Taking into account the great number of skin malignances that occur in the head and neck regions, problems related to their surgical treatment represent a constant concern for plastic surgeons. They have to deal with the difficulties of radical excision and also with reconstructive possibilities. More than 2/3 of these malignances are basal cell carcinomas, which, if left untreated might become very invasive, surgical treatment being more difficult in such cases. Recurrent carcinomas combined with radiation injuries represent a serious challenge even for experienced surgeons regarding the size of the defect and anatomical structures involved. In this paper we present a case of a patient with basal cell carcinoma of the parietal region and the reconstructive treatment involving neurosurgery and plastic surgery team approach, using microsurgery techniques. We also present the difficulties of the case as well as the final outcome that we consider the best option for the patient. The neurosurgical stage consisted in fongus removal, excluding all brain tissue located outside the dural limits, craniectomy, duraplasty and cranioplasty. Because of the large size of the defect and also because the local resources were exhausted we chose as a covering solution the free tissue transfer consisting in a latissimus dorsi musculocutaneous flap. The difficulties of these cases consist both in the aim of radical excision and the limited reconstructive options. In this case, our collaboration with the neurosurgery team proved to be crucial, permitting us to solve this case in a single operative time, with deep excision, reconstruction of the dura mater and cranioplasty, and reconstruction of the soft tissues with microsurgical free transfer. Taking into account the great number of skin malignances that occur in the head and neck regions, problems related to their surgical treatment represent a constant concern for plastic surgeons. They have to deal with the difficulties of radical excision and also with reconstructive possibilities. More than 2/3 of these malignances are basal cell carcinomas, which, if left untreated might become very invasive, surgical treatment being more difficult in such cases. Recurrent carcinomas combined with radiation injuries represent a serious challenge even for experienced surgeons regarding the size of the defect and anatomical structures involved.We are presenting a case of a 68 year old patient, who presented in our clinic with a basal cell carcinoma of the parietal region in 2004. He suffered multiple operations consisting in excision and cover with local flaps and skin grafts. Nowadays, he presented with local recidiva, necrotic infection in the exposed calvaria and a fungoid mass in the frontal part of the brain.After clinical and paraclinical investigations, including CT and MRI, and also careful preoperative preparation, we performed the operation together with the neurosurgery team.The neurosurgical stage of the operation began under general anesthesia with orotracheal intubation, and consisted in the next events:A rigorous mechanical and chemical cleaning on the exposed surfaces, with peroxide and povidone iodine, in order to displace all debris and to appreciate exactly the limit of the living tissue. The inspection shows a tiny layer of dura mater, covering the superior sagital sinus and the convex aspects of both hemispheres, and a cerebral fongoid mass located on the right side, frontally, of about 3.5x5.5x3cm (see MRI images).The bony defect of the calvaria was carefully inspected. The pericranium and the remnants of the scalp were detached circularly and isolated with cotton pads. The calvarial resection progressed from the frontal bone to the occipital, bilaterally, and with the aid of a Gouge rongeur the bone was sacrificed until bleeding, as the normal bone, was actually increasing significantly the defect to safe margins. The removed pieces were infected, necrotic and presented many micro-abscesses. The normal dura mater was exposed below the bony resection.A new cleaning procedure with iodine was applied, especially on the bony edge.The next step consisted of fongus removal, excluding all brain tissue located outside of the dural limits, proceeding to an adequate hemostasis, but not opening the subdural spaces (this method prevents CSF further leakage or fistula).The entire surface of the defect was thereafter, covered with Duraform®, and abundantly placed over all. Saline 9 ‰ was used for humidification. Another layer of dural substitute made from cabalin pericardium, Neuropatch®, was prepared and applied over Duraform®, to seal any potential source of CSF leakage.The last step was a cranioplasty, made from acrylic radio-opaque cement, Aminofix ®, adjusted on the place, which entirely covered the defect bone. The cranioplasty was fixed with the aid of Nylon 5 stitches, tractioned from the healthy parts of the pericranium, in an X shaped manner.Because of the large size of the defect and also because the local resources were exhausted we chose the free tissue transfer consisting in a latissimus dorsi musculocutaneous flap as a covering solution. The criteria for choosing this flap were: the large size of the defect, the great reliability of this flap considering the radiation injuries in the recipient area and also less morbidity of the donor area.The flap was harvested from the left side of the back, and was about 35 cm long and 25 cm wide, with careful dissection of the toracodorsal pedicle and also the pedicle for serratus anterior muscle. After harvesting, the flap was sutured in the new position and the vascular anastomoses were performed in the next manner: the toracodorsal artery was sutured end to end to the left superficial temporal artery, with atraumatic 8.0 wires, the toracodorsal vein was sutured end to end to a commitant vein of the superficial temporal artery and the vein from the pedicle for serratus anterior was sutured to another commitant vein. We decided to perform two venous anastomoses taking into account the large size of the flap, and also the bad condition of the recipient vessels.The microsurgical stage of the operation took place without major problems, both arterial and venous anastomoses were patent and the flap was sutured to cover the defect. The donor area of the flap was closed with skin grafts.The local postoperative evolution was good, without major complications, only with a short period (a few hours) of venous congestion of the flap. In the next days the flap showed no signs of vascular suffering, with complete integration. Postoperative check-up consisted of general monitoring of the patient, blood tests, ESR, etc. He received Avelox® 400 mg a day, for 21 days, in order to cover any septic potential complications to an irradiated and potentially immuno-compromised patient.The meningism signs and thermal curve were controlled twice a day. A CT scan was ordered in the third day, and in the 14th day after operation. Images were evaluated, and compared, and blood or fluid collection inside the skull was excluded. Cranioplasty acrylic plate remains on place. A right frontal paraventricular hypodense area, below the operated fongus, remains as the previous, before surgery. Patient presents a moderate frontal syndrome, unchanged, as before surgery, without any motor or sensory deficit, in direct relation with surgery.Recurrent, irradiated basal cell carcinomas of the head which involve both skin and subjacent structures  need large excisions, followed by large defects with serious covering problems.Difficulties of these cases consist both in the aim of radical excision and the limited reconstructive options.In this case, our collaboration with the neurosurgery team proved to be crucial, permitting us to solve this case in a single operative time, with deep excision, reconstruction of the dura mater and cranioplasty, and reconstruction of the soft tissues with microsurgical free transfer.


