommended. Aggressive angiomyxoma (AA) is a rare, acquired mesenchymal neoplasm with predilection for the pelvic and perineal regions, first described in 1983 by Steeper and Rosai. It is more frequent in young women with a female to male ratio of 6 : 1 and usually occurs around the third or fourth decades of life . AlthougWe present one case of vaginal aggressive angiomyxoma in a pregnant woman, whose initial diagnosis was different from the operative diagnosis, highlighting the importance of high suspicion indexes by both gynaecologists and pathologists.A 25-year-old healthy woman, 9 weeks' pregnant, presented to our institution with a progressive swelling in the vagina with exponential growth in the latest weeks. The patient also referred to dyspareunia and coital bleeding. Her gynaecological history included excision of a similar mass four years ago in another hospital, without a histological diagnosis. Clinical examination revealed a glistening, gelatinous, nontender vaginal mass. The tumor arose from the right lateral vaginal fornix and it was a large, well-circumscribed, pedunculated mass . UltrasoThe patient was submitted to a complete surgical excision of the mass during the 13th week of gestation Figures , which rAt macroscopic examination, the tumor measured 12 × 5 cm with a lobulated appearance, solid and rubbery. The cut surface was whitish and homogeneous. Microscopy revealed a paucicellular neoplasm composed of round and stellate cells with ill-defined cytoplasm and bland cytomorphology in a loose myxoid stroma. There was a prominent population of thick and thin-walled vessels, with no mitotic figures . ImmunohIn spite of not having access to the definitive diagnosis of the vaginal mass excised a few years ago, we believe that this might be a case of recurrent AA.The postoperative follow-up was uneventful and pregnancy was held to term without complications. The patient has a three-year follow-up free of disease.AA is an uncommon mesenchymal neoplasm that occurs predominantly in young female adults in the pelvic and perineal regions . This tuClinically AA typically presents a slow and insidious growth and is often asymptomatic . The sizGrossly AA is typically a poorly circumscribed lesion with a gelatinous, myxoid, or fibrous consistency . MicroscThe pathogenesis of these tumors is unclear. Recently an association between chromosomal 12 translocation 12q13-15) and consequent aberrant expression of the high-mobility group protein isoform 1-C (HMG1-C) has been suggested [-15 and cUltrasound evaluation of these lesions usually shows a hypoechoic mass that can have a cystic appearance. CT scans appearances are variable, but AA usually manifests as a well-defined homogenous hypodense mass relative to muscle, a hypoattenuating solid mass with swirling internal pattern with contrast or as a cystic mass with solid components . The mosIt is consensual that wide surgical excision with free margins is the treatment of choice for AA. However, complete excision is difficult in some cases . HormonaDue to low mitotic activity, it is unlikely that radiation or chemotherapy will be a useful adjunct to primary surgical treatment , 12. HowThe local recurrence rate for AA is about 30–40% and can occur after 10–15 years of primary excision (2 months to 15 years) . Some auIn conclusion, although AA is a rare mesodermal tumor, it should be considered in differential diagnosis of pelvic and perineal mass in young female patients. Its positivity to estrogen and progesterone receptors can justify enlargement and recurrence of these tumors during pregnancy, although few cases are reported. Early recognition demands high index of suspicion for both gynaecologists and pathologists. In the case we describe, angiomyxoma was not considered as a differential diagnosis and so we did not perform any additional studies as biopsy of the lesion or MRI. If clinically suspected preoperatively, CT scan or MRI should be done to help in planning the surgery.Wide surgical excision with tumor free margins is the basis of curative treatment of AA. We believe that high recurrence rates are due to an initial misdiagnosis and insufficient surgical excision. Adjuvant therapy may be necessary for residual or recurrent tumors. The hormone dependent characteristics suggest that hormonal treatment may be valuable.Long-term follow-up with MRI examination is recommended because the tumor may be indiscernible at pelvic and ultrasound examination.


Central accessory cusp in primary molars is an extremely rare condition which is due to abnormal proliferation and folding of inner enamel epithelium during morphodifferentiation stage of tooth development. The extension of the pulp in the cuspal area is often the reason for early involvement of pulp by the caries process. Therefore, early diagnosis and treatment planning is necessary to maintain the integrity of primary dental arch. This article reports such a case of central accessory cusps involving maxillary second primary molars.How to cite this article: Chandra B, Das M. Accessory Central Cusp in the Maxillary Second Primary Molars: A Rare Entity among the Rare. Int J Clin Pediatr Dent 2014;7(3):202-205. In this world, every normal creation has a possibility to present with abnormality to some extent, called rare and these rare events or things are always exceptional but special. After a long developmental process including histodifferentiation and morphodifferentiation a tooth gets its adult form. Any change or deviation of this pattern of development may alter the normal consequences. Both primary and permanent dentition may show structural variations involving crown and/or root of the affected teeth.1 The frequency of occurrence of these variations differ depending on their type; Talon's cusp (between 1 and 7.7%) and Carabelli's cusp (68%) and Leong's tubercle (8%).2Accessory cusps are occasional to rare. They present as minor variations to gross defects of the cusps. The cusp of Carrabelli of the molars is the most commonly reported accessory cusp, whereas talon's cusp in the incisors and leong's tubercle in premolars are not so common incidences. These morphological variations can be seen both in primary and in permanent dentitions.3 Several authors subsequently described the central cusp in their literature,45 in different teeth with different forms. Lau,4 Merrill6 and Schulze7 suggested different forms of central cusps and grouped them on the basis of their location and shapes with special regard to the premolars.The ‘central cusp’ is actually an occlusal supernumerary cusp, situated between the buccal and the lingual cusp tips of the premolars and molars and on the lingual surface of the incisors and canine. Leigh in 1975, first reported the central cusp as an enamel tubercle on the maxillary right third molar of an Eskimo's skull.8 During bell stage of tooth development, the altered proliferation and folding of a portion of the inner enamel epithelium and subjacent ectomesenchymal cells of the dental papilla forms a supplemental solid elevation on the crown surface. The embryological evidence suggests that tooth morphogenesis is characterized by transient signalling centers in the epithelium. They consist of epithelial cell clusters that correspond to the initiation of the formation of individual cusps.910 These signalling centers of epithelial cells and the primary and secondary enamel knots, serve a regulatory function.11It was assumed in the past that the formation of accessory cusp occurs due to over activity of the dental lamina. But now it is believed that the PAX and MSX genes are responsible for the abnormal cuspal morphology of the teeth.12The primary enamel knot regulates the advancing cuspal morphogenesis of the crown through expression of up to 20 molecules including fibroblast growth factors FGF-4 and FGF-9, transforming growth factor-b and bone morphogenic proteins BMP-2,4 and 7, etc. The mesenchy-mal BMP-4 induces expression of p21, possibly linked to the programme disappearance of the primary knot cells.13 The actual number of cusps are determined by the specific signalling molecules from the mesenchymal cells and simultaneous production of other molecular signals may induce differentiation of Hertwig's epithelial root sheath at the cervical loop.12Activator from the primary enamel knot regulates the expression of secondary enamel knots. The resultant cusp morphogenesis and positions appear to be determined sequentially, and cusps that form late in development, are typically small.The present article reports a case of bilateral anomalous accessory central cusp of the maxillary second primary molars including the associated clinical problems.The incidental clinical findings of central accessory cusps were observed in a 5 years old Bengali girl who reported to the Outpatient Department of Pedodontics and Preventive Dentistry, Guru Nanak Institute of Dental Science and Research, Kolkata. Her major complain was of pain in the right and left lower back tooth region since 1 month. The pain history revealed aggravation of pain at night with an associated swelling in relation to the affected teeth. No significant relevant medical and dental histories were reported by the parents.On clinical examination, patient had no relevant extraoral findings. The intraoral presentation revealed that the patient was in a primary dentition stage. There was presence of dental caries leading to dentoalveolar abscess with pus discharging sinus in relation to 75 and 85 . The cenThe central cusps were presented as a central projection (3 mm by 4 mm in size) on the occlusal surfaces of both the right and left maxillary second deciduous molars  respectiThe established treatment plan was pulpectomy of lower second molars followed by stainless steel crown; and light cure composite restoration of 55 and 65.In the first phase of treatment, pulpectomy of 75 and 85 were done after draining the sinus followed by placement of stainless steel crowns in both the teeth respectively.In the second phase of treatment, occlusal pit and fissure caries of 55, 65 were removed with the help of spoon shaped excavator  and no. 2 round diamond bur. The prepared teeth were then restored with the matched shade of packable composite (3M) preserving the cuspal morphology . Next, pNo two teeth are alike. Variation of teeth has been of enduring interest to the clinical practitioner and the laboratory scientist. The day-to-day variations of teeth that are seen is the norm. The odd, peculiar, and strange variations of teeth; are called anomalies.13 Dens evaginatus occurs mainly in the people of Asian descent, including Japanese, Chinese, Malay, Eskimo, Thai, Filipinos and American Indians . 