Tumors of the vermiform appendix are relatively rare. More than 50% of appendiceal tumors are carcinoid tumors. The author reviewed 512 consecutive pathological specimens of appendectomies in last ten years in our pathology laboratory in search for appendiceal tumors. As the results, 4 cases (incidence: 0.8%) of non-invasive adenocarcinoma were found. No other tumors including carcinoid tumors were recognized. The age of the 4 patients with adenocarcinoma was 48, 39, 84 and 86 years, respectively. Male to female ratio was 3:1. The clinical diagnoses were acute appendicitis in 2 cases and suspected malignancy in 2 cases. The post-operative outcome was good without metastasis, recurrence, and pseudomyxoma peritonei. Pathologically, all the 4 tumors were non-invasive adenocarcinomas: 2 cases were flat type adenocarcinoma, 1 case was papillary adenocarcinoma, and 1 case was mucinous adenocarcinoma. Immunohistochemically, expression of p53 protein was observed in all the 4 cases, and Ki-67 labeling ranged from 40% to 90%. The results suggest that incidence of appendiceal adenocarcinoma was 0.8% of all appendectomies, and that non-invasive adenocarcinoma of the appendix shows variable morphologies, and that postoperative clinical outcome of non-invasive appendiceal tumor is good. Tumors of vermiform appendix are relatively rare conditions. More than 50% of appendiceal tumors are carcinoid tumor . AdenocaThe author reviewed 512 consecutive pathological specimens of appendectomies in last ten years in our pathology laboratory in search for appendiceal tumors. Clinical records were also reviewed. In carcinoma cases, an immunohistochemical study was performed, using Dako’s Envision methods, , as previously reported , 12. TheAmong the 512 appendiceal specimens, 4 cases of non-invasive adenocarcinoma were identified. Therefore, the incidence of appendiceal adenocarcinoma was 0.8% of all appendectomies. No cases of other tumors including carcinoid tumors were found.A 48-year-old woman was admitted to our hospital because of acute right lower abdominal pain. Clinically, acute appendicitis was diagnosed, and an appendectomy was performed. Pathologically, the appendix was small and fibrotic . PapillaAn 84-year-old man was admitted to our hospital complaining of acute abdominal pain. Clinically, acute appendicitis was diagnosed, and an appendectomy was performed. Pathologically, the appendix showed acute phlegmonous appendicitis. Flat type adenocarcinoma was recognized in the mucosa . The carA 39-year-old man presented with chronic abdominal pain and fever. Imaging modalities including US, CT and MRI revealed cystic dilation of the appendix. Resection of appendix, terminal ileum and cecum was performed  under thAn 86-year-old woman was admitted to our hospital because of acute chronic abdominal pain. Colon endoscopy revealed a tumor at the orifice of the appendectomy, and biopsies from the tumor showed atypical cells suggestive of adenocarcinoma. Therefore, resection of appendix, terminal ileum and cecum was performed under the clinical diagnosis of probable appendiceal adenocarcinoma. Grossly, the appendiceal lumen was filled with papillary epithelial proliferation and much mucus . HistoloThe most common appendiceal tumor is carcinoid tumor, followed by carcinoma , 2. The In the present series, the incidence of adenocarcinoma was 0.8 % of all appendectomies. In the WHO blue book , the incClinically, two cases (case 1 and case 2) of the present series showed clinical features of acute appendicitis. The other two cases (case 3 and case 4) in the present cases showed some clinical features of appendiceal tumors. In particular, imaging modalities identified abnormities of the appendix in these two cases. These findings suggest that clinicians should be aware of appendiceal carcinoma even in patients with typical clinical features of acute appendicitis. Further, imaging techniques including US, CT and MRI are essential for identification of appendiceal tumors. Pathologists also should carefully examine the appendectomies. The prognosis was good in the present series.Pathologically, the appendiceal adenocarcinoma of the present series was papillary (or villous) adenocarcinoma in one case, flat type adenocarcinoma in two cases, and mucinous adenocarcinoma in one case. All the four cases were in situ adenocarcinomas without apparent invasion. These findings may indicate that the present adenocarcinomas were in early stages in the carcinomatous progression. No pseudomyxoma peritonei -10 was nIn the present series, immunoreactive p53 protein was expressed in all the 4 adenocarcinomas. Kabbani et al  suggesteIn summary, the present series suggest that incidence of appendiceal tumor was 0.8% of all appendectomies. All the detected 4 adenocarcinomas were non-invasive adenocarcinomas of the appendix showing variable morphologies.


Malignant mesothelioma of the tunica vaginalis testis (MMTVT) is an extremely rare tumour, usually mimicking benign pathologies of the scrotum.Our case is an 84-year-old male patient who appealed with a painless, left-sided scrotal swelling longer than 2 months. Although the level of tumour markers was normal, ultrasonographic examination results forced us to perform an inguinal scrotal exploration. Multiple small papillary tumours, both on tunica vaginalis and tunica albuginea, were detected intraoperatively. Due to these findings, radical orchiectomy was performed.A pathological evaluation showed malignant mesothelioma (MM) of the tunica vaginalis testis. Exposure to asbestos is a well-known risk factor. Furthermore, a history of trauma, herniorrhaphy and chronic hydroceles is blamed as a possible risk factor. Scrotal ultrasonography is the mainstay of primary diagnosis and, therefore, it should not be overlooked when dealing with benign scrotal cysts or hydroceles, which are very common pathologies at these decades, too.Radical inguinal orchiectomy is the primary treatment choice for localised MMTVT disease, whereas in signs of lymph node metastasis, inguinal lymph node dissection is required. Radical resection should be completed with chemotherapy and/or radiotherapy for an advanced or recurrent disease. This case, which is very rarely reported in the literature and detected during inguinal exploration, along with the pathological works that supported the diagnosis, was presented with this report. Malignant mesothelioma of the tunica vaginalis testis (MMTVT) is an extremely rare tumour, usually mimicking benign pathologies of the scrotum . MesotheWe are presenting an 84-year-old male patient who appealed with a painless, left-sided scrotal swelling longer than 2 months. The right testis was normal and no inguinal palpable lymph node was detected. Also, there was no history of previous surgical intervention or trauma to this region reported by the patient. An ultrasonographic evaluation demonstrated irregular thickening and well vascularized, multiple papillary solid lesions of the tunica vaginalis. Testicular tumour markers, alpha-fetoprotein (AFP) and beta human choriongonadotropin (B HCG) levels were within normal ranges. A computed tomography (CT) of the pelvis, abdomen and thorax showed no sign of metastasis to solid organs to lymph nodes. Although a normal level of tumour markers was detected, ultrasonographic examination results caused an inguinal exploration. The skin was incised 2 cm above the inguinal ligament from the deep ring to the superficial ring and the inguinal canal was detected. After clamping the testicular cordal structures, tunica vaginalis was exposed from the adjacent structures. When it was incised vertically, multiple small papillary tumours, both on the tunica vaginalis and the tunica albuginea, were detected Figure .According to these intraoperative findings, a radical orchiectomy was performed. A pathological gross examination of the specimen showed multiple, papillary, tan/white-coloured nodulations on the parietal and visceral leaves of the tunica vaginalis, with an average size of 0.5-1 cm. Similar papillary masses on the outer surface of the tunica albuginea were also found. Macroscopically, the testis showed the normal structure of the parenchyma and no tumour invasion to the tunica albuginea was determined. A microscopic evaluation showed a solid and nesting pattern, which was composed of atypical epithelioid cells with clear and eosinophilic cytoplasms. The nuclei of the tumour cells were pleomorphic and hyperchromatic, with prominent, big nucleoli Figure .Immunohistochemically, tumour cells were extensively positive with calretinin and creatine, focally positive with epithelial membrane antigen (EMA) and showed a focal nuclear reaction with WT-1. But, it was negative with vimentin, cytokeratin (CK5), carcinoembryonic antigen (CEA) and B72.3 (Figure Less than 300 cases of MMTVT, with poor information of long-term follow-up and multimodal therapy, have been reported . AsbestoMMTVT should not be overlooked when dealing with benign scrotal cysts or hydroceles, which are very common pathologies in the same decades. Also, aggressive surgery and adjuvant therapy are necessary to achieve long-term survival.