3 The reported prevalence of dens evaginatus in the Asian population is 15%; with rare occurrence in whites. Histologically, these anomalies may compose of enamel, dentine and with or without pulpal projection into them .14Among different populations; the cuspal morphology of teeth varies and the accessory cusps are one group of variation which includes three patterns: cusp of Carabelli, talon's cusp and dens evaginatus. The central cusp on the occlusal surface of posterior teeth has also been given several descriptions, such as supernumerary occlusal cusp, occlusal tubercle, premolar odontome, tuberculated premolar and Leong's premolar (Scott and Turner 1997).215Though dens evaginatus is common in permanent dentition, the occurrence of central cusps in primary dentition is rare. According to the best of our knowledge, there are only two reported cases of dens evaginatus in primary second molars.7There are various types of dens evaginatus documented by the authors. According to Schulge, five types of dens evaginatus for posterior teeth are distinguished.They can be either A cone-like enlargement of the lingual cusp. A tubercle on the inclined plane of the lingual cusp. A cone-like enlargement of the buccal cusp. A tubercle on the inclined plane of the buccal cusp, or A tubercle arising from the occlusal surface obliterating the central groove.5 presence of central cusp or tubercle at the center of a tooth is often said to be due to abnormal proliferation of the epithelial fold during the morphodifferentiation phase of tooth development. The five categories are listed as follows along with their percentage of occurrence:According to Oehlers 1956; Wide pulp horns (34%) Narrow pulp horns (22%) Constricted pulp horns (14%) Isolated pulp horn remnants (20%) No pulp horn (10%)15 reported a case of accessory central cusps of the right and left maxillary deciduous second molars. He found that, the maxillary second primary molars when in occlusion, created depressions on the occlusal surface of both the opposing teeth (right and left mandibular 2nd deciduous molars). He also noted that, the cuspal tips of the accessory cusps showed some degree of attrition. In this case report, the accessory cusp is situated centrally on the oblique ridge and the cuspal tip extends above the level of other cusps. Looking at the clinical presentation, it is assumed that the cause of formation of the central cusp is similar to dens evaginatus. S Nagarajan MP et al (2009)An interesting finding related to the central cusp in this case was presence of occlusal caries in both the maxillary and mandibular second molars without involving any other teeth. This phenomenon reveals a strong correlation of caries and central accessory cusp. The reason behind the caries affecting the maxillary second primary molars can be attributed to the food lodgement in the deep pits and fssures surrounding the central accessory cusps. Another factor can be the improper cuspal contacts between the maxillary and the mandibular second primary molars. The depressions caused by the maxillary molars were not evidenced in mandibular molars as they were grossly carious. A long-term observation is required until the permanent successors erupt; to know the evidence of dens evaginatus or the occurrence of any other morphological abnormalities.The presence of accessory cusps in this case-report was an incidental or rather accidental finding. Though it is reported as rare but we feel many of these cases either go unnoticed or report to the clinic in such a grossly decayed condition that these cusps get obliterated. Hence, careful monitoring of such cases is needed and early and prompt management should be implicated as soon as such findings are noted. The presence of these accessory cusps make very difficult to maintain routine oral healthcare in those sites. As the pits and grooves surrounding the cusps are highly susceptible to caries it is necessary to seal them at once. Premature contacts or occlusal interferences if present should be removed to prevent development of temperomandibular disorders or habitual posturing of the jaws. The anomalous teeth should be kept under periodic monitoring to check for caries status and pulp vitality.


Background. Ewing sarcoma (ES) is a high-grade malignant tumor that has skeletal and extraskeletal forms and consists of small round cells. In the head and neck region, reported localization of extraskeletal ES includes the larynx, thyroid gland, submandibular gland, nasal fossa, pharynx, skin, and parotid gland, but not the external ear canal. Methods. We present the unique case of a 2-year-old boy with extraskeletal ES arising from the external ear canal, mimicking auricular hematoma. Results. Surgery was performed and a VAC/IE  regimen was used for adjuvant chemotherapy for 12 months. Conclusion. The clinician should consider extraskeletal ES when diagnosing tumors localized in the head and neck region because it may be manifested by a nonspecific clinical picture mimicking common otorhinolaryngologic disorders. Ewing sarcoma (ES) is a malignant tumor that consists of small round cells. It occurs in both skeletal and extraskeletal forms –3. The fA 2-year-old boy was admitted via our emergency services with a six-day history of an auricular mass which had not responded to three days of treatment with an amoxicillin clavulanate. There was no history of trauma, fever, or pain or of other features to suggest otitis media, or another upper airway infection. On physical examination, there was a painless, smooth, rubbery mass in the left postauricular area, pushing the conchal cartilage anteriorly and obstructing the EEC . ComputeThe tumor originated from the cartilage part of the EEC, pushing the conchal cartilage anteriorly. Pathological examination revealed a small blue round cell tumor. It was further using a panel of immunohistochemical stains. It stained negatively with markers for melanoma, lymphoma, neuroendocrine carcinomas, and rhabdomyosarcoma, but >50% of cells stained positively for CD99 and diffusely for FLI1 and vimentin. It had a high proliferative index with Ki-67 . DiagnosLeukemia markers were negative for CD13, CD33, CD34, CD117, and MPO to rule out acute myeloid leukemia (granulocytic sarcoma).To exclude a metastatic tumor from an unknown primary site, whole body imaging was performed. A CT scan of the thorax, abdomen, and pelvis was normal. Cranial magnetic resonance imaging (MRI) showed only secondary intensity changes at the operation site. A radionucleotide bone scan was normal . The fin2, adriamycin 75 mg/m2, and cyclophosphamide 1200 mg/m2 given alternately with ifosfamide 1800 mg/m2/day (5 days) and etoposide 100 mg/m2/day (5 days) every 21 days. The treatment duration was 12 months. Given that surgery had achieved complete gross and microscopic tumor removal, radiotherapy was not given.Following surgery, the patient was treated with the VAC/IE chemotherapy regimen for a year. This comprises vincristine 2 mg/mEES is an uncommon tumor, accounting for 1.1% of soft tissue malignancies . It usuaIts clinical and radiological features are nonspecific and a histological diagnosis is essential. In our patient, both the clinical and the radiological picture suggested an auricular hematoma. Following standard histopathological examination, the differential diagnosis included rhabdomyosarcoma, lymphoma, metastatic neuroblastoma, or a desmoplastic tumor. The definite diagnosis required immunohistochemical staining.EES, when localized, is treated by surgical excision with adjuvant or neoadjuvant chemotherapy. It is responsive to a number of chemotherapeutic drugs, including vincristine, doxorubicin, and cyclophosphamide . The VACThe following are reported to be good prognostic indicators: absence of metastases at presentation; tumor size less than 10 cm diameter; wide excision with clear surgical margins; chemotherapy given for eight or more cycles; a good histological response to induction chemotherapy; and combined modality treatment at presentation .In conclusion, we report a unique case of EES arising from the EEC. A high index of suspicion is needed to diagnose EES, especially when they present in unusual sites such as the head or neck. Their clinical features tend to be nonspecific, and they may mimic common benign conditions, including otorhinolaryngologic disorders.


Dear Editor,A white, 32-year-old man was admitted to the emergency department with severe painprincipally in the right inferior quadrant of the abdomen, abdominal distension andvomiting for one day.Abdominal radiography, ultrasonography and computed tomography demonstrated small bowelloops distension  and signAnatomopathological study in association with immunohistochemical analysis diagnosedangiomyolipoma (AML) as follows:Macroscopy: Bowel loop containing a non-encapsulated delimited,submucosal, polypoid yellowish lesion measuring 3.0 × 2.5 × 2.3 cm, with no sign ofmalignancy.Microscopy: Masson’s trichrome staining diagnosed AML compromising theentire intestinal wall, from the serosa to the mucosa.Immunohistochemical analysis: Desmin, HHF 35, CD31, CD34, protein S100,smooth muscle actin 1 to 4 = positive.,2.Intussusception is the invagination of a proximal intestinal segment with its mesentericfold with the corresponding vascu- larization into the lumen of the distal intestinalportion, which may lead to obstruction, inflammatory process and segmental ischemia. In this age group, it is estimated that in 90% of cases one findsorganic intraluminal causes called “intussusception heads”  orextraluminal causes  ,3.In adult individuals, this condition corresponds to about 5% of the general cases, out ofwhich only 1% of cases cause obstruction . The treatmentis generally surgical for organic causes, complications such as obstruction and intestinalischemia,3.Intussusception heads in the small bowel are most frequently associated with benignlesions, while in the colon they are most associated with either primary or secondarymalignant neoplasias ,4.The clinical signs of intussusception are related to the occurrence of subocclusion,obstruction and enterorrhagia,3.Intussusceptions are classified according the involved intestinal segment, as follows:enteroenteric, colo-colic, ileocolic and ileocecal intussusception. The diagnostic accuracy of ultrasonographyapproximates to 98%, but the method isoperator-dependent. Computedtomography presents accuracy of 58% to 100%.Typical radiological findings include: “target sign” and “pseudokidney sign”,6–8. Thesetumors and other lesions such as lymphangioleiomyomatosis and clear cell lung tumors werebrought together under the classification of PEComas (perivascular epithelioid celltumors).AMLs are benign mesenchymal tumors containing adipose, smooth muscle, epithelial andvascular cells,7.The prevalence of renal angiomyolipomas ranges from 0.3% to 3%. According to the literaturethis tumor is sporadic in about 80% of cases, and the remaining cases are associated withlymphangioleiomyomatosis and mainly tuberous sclerosis,6–8 (about 50 cases),7.Extrarenal AMLs are extremely rare, and the liver is the most reported site , and its occurrencein the gastrointestinal tract is rarely described,7,8.When located in the gastrointestinal tract their radiological diagnosis is hardly achievedbecause of their rarity and adipose nature, similarly to lipomas which are much morefrequently found


The authors report the first example of an adult achondroplastic dwarf with progressive quadriparesis secondary to atlantoaxial subluxation as a consequence of an os odontoideum. Actually, craniocervical region is a frequent site of compression and myelopathy in achondroplasia particularly in children as a result of small foramen magnum and hypertrophied opisthion. Moreover, very rarely in achondroplastic patients, coexistence of atlantoaxial instability as the sequel of os odontoideum can result in further compression of the already compromised cervicomedullary neural tissues, the scenario that has been reported only in five achondroplastic children. Herein, a 39-year-old achondroplastic male suffering such an extremely rare combination is presented. With C1-C2 screw rod instrumentation, atlas arch laminectomy, limited suboccipital craniectomy, and release of dural fibrous bands, reduction, decompression, and stabilization could be achieved properly resulting in steady but progressive recovery.  Achondroplasia is an autosomal-dominant condition characterized by dwarfism, macrocephaly, rhizomelic shortening of the extremities, saddle nose deformity, and trident hand all resulting from a defect in endochondral bone formation , 2. Os oHerein, a 39-year-old achondroplastic male with severe quadriparesis secondary to atlantoaxial dislocation due to an os odontoideum is presented. He was successfully managed with C1-C2 screw rod instrumentation and cervicomedullary decompression. To the best of our knowledge, this is the first adult example with this association in the literature. Further, in this report, we will describe the similarities and the dissimilarities between the clinical picture and the surgical strategies in these two different pathologies, in isolation and in their coexistence.This 39-year-old achondroplastic dwarf man was admitted with a 9-month history of progressive weakness of all his extremities following an insignificant fall from the stairs. Before this event, his developmental landmarks and intelligence had been normal. On examination, an incontinent and bedridden dwarf even dependent in feeding was noticed. His general weakness was graded 1/5 on the right and 0/5 on the left. His tendon reflexes were increased with bilateral extensor planter responses. MRI which was done as the first diagnostic method showed an os odontoideum with marked forward displacement of C1 in relation to C2 resulting in severe stenosis at cervicomedullary junction, accompanied with a hyperintense signal in T2-weighted images indicating myelopathy . LateralSurgery with atlantoaxial posterior instrumentation was decided. Subsequent to placement of screws in lateral mass of atlas, C2 pedicle was inserted on the right side. But on the left side, because of overriding vertebral artery translaminar screw was done. Later, after C1 arch laminectomy, the rods were placed and the nuts were tightened in extension. Optimal alignment could be achieved confirmed with fluoroscopy . HoweverPostoperative course was uneventful and was followed by steady neurological recovery of the patient. Plain radiographs revealed proper reduction of atlantoaxial dislocation Figures . ReconstAchondroplasia is the most common form of human short-limbed