Stercoral colitis is an inflammatory process involving the colonic wall related to fecal impaction. This rare condition is associated with high morbidity-mortality.We report a case of a 78-year-old woman with a history of dementia under clozapine who presented a clinical and sonographic presentation of acute appendicitis. The worsening of her clinical condition prompted us to review our diagnosis and modify our approach using the CT scan which was consistent with stercoral colitis. This report concerns an atypical presentation of this condition.The present case highlights the ability of severe forms of fecal impaction to precipitate very rare and life-threatening complications like stercoral colitis. It also points the importance of including stercoral colitis in the differential diagnosis of acute appendicitis in altered patients under anticholenergic drugs and the critical role of the CT scan as a crucial radiologic adjunct. Blood gasses showed metabolic acidosis with lactic acid of 4.9.A 78-year-old woman with a history of dementia, depression, and hypertension presented to the emergency department with a 3-day history of lethargy and confusion. Among the medications she was taking were clozapine for over 10 years and amlodipine. On examination, she was drowsy, tachycardic (117 beats per minute), and hypotensive (82/42 mmHg) with a temperature of 38 °C. The abdomen was distended with right lower quadrant abdominal pain and active bowel sounds. Rectal examination disclosed a small amount of stool. Blood tests showed raised inflammatory markers with a C-reactive protein of 320 mg/L and a white cell count of 18 × 10Ultrasonography abdomen revealed dilated bowel loops with a dilated appendix in a retrocecal position. Broad-spectrum empiric antibiotics were started, and the surgeon was contacted to perform appendectomy.Escherichia coli susceptible to previous antibiotics. With this recent event, the diagnosis of acute appendicitis as the cause of septic shock seemed very unlikely. A CT scan of the abdomen and pelvis was then performed to clarify the diagnosis that showed thickened and edematous rectal wall  is a common gastrointestinal disorder with potential for major morbidity, especially in the elderly population. Risk factors for this condition include cognitive impairment, cerebral palsy, immobility, rectal hyposensitivity, poor water intake, and use of constipating drugs. Constipation can be a serious side effect of clozapine . The mecFI causes increased colonic intraluminal pressure that exceeds the capillary perfusion pressure in the bowel wall  and resuCT scan is the modality of choice for diagnosis and shows a thickened rectum impacted with feces, dilated rectosigmoid colon with pericolic fat stranding . In the The present case highlights the ability of severe forms of FI to precipitate very rare and life-threatening complications like stercoral colitis. It also points the importance of including stercoral colitis in the differential diagnosis of acute appendicitis in altered patients under anticholenergic drugs and the critical role of the CT scan as a crucial radiologic adjunct.


Ganglioneuroblastoma is a tumor of peripheral neuroblastic tissue which occurs predominantly in the pediatric age group; it is a rare occurrence in the newborn period with only one case reported at birth to date.We report the case of a newborn male baby of Brahmin ethnicity from Nepal who presented with respiratory distress and blueberry muffin skin lesions after birth. A computed tomography scan showed a mass lesion in the posterior mediastinum, which was diagnosed as ganglioneuroblastoma on fine-needle aspiration cytology. He also had metastases to multiple sites including heart, lungs, skin and brain.Ganglioneuroblastoma is a rare tumor in newborns. Any newborn presenting with respiratory distress associated with blueberry muffin skin lesions should be evaluated for neuroblastic tumor. With an annual incidence of 7.6 per 1,000,000 population according to the Surveillance, Epidemiology and End Results (SEER) Registry, they are the most common solid extracranial tumors in children [et al. .th day of life. On follow-up, it was found that the baby had died the day after discharge from hospital.A newborn baby of Brahmin ethnicity from Nepal presented within a few hours of life with respiratory distress and bluish skin nodules (blueberry muffin rash) all over his body. The baby was a male, weighing 2200 grams (below the tenth percentile for gestation), born at term to a 22-year-old primipara out of a nonconsanguineous union without any antenatal checkups. The baby was born with good Apgar scores and had multiple bluish skin nodules all over his body. Within 3 hours of birth, the baby developed severe respiratory distress and difficulty in feeding, with a temperature of 100.2° F. A physical examination showed a grunting, pale baby with peripheral cyanosis and tachypnea. There were multiple (23 in number) bluish nodules over his whole body; the largest one, measuring 1 × 1 cm, was over his nose Fig. . Auscultet al. reported a series of 80 cases all of which arose from the posterior mediastinum [Respiratory distress is a very common problem in newborns. It can be due to several causes, notably pneumonia, sepsis, heart disease, congenital anomalies of the respiratory tract, and so on. GNB is a rare tumor in the newborn period. Rarer still is GNB presenting with respiratory distress. Most cases present before 10 years of age. After a literature search, we could find only one case which had presented at birth and another case at 13 days of life . GNB ariiastinum . Rarely,A CT scan is the standard modality of imaging to evaluate neuroblastic tumors . It showDiagnosis is confirmed by histological study, preferably done by biopsy, which shows the typical small, round, blue cells with hyperchromatic nuclei, scanty cytoplasm, and ganglion cells characterized by abundant basophilic cytoplasm containing Nissl material, a large vesicular nucleus, and a prominent nucleolus. We diagnosed our case on the basis of FNAC. However, we could not differentiate the subtype of GNB as the baby was taken home before a biopsy could be performed. Bone marrow aspirate may show evidence of metastatic disease. However, Adam et al, in their study found negative marrow findings in 38 of 40 cases studied . As in oStaging of disease is done as per the International Neuroblastoma Staging Study (INSS) guidelines based on clinical, radiological, and surgical features. Treatment depends on the stage and includes surgical excision, chemotherapy, radiotherapy, and bone marrow transplantation. Prognosis depends on the age of the patient and stage of disease. Age less than 1 year, primaries in the mediastinum, and stage 1–2 and 4S are usually associated with a better prognosis. However, in our case even though the primary site was in the mediastinum, presence of widespread metastases to the vital organs could have led to early mortality.Ganglioneuroblastoma is a very rare cause of respiratory distress in newborns. However, when respiratory distress is seen with blueberry muffin skin rashes, neuroblastic tumors should be ruled out. CT scan with CT-guided FNAC was found to be a good modality of diagnosis in our set-up.


Isolated bilateral cerebral peduncular infarctions (BCPI) presenting as acute pseudobulbar palsy are rarely reported and, to the best of our knowledge, most of the previous reports of BCPI were related to locked-in syndrome and disturbance of consciousness. Herein, we described a case of a 55-year-old man who presented with acute pseudobulbar palsy and mild tetraparesis, but preserved eye movements, with no consciousness disturbance. DWI revealed an acute infarction involving the central portion of the cerebral peduncle with a characteristic “traditional Chinese eight character” sign. The relationship between the infarcted range in the cerebral peduncle and the clinical manifestation was discussed in our report. Bilateral cerebral peduncular infarctions (BCPI) are an extremely rare neurological disorder, occurring in only 0.26% of patients with acute ischemic stroke . The anaA 55-year-old man with known longstanding hypertension and diabetes mellitus type 2 presented with acute onset of dizziness, slurred speech, and unsteady gait. On admission, his blood pressure was 178/100 mmHg and heart rate was 92 beats/min. He was dispirited but oriented. Additionally, he did not take the initiative to speak but could answer simple questions in a whisper with a few words. Neurological examination revealed severe dysphagia, dysarthria, mild tetraplegia , and ataxia of the trunk and all four limbs. Pupillary reflexes and extraocular movements  were intact. Bilateral Babinski signs were positive. Meanwhile, he also showed spasmodic crying and laughing on examination. Magnetic resonance imaging (MRI) of the brain performed 36 hours after the onset of symptoms revealed acute, bilateral, symmetric, cerebral peduncular infarcts (BCPI), which showed increased signal intensity on diffusion-weighted imaging (DWI) with low values on apparent diffusion coefficient (ADC) map Figures , in linePreviously, a case of bilateral cerebral peduncular infarction involving most of the lateral portion of the peduncle with a sign termed “Mickey Mouse ears” on DWI has been reported . InteresThe circulation of the cerebral peduncle is mainly supplied by perforating branches from the collicular artery, posteromedial choroidal artery, and circumflex branches from the p1 or p2 segment of PCA. In addition, it also receives contribution from the posterior communicating artery and anterior choroidal artery . DespiteIn summary, we reported a case of a patient who presented with acute pseudobulbar palsy, preserved eye movements, and only mild tetraparesis resulting from isolated bilateral cerebral peduncular infarction. It was different from previous reports in which BCPI were associated with locked-in syndrome and disturbance of consciousness. In addition, the hypoperfusion caused by vertebrobasilar artery stenosis probably was the key etiology of BCPI in our study, which was in accordance with the literature.


Torsion of a communicating hydrocele is extremely rare, and the cause is unclear. We report the case of a 3-year-old boy referred to us with acute scrotum. Operative findings revealed torsion of a communicating hydrocele with a 360-degree rotation of the distal end. We performed surgical excision of the necrotic cystic mass and high ligation of the peritoneal communication. A high index of suspicion is required for the correct diagnosis and treatment of this condition, which should be included among the causes of acute scrotum in childhood. Acute scrotum in children is usually caused by torsion of testicular appendages, testicular torsion, or epididymitis/orchitis . TorsionA 3-year-old boy presented with pain and swelling of the left hemiscrotum lasting 13 hours. Occasionally, enlargement of the left hemiscrotum without any associated symptoms had been observed. No other symptoms, such as nausea, vomiting, or fever, were reported, nor was there a history of scrotal trauma. The left hemiscrotum was enlarged , withoutEleven cases of hernia sac torsion have been reported in the English literature, and only one was characterized as hydrocele torsion. Among the cases described as hernia sac torsion, two had a history of ipsilateral scrotum enlargement, suggesting communicating hydrocele. As in our case, none of these eleven patients had gastrointestinal symptoms, such as abdominal pain, nausea, or vomiting, which occur in most children with testicular torsion . All patIt is difficult to unmistakably determine the correct etiology of acute scrotum. The diagnosis established on a clinical basis or through exploratory tests is not precise. Ultrasonography, particularly color Doppler imaging, continues to play a central role in the investigation of children with pain and edema of the scrotum . ScintigThe mechanism of torsion of the hernia sac or hydrocele of the scrotum is unknown. Shiraishi et al. suggested that these disorders may develop from a bell-clapper deformity, in which the hydrocele remains loose or there is slight adhesion, allowing the torsion to happen . As seenAlthough a conclusive preoperative diagnosis is not possible, data provided by US, coupled with history and physical findings, may point to this rare cause of acute scrotum, allowing the use of a better strategy, such as an inguinal approach, for the surgical treatment. As a result, the scrotal approach, used in most patients with acute scrotum, may be avoided.