dwarfism and is one of a spectrum of diseases caused by mutations in the Fibroblast Growth Factor Receptor 3 (FGFR3) gene , 2.The condition is inherited as an autosomal-dominant trait but 80% of patients are the result of new mutations , 2.Os odontoideum or separate odontoid is defined as a round, ossicular remnant of the dens that is not fused to the body of the C-2 vertebra –5. WhethThe signs and symptoms of both os odontoideum and achondroplasia at craniovertebral junction encompass a wide spectrum but their clinical picture is classified in two main groups, symptomatic and asymptomatic , 2, 6–8.Many subjects with os odontoideum remain asymptomatic throughout their life and might be only discovered incidentally –27. On tIn symptomatic patients affected by these two pathologies, the clinical picture of cervicomedullary compression is caused in different ways. In achondroplasia, cervicomedullary compression is the consequence of the shortening of the skull base and clivus presented as abnormally small posterior fossa, a horizontally oriented posterior squama of the occipital bone with indenting effect due to its forward extension, thickening of the posterior lip of the foramen magnum, and the thick bands of connective tissue constricting the already compromised neural structures , 2, 6–8.Diagnosis of cervicomedullary compression due to os odntoideum is mostly done in the second to fourth decades of life, although it might become symptomatic and detected in the elderly , 11, 28.On the other hand, most of the achondroplastic patients presented with cervicomedullary compromise are children where its presentation in adulthood should not be regarded infrequent –9. In inIn both pathologies, minor trauma plays an important role on this critical area and may cause rapid deterioration of neurological function in an asymptomatic patient with danger of serious consequences and even death , 34.Plain standard and transoral cervical radiographs might show the round ossicle which is a sufficient clue for the diagnosis of os odontoideum and lateral flexion-extension radiographs can provide valuable information regarding the instability as well as reducibility of an os odontoideum –5, 11. IReconstructed CT images are of great value in showing details of the pathology and establishment of diagnosis in these two diseases . Os odonOnce these two pathologies become symptomatic, MRI becomes the modality of choice. Effacement of subarachnoid spaces at cervicomedullary junction and abnormal intrinsic cord signal intensity are cardinal MRI features. However, in both diseases either in isolation or in combination, dynamic MRI is of greater value since it can highlight the cord compression more precisely and demonstrate the impairment of the CSF flow more properly than static MRI .In both pathologies, indication for prophylactic surgery in asymptomatic patients is controversial.Generally, in an asymptomatic subject with a stable os odontoideum and in majority of asymptomatic achondroplastic patients with insignificant narrowing of foramen magnum, observation suffices. However, the patients or the relatives should be informed adequately of the risk inherent in conservative management.However, surgery is strongly advocated in asymptomatic patients harboring os odontoideum with apparent atlantoaxial instability where permanent damage of the spinal cord and even death in response to relatively minor to moderate intensity traumas have been documented by some authors , 34. TheNonetheless, the indication of surgery in symptomatic cases both in os odontoideum and in achondroplasia is straight forward. In achondroplasia, where progressive quadriparesis and documented sleep apnea both due to cervicomedullary compromise are the absolute surgical indications in achondroplasia, appearance of cervicomedullary myelopathy and vascular insufficiency are the indications of surgery in os odontoideum , 37, 38.In achondroplasia, the procedure typically consists of suboccipital craniectomy and C-1 arch laminectomy and release of dense fibrotic bands where duraplasty remains optional. Craniectomy should be limited to approximately 3 cm in order to avoid complications such as cerebellar sagging and craniocervical instability , 38.Notably, the surgical strategy in os odontoideum depends on reducibility of atlantoaxial subluxation determined in dynamic lateral radiographs. However, besides flexion-extension X-rays, skull traction is necessary in order to differentiate reducible from irreducible cases and for final assessment of reducibility especially after anesthesia , 39. In Irreducible atlantoaxial dislocation secondary to os odontoideum is a considerable challenge for the surgeons. Nowadays and in modern era release of C1-C2 facet joints is preferred to odontoidectomy for reduction of atlantoaxial subluxation due to os odontoideum –50. CorrRemoval of the os odontoideum to get rid of its persistent compressive effect on the cervicomedullary region has always been an option in irreducible conditions. This can be done through the transoral-transpharyngeal, transnasal, and transcervical corridors. Via these techniques, the odontoid, the coexisting offending anterior bony masses, redundant ligaments, granulation, and hypertrophic scar tissues should be removed till adequate ventral decompression can be achieved.Transoral odontoidectomy for os odontoideum dates back to 1968, when Greenberg et al. accomplished this procedure in two patients with IAAD due to hypoplastic separate odontoid successfully  in the lHowever, because of the drawbacks and morbidities encountered in the transoral-transpharyngeal corridor such as local infection, retropharyngeal abscess, meningitis, palatal dehiscence, pharyngeal dehiscence, delayed pharyngeal bleeding, velopalatine incompetence, and persistent hoarseness, the transnasal endoscopic odontoidectomy was introduced.Leng et al. described endonasal endoscopic resection of os odontoideum with fewer complications in comparison to the former routes . MagriniA much safer route is endoscopic odontoidectomy via transcervical corridor that has been described by Wolinsky et al. . This roRegardless of the corridor used for resection of the os odontoideum and other offending tissues, instability of the atlantoaxial joints exists and might become