Santa Casa de Misericórdia Hospital in Vitória, Espírito Santo, Brazil. Pathology revealed bilateral testicles with Sertoli cell tumors. According to the international literature, prophylactic gonadectomy following puberty is recommended due to the progressive risk of neoplastic transformation in the residual gonads. Androgen insensitivity syndrome is the most common cause of male pseudohermaphroditism and the third most common cause of primary amenorrhea. This genetic alteration is a consequence of inherited defects on the X chromosome causing total or partial damage to the intrauterine virilization process due to functional abnormalities in the androgen receptors. The present report describes a 22-year-old patient with a female phenotype and a 46, XY karyotype, presenting with bilateral inguinal tumors. The tumors were surgically removed at the Complete androgen insensitivity syndrome, formerly known as testicular feminization, was first described in detail in 1953 in a classic report of 82 cases published by Morris [The androgens synthesized in the gonads during embryonic life are of vital importance in determining the male phenotype in an embryo with the 46, XY karyotype. Structural changes in the hormone receptor as a consequence of possible mutations in the X chromosome prevent it from functioning, leading to the expression of a female phenotype in an individual with a male karyotype. All the sex characteristics develop completely; however, the internal genitalia are absent, since production of the anti-Müllerian hormone is maintained. Anti-Müllerian hormone is produced by the Sertoli cells; therefore, detection of this hormone in serum suggests the presence of testicles. In the case of complete androgen insensitivity syndrome (CAIS), serum levels are high, while, in other intersex states, levels may be below normal or undetectable. Therefore, measuring anti-Müllerian hormone levels is of key importance in the diagnosis of male pseudohermaphroditism , 4.The clinical signs that characterize the disease consist of female behavior, complete lack or scarcity of axillary and pubic hair, adequate female breast development, female external genitalia, blind vaginal pouch, absence of internal genitalia, and the presence of gonads with seminiferous tubules; however, spermatogenesis is absent , 5.Individuals with androgen insensitivity syndrome should be submitted to prophylactic gonadectomy due to the risk of developing testicular malignancy. Studies in adults have shown that 50% of testicles containing a carcinoma in situ develop an invasive tumor within five years of diagnosis when left untreated. The question under debate is the ideal moment for surgery , 7.Historically, surgery was performed as soon as possible to avoid uncomfortable psychosexual questions during adolescence or at the beginning of adult life. More recently, surgery at the end of the teenage years or in the early twenties has been preferred; nevertheless, when opting for this management, it should be remembered that the prevalence of carcinoma in situ is between 2 and 5% during puberty , 8. Santa Casa de Misericórdia, Vitória, Espírito Santo, Brazil.The present report describes the histopathology findings of bilateral Sertoli cell tumors in a patient with androgen insensitivity syndrome receiving care at the Department of Gynecology and Obstetrics, Santa Casa de Misericórdia Hospital approved the procedures involved in this report on April 27, 2016, under reference number CAAE 51407815.2.0000.5065. Written informed consent for the publication of this report and corresponding images was obtained from the patient.The internal review board of the Santa Casa de Misericórdia Hospital on April 25, 2014 reporting primary amenorrhea. She had begun her sexual life at 19 years of age and had never conceived. She reported having been monitored by a multidisciplinary medical team since she was 17 years old.An unmarried, white 22-year-old patient with a female phenotype, living in a small town in the state of Espírito Santo, presented at the gynecology outpatient clinic of theThe patient had a history of hypothyroidism and was undergoing treatment with 100 mcg/day of levothyroxine. There was a positive family history of malformation of the genital tract (two aunts with uterine agenesis). The patient was 1.70 meters in height and weighed 52 kg. Physical examination showed her to be in good general health, with good coloring, well-hydrated, acyanotic, and anicteric and with no swelling. She had poorly developed secondary sex characteristics, small breasts and sparse body hair (vulva and axillae). Her cardiovascular and respiratory systems were normal. Abdominal palpation revealed two inguinal masses, both approximately 4.0 cm in size, mobile and painless.Gynecological examination showed a trophic vulva, sparse pubic hair, and no lesions or dyschromia. Pelvic examination revealed a vaginal canal about 7 cm in length with normal elasticity and rugae, no apparent lesions, and blind vaginal pouch. Bimanual palpation revealed absence of the cervix.μIU/mL, cortisol 24.27 μg/dL, 17-hydroxyprogesterone 198 ng/dL, estradiol 33 pg/mL, and 8:00 a.m. cortisol 23.99 μg/dL.The patient had brought the results of imaging exams. Magnetic resonance imaging (MRI) showed normal bone morphology with normal signal intensity patterns; no uterus and no ovaries; bladder of normal size with regular walls; and partial vaginal canal with the upper segment missing. There was no free fluid in the abdominal cavity. Nodular formations with regular contours and well-defined borders were located at the level of the inguinal canal, bilaterally and symmetrically, measuring around 3.4 × 2.4 × 1.4 on the right side and 3.6 × 2.5 × 1.4 on the left side, with a slight bulging of the corresponding subcutaneous tissue. Clinically, this finding could correspond to ectopic gonads (cryptorchidism). Ultrasound of the urinary tract was normal. Pelvic ultrasonography showed absence of a uterus at the usual site (agenesis). The ovaries were not visualized, and there was no sign of tumors in the ovarian region or in the pelvis. No free fluid was detected in the pelvis. Previous laboratory tests showed FSH 12.0 mIU/mL, LH 52.5 mIU/mL, TSH 1.092 G-banded karyotype was performed on a peripheral blood sample, with results showing the 46, XY genotype.The patient was referred to the hospital's Department of Gynecology for bilateral gonadectomy . The proThe patient is being monitored clinically at the gynecological endocrinology clinic and is currently under hormone replacement therapy with 2 mg of estradiol valerate .Androgen insensitivity syndrome is a metabolic disease characterized by complete resistance to the effects of androgens. According to the literature, its prevalence ranges from 1 in 20,400 to 1 in 99,100 male infants and the disorder is associated with X-linked recessive inheritance .Although diagnosis is usually reached after puberty, it is known that 1-2% of the children who present with an inguinal hernia or a palpable lump in this region may have alterations associated with the syndrome . In adolPelvic examination and evaluation of the gonads can be made by supplementary tests such as ultrasonography and MRI, which, according to the review published by Khan and Craig, are the most accurate tests in such cases . UltrasoIn the case presented here, both ultrasonography and MRI revealed the fact that the upper genital tract was absent and that the gonads were missing at the usual site. Compared to ultrasonography, MRI provided a more precise definition of the site and size of the ectopic testicles. Neither of the two methods identified any malformation of the urinary tract.The incidence of testicular cancer is higher in developed countries, with the disease affecting 1.5/100,000 inhabitants worldwide. Female patients diagnosed with Morris syndrome may develop gonadal tumors. According to the literature, the risk of these patients developing malignant tumors is distinctly higher than that of the general population –11. ManuThe malignant tumors often associated with this syndrome are seminomas and gonadoblastomas, although other histological forms such as teratomas, choriocarcinomas, yolk sac tumors, and embryonal tumors may also be present. Likewise, benign tumors such as adenomas, Leydig cell, and/or Sertoli cell tumors are other possible findings –16.In the case described here, prophylactic gonadectomy was performed after puberty and the histopathology finding of Sertoli cell tumors in both gonads was unusual. The prevalence of this type of benign tumor is low in the population, representing 0.4 to 1.5% of testicular tumors in adults and 4% in children , 17.Treatment is not restricted to just removing the gonads but demands multidisciplinary management in an attempt to minimize the psychological impact, since there is discordance between the individual's 46, XY genotype and female phenotype , 19. Gui Santa Casa de Misericórdia Hospital in Vitória.Following surgery, estrogen replacement therapy is recommended . Some st