even worse. Therefore, after achievement of adequate decompression for avoiding serious sequels of further instability and in order to allow early mobilization and rehabilitation, a second operation for stabilization is mandatory. For this purpose, occipitocervical instrumentation and fusion are recommended by most authors –60.Coincidence of os odontoideum in achondroplastic patient and possibility of cervicomedullary compromise due to augmented effect of these two pathologies is an extremely rare entity revealing only five children with this combination in a very careful review of the literature –20. TherWhen a surgeon is faced with combination of these two pathologies attempts should be directed toward the management of both –20. ThisThe outcome before establishment of myelopathy is good in both instances in isolation where delay in diagnosis and surgery in myelopathic patients will require a longstanding course of physiotherapy and patience although some deficits might remain irreversible. Undoubtedly, prognosis in the presence of both pathologies is worse than each disease in isolation.In summary, presence of os odontoideum in achondroplasia is extremely rare event in particular in adults. Dynamic cervical radiographs and axial and reformatted CT as well as MRI are required for early detection of this coexistence. In coexistence of these two pathologies, instability due to os odontoideum can traumatize the already compromised cervicomedullary neural tissues. For adequate decompression of the cervicomedullary region, both pathologies should be surgically targeted. Eventually, surgical management will be more challenging than any of them in isolation; the strategy should include reduction and stabilization of atlantoaxial dislocation with subsequent small suboccipital craniectomy, C1 arch laminectomy, and removal of the dense fibrous bands. Degree of recovery depends on the neurological status at the time of surgery. Obviously, in those with disabling quadriparesis chance of good recovery is very low.


Calcifying aponeurotic fibroma (CAF) is a rare, locally aggressive fibroblastic lesion that occurs predominantly in the distal extremities of children and adolescents. In the present study, a case of pathologically proven CAF arising in the right little finger of a 69-year-old woman is presented. Physical examination revealed a firm, immobile, non-tender mass. Plain radiographs showed a faintly calcified soft tissue mass without bone involvement and computed tomography scans clearly demonstrated the presence of the lesion. Magnetic resonance imaging revealed that the lesion exhibited low to intermediate signal intensity on T1-weighted images and heterogeneous high signal intensity with small foci of low signal intensity on T2-weighted spectral presaturation with inversion recovery images. Contrast-enhanced fat-suppressed T1-weighted images demonstrated intense heterogeneous enhancement throughout the mass. The patient underwent an excisional biopsy. Histologically, the tumor showed a biphasic pattern, composed of a moderately cellular fibromatosis-like component and irregular calcified areas with polygonal epithelioid cells. There has been no evidence of local recurrence four months following surgery. To the best of our knowledge, this case report describes the oldest patient with this condition. Calcifying aponeurotic fibroma (CAF), also known as juvenile aponeurotic fibroma, is a rare, benign fibrous tumor that occurs in the distal extremities, in particular on the palmar surface of the hand. It usually presents as a slowly growing, painless, firm mass in children and adolescents, with a slight male predominance . Due to A 69-year-old woman presented with a 7-year history of a slow-growing, painless mass in the radial side of the right little finger at the proximal interphalangeal joint. There was no history of antecedent trauma. Physical examination revealed a firm, immobile, non-tender mass. Neurovascular examinations were normal and the results from laboratory tests were within the normal limits, including C-reactive protein levels and white blood cell counts. The patient’s past medical history was unremarkable.Plain radiographs revealed a faintly calcified soft tissue mass without bone involvement , and comAn excisional biopsy was performed under general anesthesia with tourniquet control. A midlateral incision was made on the radial side of the right little finger. The mass was adherent to the surrounding fibrous tissues; however, the mass was fully excised. No adjacent bone involvement was noted. Histologically, the tumor showed a biphasic pattern, composed of a moderately cellular fibromatosis-like component and irregular calcified areas with polygonal epithelioid cells  and fociThe postoperative course was uneventful, and there was no evidence of local recurrence four months following surgery.Written informed consent for publication was obtained from the patient and this study was approved by the Institutional Review Board .CAF occurs in patients over a wide age range; however, it is most common in children and adolescents . The medCAF grows in a diffuse, poorly circumscribed manner and is often attached to the aponeurosis, tendons or fascia. Complete local excision is the treatment of choice. The histological examination of the lesion reveals two components: i) fibromatosis-like spindle-shaped cell elements, and ii) nodules of calcification, accompanied by more rounded, epithelioid cells . Two phaThe pathogenesis of CAF remains uncertain; however, a fibroblastic/myofibroblastic origin has been suggested . It has Only a few studies have described the imaging features of CAF. Plain radiographs may show a nonspecific soft tissue mass, with a variable extent of finely stippled calcifications , and bonIn older patients, the distinction of CAF from soft tissue chondroma may be difficult. As with CAF, soft tissue chondroma occurs most commonly in the finger or hand, with no connection to the underlying bone . HoweverIn conclusion, in the present case report the imaging findings of CAF with pathologic correlation in an elderly patient are described. Although rare, CAF should be considered in the differential diagnosis of elderly patients with a calcified soft tissue mass, in particular in the finger or hand.


Spleen vascular tumors such as hemangiomas, albeit rare, can present during neonatal period with unexplained circulatory shock. We present a case of a newborn with refractory hypovolemic shock and acute abdomen that underwent emergency splenectomy due to spontaneous rupture of a splenic hemangioma.  A 24-day-old male baby presented to us with acute abdomen and circulatory shock. The baby was born vaginally to a healthy mother at term after an uneventful prenatal course. Several prenatal ultrasounds were performed and reported as normal. Apart from being small for gestational age , birth and clinical histories were unremarkable and newborn and mother were discharged 24 hours after birth. Before presentation, the patient became suddenly pale, vomited once and was treated by his pediatrician with antiemetic drugs, lactobacilli and simethicone with slight clinical improvement. A few hours later, he again became irritable and taken to the referring hospital, where he was found to have signs of shock; he received red blood cell and fresh frozen plasma transfusions, respiratory support, and broad-spectrum antibiotics. Given the persistence of hemodynamic instability and tender abdomen, the patient was referred to our hospital suspecting bowel perforation. The patient was pale, with tender abdomen but there was no ecchymosis or palpable masses on abdominal examination. Ultrasound abdomen showed abundant free fluid. The caregivers categorically denied abdominal trauma or falls. Emergency laparotomy revealed hemoperitoneum and a laceration of the spleen at its lower pole . Splenectomy was performed. Patient slowly recovered from shock and its clinical derangements, and was discharged uneventfully after 10 days. Penicillin was started and immunization against encapsulated agents was given at age 2 months. Histopathology analysis reported a ruptured cavernous hemangioma of the spleen .The spleen can be involved by various conditions such as infectious processes, inflammatory diseases, vascular, or hematologic conditions and neoplasms . The incidence of splenic hemangioma ranges between 0.02% and 0.16% ; it is seldom reported in the neonates. It may present as incidental lesions or it may produce significant splenomegaly and predispose to splenic rupture [3]. Other presentations include Kasabach-Merritt phenomenon, portal hypertension,[5] anemia, thrombocytopenia, coagulopathy and bleeding due to sequestration and/or destruction of blood components within the tumor [3]. Splenic hemangioma can have various complications such as splenic thrombosis, infarction, and spontaneous rupture which may be fatal if not dealt emergently [4]. Hypovolemic shock due to abdominal hemorrhage in neonates is very rare and can be caused by obstetric trauma, [6] laceration of umbilical artery during catheterization or unidentified causes . Ruptured splenic hemangioma is extremely rare cause of abdominal hemorrhage . Regardless of the cause of hemoperitoneum the morbidity and mortality can be very high if not identified and managed timely. Patients with splenectomy are prone to overwhelming infections, therefore, must be vaccinated as soon as possible, particularly against encapsulated microorganisms. In neonates, vaccination should be done at two months to get proper titers of antibodies [9]. To conclude, ruptured vascular malformation of spleen in neonates is very rare entity, and it must be considered when facing a neonate with unexplained shock and acute abdomen.Source of Support: NilConflict of Interest: Nil


Low-grade endometrial stromal sarcoma is very rare and difficult to diagnose in the early stage. A standard treatment has not been established. In this case report of a patient with long-term survival, we describe an effective treatment for advanced low-grade endometrial stromal sarcoma.A 24-year-old Japanese woman who presented with prolonged menstruation was diagnosed with leiomyoma on the basis of a specimen resected transvaginally. She underwent ten resections in 10 years without a malignancy diagnosis. During this period, she gave birth. At age 34 years, she visited our hospital, complaining of lower abdominal pain. A 10cm tumor was detected behind her uterus. The disease was diagnosed as an advanced malignant ovarian tumor before surgery. A laparotomy was performed, with many remnants left in the abdominal cavity. The final diagnosis was advanced low-grade endometrial stromal sarcoma. After 12 cycles of gemcitabine and docetaxel combination chemotherapy, the tumor disappeared completely. A retrospective pathological review of the specimens resected transvaginally showed that the tumors included low-grade endometrial stromal sarcoma elements. When the patient was age 42 years, the sarcoma recurred. It was detected around the right diaphragm and liver. Despite administration of gemcitabine and docetaxel, ascites and pleural effusion accumulated. Administration of medroxyprogesterone acetate, leuprorelin acetate, and anastrozole gradually reduced the ascites and pleural effusion. In addition to the three hormone drugs, 18 cycles of paclitaxel and carboplatin were administered. The patient recovered from her critically ill state and is currently alive with reduced tumor at age 45 years.Our patient with low-grade endometrial stromal sarcoma whose disease began in her youth gave birth and experienced long-term survival with surgery, chemotherapy, and hormone therapy. Low-grade endometrial stromal sarcoma (LGESS) is very rare and tends to grow slowly. It develops in the supporting connective tissue (stroma) , 2. KossWe report a case of a patient with LGESS who has experienced long-term survival. She gave birth in the early stage of LGESS, received chemotherapy after surgery in the advanced stage, and was revived by hormone therapy and additional chemotherapy in the recurrent stage. We hope this case report plays a role in establishing effective treatment for LGESS.We report a case of a 24-year-old Japanese woman, gravida 0, who had no relevant past medical history or family history when she became ill. She complained of prolonged menstruation and was examined with Cusco’s speculum. The patient presented with a uterine cervical tumor and was diagnosed with uterine leiomyoma. She underwent her first transvaginal resection of the tumor with a myomectomy and endometrial curettage. The pathological diagnosis was leiomyoma and endometrium, no malignancy, as a benign tumor. She underwent four additional tumor resections: polypectomy, two myoma resections, and a submucosal