To the best of our knowledge, this is the first documented case of pancreatic adenocarcinoma to metastasize both to the placenta and multiple maternal sites  in a pregnant patient. Unpredictable metastases to the placenta may be encountered and may even lead to definitive diagnosis, as in our case. Therefore, the placenta in any patient with known malignancy should be sent for pathologic evaluation.The occurrence of coexisting cancer in pregnant women is not a common phenomenon. It complicates approximately 1 in 1000 to 1500 pregnancies. We present a multiparous woman aged 27 years in her 28 It is estimated that about 3.500 new cases of cancer are diagnosed annually in pregnant women in the United States of America, which corresponds to 1 case for every 1.000 gestations. The most frequently diagnosed malignancies in pregnancy are breast cancer, cervical cancer, malignant melanoma, and lymphomas, respectively. The most common tumor that metastasizes to the fetus or placenta is malignant melanoma, which accounts for 30% of all pregnancy-associated tumors. The second most frequently metastasizing malignancies are leukemia and lymphoma, followed by carcinoma of the breast and lung. Herein, we present a multiparous woman aged 27 years in her 28th week of pregnancy who was admitted to our clinic with right upper quadrant pain and was finally revealed to have multiple metastatic pancreatic adenocarcinoma.The occurrence of coexisting cancer in pregnant women is not a common phenomenon. It complicates approximately 1 in 1.000 to 1.500 pregnanciesth week of gestation presented with right upper quadrant pain, nausea, and vomiting. The patient was admitted to the hospital with a preliminary diagnosis of acute cholecystitis. Murphy’s sign was positive. There were no signs of acute peritoneal irritation and she was initially managed with a nil-by-mouth status, intravenous hydration, and antispasmodic medication for abdominal cramps. She had a serum indirect bilirubin titre of 0.7 mg/dL (direct 0.2 mg/dL), and alkaline phosphatase titre of 123 u/L. Serology against viral hepatitis antigens were all negative. Levels of cancer antigen (Ca) 19-9, Ca 125, and alpha-fetoprotein tumor markers were all elevated to >2.064 U/mL, 985 U/mL, and 45 ng/mL respectively. Obstetric ultrasonography revealed an intrauterine male fetus with normal biometric measurements and biophysical profile scores. Right upper-quadrant abdominal ultrasound revealed a normal gallbladder, no dilatation of the common bile duct, and no periportal mesenteric pathologic lymphadenopathy. However, cervical ultrasound revealed bilateral cervical and left supraclavicular conglomerate lymphadenopathy. Supracervical lymph node excisional biopsy was performed. The pathologic diagnosis was reported to be anaplastic carcinoma, possibly originating from the gastrointestinal tract, gallbladder or pancreas. Due to relatively advanced gestational age of the fetus and poor prognosis of the mother if left untreated, corticosteroids were administered to enhance fetal lung maturity and cesarean section was performed at 28 weeks to deliver a 1.000-g male fetus with Apgar scores of 6-8, 1-5 minutes. The neonate was transferred to the neonatal intensive care unit but died of sepsis 30 days post-partum. The family refused autopsy of the neonate and mother; therefore, no pathologic condition could be observed in the neonate.A multiparous patient aged 27 years in her 26Intraperitoneal massive ascites was observed perioperatively, a sample of which was sent to pathologic examination together with the placenta. Pathology revealed malignancy-positive ascites cytology and metastasis of pancreatic adenocarcinoma to the placenta . Postope. Risk factors for pancreatic cancer include cigarette smoking, advanced age, male sex, African-American ethnicity, family history, genetic background/tendency, diabetes mellitus, and chronic pancreatitis. The most common presenting symptoms in patients with pancreatic cancer are abdominal and epigastric pain, dark urine, jaundice, nausea, back pain, diarrhoea, and vomiting. Ultrasound and magnetic resonance imaging are the mainstays of imaging during pregnancy. Other options may be used if necessary. Fetal outcomes are generally very good, with iatrogenic prematurity being the most common complication. Surgical resection followed by adjuvant therapy is the standard of care for early-stage disease. Surgical intervention in the first trimester carries a risk of spontaneous abortion, and the size of the uterus is prohibitive in the third trimester; therefore, the second trimester is the ideal time for tumor resection. Only 24 cases of pancreatic adenocarcinoma diagnosed antepartum have been described in the literature. The gestational age of the patients range from 4 to 36 weeks. Khatsiev et al. reported a case of pancreatic adenocarcinoma in a pregnant woman aged 29 years with situs inversus in the first trimester. Patient underwent pancreatoduodenectomy following abortion. Onuma et al. described a woman at 34 weeks gestation who presented with uterine contractions and a large retroperitoneal mass. Pancreatic cancer was detected after emergency cesarean delivery, and pancreatoduodenectomy was performed. Kakoza et al. described a woman who was diagnosed as having cholecystitis and gallstone pancreatitis at 24 weeks gestation. Our patient presented as having acute cholecystitis.Cancer-complicated pregnancy is a rare coexistence and presents in approximately 1 in 1.000 to 1.500 pregnancies. Among those, pancreatic cancer is even rarer in a pregnant patient. The most common tumor that metastasizes to the fetus or placenta is malignant melanoma, which accounts for 30% of all pregnancy-associated tumors. The second most frequently metastasizing malignancies are leukemia and lymphoma, followed by carcinoma of the breast and lung. The liver and peritoneal cavities are the most common sites of metastases in pancreatic cancer. Marci et al reported a case of pancreatic carcinoma in a women at 35 weeks gestation with multiple liver metastasis. Metastases to the placenta and supraclavicular lymph nodes are extremely unusual. A review of the literature revealed only six cases of supraclavicular metastases of pancreatic adenocarcinoma. Al-Adnani et al. reported a case of maternal pancreatic carcinoma metastatic to the placenta. In our case, placental and multiple maternal metastases were documented. Metastatic disease to the fetus is incredibly rare and has been documented only in melanoma, leukemia, and lymphoma.Vertical transmission of cancer is exceptionally rare, although maternal cells do reach the fetus. From 1866 to 1999, 58 cases of documented maternal malignancy metastatic to the placenta and fetus were reported in the English literatureTo the best of our knowledge, this is the first documented case of a pancreatic adenocarcinoma to metastasize both to the placenta and multiple maternal sites  in a pregnant patient. Unpredictable metastases to the placenta may be encountered and may even lead to definitive diagnosis, as in our case. Therefore, the placenta in any patient with known malignancy should be sent for pathologic evaluation. Moreover, optimal care of a pregnant woman with metastatic cancer requires a prompt multidisciplinary approach, with special focus on maternal wellbeing and survival, while trying to minimize the negative impact on the fetus secondarily.