leiomyoma resection via hysteroscopy. The resections were performed at intervals of 10 months, 9 months, 9 months, and 6 months, respectively. After these resections, she gave birth to a 3396g boy via cesarean section at age 28 years. During her pregnancy, she was diagnosed with oligoamnios. The birth took place at the 39th week of pregnancy. At age 30 years, the patient developed a sixth tumor (a fibroid in statu nascendi), which was removed by exeresis at age 30 years. Four subsequent tumor resections were performed, at intervals of 23 months, 4 months, 12 months, and 3 months, respectively. When the seventh tumor was resected by polypectomy at age 32 years, the pathology report revealed for the first time the high density of the stroma and the necessity of careful follow-up. At the time these ten tumor resections were performed (five before and five after childbirth), the tumors were not diagnosed as malignant. The correct diagnosis was obtained by analysis of advanced-stage surgical specimens. The discovery of minimal elements of LGESS by a retrospective study of the transvaginally resected tumors led to a correct diagnosis of early-stage LGESS.The patient visited our hospital, complaining of lower abdominal pain at age 34 years and was found to have a tumor 10cm in diameter behind the uterus  version 3, even after granulocyte colony-stimulating factor was administered. No recurrent tumor was detected for the next 18 months.A small accumulation of fluid was detected in the pouch of Douglas when the patient was age 42 years. Computed tomography (CT) revealed recurrence of the tumor in the form of many disseminations in the right pleura, peritoneum, right diaphragm pleural and peritoneal surface, and liver surface Fig. . Althoug2 and carboplatin (area under the curve =4) combination chemotherapy (TC) was started with MPA, leuprorelin acetate, and anastrozole. The dose of TC was reduced because during the time five cycles of GD were administered after the recurrent tumor on the ileum was resected the patient’s neutropenia gradually worsened to CTCAE grade 4. The tumor size and state were confirmed by CT after every three cycles of chemotherapy. The tumor gradually reduced, and the liver returned to the normal position. Chemotherapy was stopped after 18 cycles  Fig. . The staLGESS is a rare disease. Several case reports describing pregnancy after contracting this disease have been published –6. In ouThe tumors, which had the appearance of uterine myomas or cervical polyps, grew repeatedly during the early stage over a period of 10 years. The pathological diagnosis of LGESS could not be made until a hysterectomy was performed. The usual pathological features of LGESS are tongue-like myometrial invasions with prominent lymphatic involvement and a whorled pattern of oval to short spindle tumor cells around the arterioles. When these characteristic features are identified, the pathological diagnosis is usually straightforward. However, myometrial invasion is difficult or almost impossible to identify in myomectomy or polypectomy specimens. In addition to the whorled pattern, LGESS has a wide spectrum of less common histological patterns, including fibroblastic or myofibroblastic appearance, epithelioid arrangement of the tumor cells with abundant clear or eosinophilic cytoplasm, hypocellular myxoid or fibrous change, sex cord–like elements, glandular differentiation, and pseudopapillary pattern . In the It was difficult to find effective treatments in a search of the literature because articles on this topic have been published only sporadically and report cases only at the time the tumor had worsened to the advanced stage. Although surgery, radiation therapy, chemotherapy, and progestin have been reported as treatments for LGESS, the effect of these treatments has been limited and ineffective in advanced cases. When the next treatment was investegated after the first abdominal surgery, GD had been reported as an effective therapy for unresectable leiomyosarcoma in a phase II trial . GD was In the second recurrence of our patient’s disease, GD treatment did not produce a response. It was considered that the tumor had acquired anti-cancer drug resistance against GD because of repeated GD administration. Because MPA as a next treatment was not effective enough, leuprorelin acetate was administered in addition to MPA. After administration of leuprorelin acetate, the patient’s pleural effusion and bleeding accelerated. Leuprorelin acetate caused a flare-up of gonadotropin secretion. However, the bleeding stopped after the period of flare-up of gonadotropin secretion, and the pleural effusion decreased gradually. There was no change in the patient’s ascites, though, so anastrozole was administered in addition to MPA and leuprorelin acetate. This treatment was effective in reducing the ascites, and the patient could be discharged from the hospital. The three drugs did not produce side effects, except for bleeding caused by leuprorelin acetate.In the next step, TC was administrated in addition to hormone therapy. TC was selected because it is a standard treatment for uterine corpus cancer and is easy to administer. TC with MPA, leuprorelin acetate, and anastrozole is effective for patients with LGESS resistant to GD chemotherapy. Although our patient experienced the typical side effects of chemotherapy, such as alopecia, bone marrow suppression, and numbness, she underwent 18 cycles of TC chemotherapy without any more severe side effects than were expected. A reduced dose would be good for the patient to continue the repeated chemotherapy. The patient has survived more than 10 years after the disease spread throughout her abdominal cavity. However, this chemotherapy and hormone therapy will cease to be effective when the patient eventually develops resistance to the anti-cancer drugs currently used to treat her. A new treatment option is desired for the next recurrence of LGESS.The diagnosis of LGESS in the early stage is very difficult only on the basis of transvaginally resected specimens. Our patient gave birth in the early stage of the disease. Long-term survival was accomplished via surgery, GD in the advanced stage, and TC combined with hormone therapy for the GD-resistant tumor in the recurrent stage. This report should give hope to patients with LGESS. We hope that combination chemotherapy together with hormone therapy can be established as an effective treatment for advanced LGESS.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.