The simultaneous occurrence of Klinefelter Syndrome (KS) and Congenital Adrenal Hyperplasia (CAH) is an exceptional event: there are just three case reports (two children and a 51 years old man) describing males affected by both KS and 21OHD (21-hydroxylase deficiency) CAH, the first causing androgen deficiency, the latter leading to androgen excess.We report the 4th case of association of KS and CAH in a young man with CAH with good androgen control and with normal secondary sex characteristics, whose Klinefelter syndrome was diagnosed because of reduced testicular volume. He was the first reported case of association of KS and CAH who started androgen replacement therapy in the pubertal age and whose pubertal development was described and followed up step by step.In a boy with CAH and small testicular volume, it’s important to consider that hypogonadism may be masked by the adrenal androgens excess and a karyotype should be performed once testicular adrenal rests have been ruled out. In males with congenital adrenal hyperplasia (CAH), the fertility rate is reduced compared with the healthy population and the most frequent cause is testicular adrenal rest tumor. However high levels of adrenal androgens may hide hypogonadism.We report a case of a boy with CAH caused by a 21-hydroxylase deficiency (21OHD) and small testicular volumes in which a chromosomal analysis revealed the association of Klinefelter syndrome .A CAH was suspected when he was two-years-old because of an increased growth rate with advanced bone age, precocious pubarche, and acne. The diagnosis of CAH was then confirmed by a pathologic ACTH stimulation test and by the genetic analysis, revealing a steroid 21OHD (classic form) due to a compound heterozygosity: he inherited the paternal chromosome carrying the point mutation A/CG intron 2 (655) and the maternal chromosome with the point mutation Ile-Asn-17 (999).He started replacement treatment with oral hydrocortisone and fludrocortisone acetate with good compliance. Biochemical and somatic parameters were monitored every 3 months, adjusting the therapy to gain adequate androgen control. Some follow-up controls revealed adrenal androgens excess.Child’s development proceeded regularly, but he didn’t start gonadal puberty. When he was 16 years-old, his testicular volume was still of 3–4 ml, assessed both using orchidometer and scrotal ultrasound. Testes ultrasound showed no signs of adrenal rest tumor.He had wholly developed pubic hair and a pubertal penis length. He also had a family history of delayed puberty and his bone age was delayed by 3 years. Blood examination revealed low basal testosterone (1.82 ng/mL), normal basal FSH (10.1 mUI/mL) and basal LH (7 mUI/mL).LH peak after GnRH stimulation test was 34.9 mUI/ml and FSH peak 16.2 mUI/ml.A short hCG stimulation test was performed to assess the testes ability to produce testosterone: it showed a good gonadal response, with an increase in serum testosterone after hCG stimulation from a basal value of 2.45 ng/mL to 5.53 ng/ml.Serum antimüllerian hormone 17.3 ng/mL) and serum inhibin B (44 ng/dL) were normal  sho sho10] sOur patient KS phenotype could be attenuated by adrenal androgen excess despite apparent good control at the 3 months follow up blood tests.This is the 4th case report of the simultaneous occurrence of KS and CAH.In a boy with CAH and small testicular volume, it’s important to consider that hypogonadism may be masked by the adrenal androgens excess and a karyotype should be performed once testicular adrenal rests have been ruled out.When considering the frequency of both 21-OH deficiency and Klinefelter syndrome, the association between these two diseases should be expected to be higher than documented (only 3 cases reported since now).The authors affirm that the manuscript submitted is original that all statements asserted as facts are based on authors careful investigation and research for accuracy, that the manuscript does not, in whole or part, infringe any copyright, that it has not been published in total or in part and is not being submitted or considered for publication in total or in part elsewhere.


Laryngocoeles are rare cystic dilatations of the laryngeal ventricle. Obstruction of its outlet can cause entrapment of mucus and superimposed infection causes a laryngopyocoele. Such presentations, although rare, have potential to cause airway obstruction. A 67-year-old lady presented with a one-week history of hoarseness and shortness of breath. On examination, she was stridulous and had fullness of the left side of the neck. Nasendoscopy revealed large bilateral vocal cord polyps and near-complete glottis obstruction. She was taken to emergency theatre for restoration of a viable airway. Upon excision of the polyps, pus was visualised originating from the laryngeal ventricle. Literature proposes that laryngocoeles develop secondary to a one-way valve caused by an obstructing lesion distorting the saccule neck. We propose that the laryngocoele developed secondary to large obstructing polyps. Urgent excision of the polyps allowed decompression of the laryngopyocoele and reestablishment of a patent airway. Laryngocoeles are a rare dilatation of the laryngeal ventricle which can present in a variety of ways ranging from benign neck lumps to acute airway obstruction. The aetiology of laryngocoeles remains unclear, but a number of associations have been described. We present a case of a 67-year-old lady who presented with near-complete glottis obstruction. Initial examination revealed severe vocal cord polyps and she underwent emergency debridement. Debridement of the polyps resulted in the discovery of a pus-filled laryngocoele. This case represents the first description in the literature of coexisting vocal cord polyps and laryngocoeles. By considering previously described theories on the development of laryngocoeles, we speculate that the severity of the polyps in this case resulted in insufficient drainage of the laryngeal ventricle and the development of the laryngocoele.A 67-year-old lady presented to the Ear, Nose and Throat emergency clinic with a one-week history of hoarseness and intermittent shortness of breath, worse at night. She had a 40-pack-year history and was fearful of seeking medical attention. On examination, she was afebrile, stridulous at rest, tachypnoeic, tachycardic, and hypertensive. On palpation, there was fullness to the left side of her neck. Flexible laryngoscopy revealed large bilateral vocal cord polyps causing almost complete airway obstruction. The findings of such extensive polyps on laryngoscopy implied a potentially longer clinical course than originally reported. The patient underwent an emergency microlaryngoscopy under general anaesthetic for debridement of the vocal cord polyps in order to establish a definitive airway. On excising the left vocal cord polyp, pus appeared to be discharging from the left laryngeal ventricle . Drainag Haemophilus influenzae and Beta-haemolytic Streptococcus and histology was consistent with vocal cord polyps with no indication of malignancy.Due to extensive airway oedema and the risk of recollection and aspiration, the decision was made for the patient to remain intubated on ITU postoperatively. Postoperative CT scan of the patient's neck demonstrated no recollection and the patient was extubated 2 days later and was discharged after 1 week. Intraoperative pus swabs isolatedAt follow-up appointments at 8 weeks and 3 months, the patient was well with no further airway compromise. Flexible laryngoscopy revealed healthy vocal cords with no evidence of recollection within the ventricle. Haemophilus influenzae and Beta-haemolytic Streptococcus.Laryngocoeles are herniations of the laryngeal ventricle . They ar2 laser has been described in the literature as an option for treatment of laryngocoeles to prevent recurrence. An alternative treatment was described by Fraser et al. This paper presented a similar case as described here and discussed endoscopic marsupialisation of the laryngopyocoele using a microdebrider [In this case, the laryngopyocoele was drained intraoperatively with external compression. It was irrigated with normal saline. There was no recurrence at follow-up using this technique. Endoscopic marsupialisation using a COdebrider . This isThe aetiology of laryngocoeles remains unknown, but some associations have been reported within the literature. One of the most well described associations includes raised intraglottic pressure, which in itself can have a number of different aetiologies. For example, wind or brass musicians have a higher incidence of laryngocoeles compared to the rest of the population , 5. TherThere have been many studies making a connection between laryngeal carcinoma and laryngocoeles . The coeTo our knowledge, this case represents the first report of a laryngocoele coexisting with vocal cord polyps. We hypothesise that increased intralaryngeal pressure was caused by the vocal cord polyps obstructing the neck of the laryngeal ventricle leading to laryngocoele formation, in a very similar fashion to laryngeal carcinoma. The vocal cord polyps in this case were very extensive due to the patient being fearful of seeking medical attention. Therefore the unusually long length of the clinical course of this common presentation is likely to have been a contributing factor towards the development of such an uncommon condition: a laryngopyocoele.The development of a laryngocoele or laryngopyocoele is extremely rare but may develop in coexistence with more common pathology, such as vocal cord polyps. We present this case to highlight severe vocal cord polyposis as a potential association with laryngocoele formation.


Spontaneous colonic perforations are scarce, and cecal perforations even more so. Preoperative diagnosis of the latter in a pregnant woman is particularly difficult because of physiologic changes and restrictions on some diagnostic imaging techniques, such as X-rays. Furthermore, management of these patients is a big challenge.We present a case of a spontaneous cecal perforation in a 40-year-old pregnant black woman in the Regional Hospital of Bafoussam in Cameroon. The results of clinical examination and ultrasonography on admission were in line with acute generalized peritonitis in a woman at 20 weeks of a viable pregnancy, indicating an urgent laparotomy. Operative findings were a 1 × 1-cm perforation on a distended cecum with minimal fecal contamination. The treatment consisted of excision of the edges, primary suture of the perforation, and omentoplasty. The recovery of the patient was uneventful.The management of spontaneous cecal perforation in a pregnant woman was a big challenge. The perforation was repaired by primary suture and omentoplasty. Further studies comparing this approach with right hemicolectomy are recommended. Spontaneous perforation of the colon is defined as a sudden perforation of an apparently healthy colon in the absence of any other disease or injury. Fewer than 100 cases have been reported in the literature to date . The latOur patient was a 40-year-old multiparous pregnant black woman at a gestational age of 20 weeks who was not compliant with antenatal care. She had no history of weight loss, chronic constipation, or blood per rectum, nor did she or her relatives report a previous laparotomy. She was brought to our institution’s emergency department by her family 6 days after the onset of a progressively painful abdominal distention, bilious vomiting with fever, and asthenia. She had not passed stools for 3 days prior to her admission. She had no urinary symptoms, and she reported feeling active fetal movements.On physical examination, the patient was conscious and had the following parameters: blood pressure 109/55 mmHg, pulse 125 beats/minute, respiratory rate 25 breaths/minute, and body temperature 37.7 °C. She presented with abdominal distention with no scars or skin lesions. She had a generalized guarding with rebound tenderness. The evaluation by the obstetrician revealed a uterine height of 22 cm. The patient’s umbilical and inguinal orifices were not patent. Her bowel sounds were markedly diminished. On digital rectal examination, her pouch of Douglas was extremely tender, and the gloves were soiled with normal feces. Elsewhere, the patient’s physical examination was unremarkable.3 with neutrophilia and a hemoglobin level of 12 g/dl. The patient’s electrolyte profile was normal, with a K+ level of 3.9 mEq/L. Her coagulation profile was normal, and the result of her human immunodeficiency virus (HIV) serology was negative. She was classified as American Society of Anesthesiologists Physical Status classification II and Altemeier wound classification 4 after preanesthetic consultation.The diagnosis of acute generalized peritonitis in a 20-week gestational age pregnancy was retained with an indication of urgent laparotomy. Abdominal ultrasonography revealed a viable, 21-week gestational age intrauterine monofetal pregnancy with no etiologic finding that could explain the occurrence of peritonitis. Neither plain abdominal x-ray nor abdominal CT was performed. The pretherapy workup revealed hyperleukocytosis at 18,000/mmThe treatment was aimed at suppressing the cause of the peritonitis, decreasing the peritoneal bacterial contamination to its minimum, and addressing the complications of the disease if present. Preoperative resuscitation was initiated with the use of crystalloids. A nasogastric tube and urinary catheter were inserted. We performed an exploratory laparotomy. Perioperative findings included a more or less macroscopically normal peritoneal cavity and a 1 × 1-cm perforation on the anterolateral aspect of a slightly inflamed and distended cecum  and the rectosigmoid region (Sudeck point) . The cecic types . The steic types , 6. The ic types , 6. A vaic types . Spontanic types .Preoperative diagnosis of cecal perforation is infrequent, and it is more often an operative finding, as observed in our patient. The assessment of acute surgical conditions is often made more difficult during pregnancy, owing to the deviation of organs caused by an enlarged uterus and relaxation of the abdominal wall . The laxNeither plain abdominal x-ray nor abdominal CT were performed for our patient, owing to restrictions on these imaging techniques during pregnancy. Abdominal X-rays are controversial regarding their effect on the fetus . During A big question to answer in such a case is the choice of surgical technique for repairing the cecal perforation. In our patient, we found a small hole 1 × 1 cm) and minimal fecal contamination of the peritoneal cavity. After the edges were excised, the perforation was repaired by using a primary suture and omental patch. The surgical approach in treating a cecal perforation varies, depending on the primary etiology: primary suture, cecostomy, or right hemicolectomy . Only a  × 1 cm aThis case report describes a rare spontaneous cecal perforation in a 40-year-old pregnant woman who, unlike other cases, displayed none of the probable etiologic factors reported in the literature. The well-being of mother and fetus were at stake, thus prompting appropriate decision-making and subsequent treatment. The perforation was repaired by excision of the edges, primary suture, and omentoplasty. Few recent studies comparing this approach with right hemicolectomy have been done. A larger prospective study is therefore recommended.


The vast majority of patients presenting with pneumoperitoneum have visceral organ perforation and require urgent diagnostic laparoscopy. Nonsurgical causes are relatively rare and may be attributed to multiple etiologies. Here we describe the case of a 38-year-old Caucasian female who presented to the emergency department with three days of cramping, epigastric abdominal pain. Her physical exam was notable for tenderness to palpation in the epigastric area and abdominal and chest X-rays showed free air under the diaphragm. Free air around the porta hepatis was verified on CT scan. Approximately 90% of pneumoperitoneum cases are due to perforation of visceral organs and therefore require operative management. An urgent exploratory laparoscopy revealed no clear source of free air, but postoperatively the patient developed a large volume of watery discharge from her vagina. Subsequent workup revealed a 1 cm vaginal cuff dehiscence which was later repaired with no postoperative complications. Although the majority of patients with pneumoperitoneum require urgent exploratory laparoscopy, a careful diagnostic workup may reveal sources of free air that are not related to hollow viscous perforation. Vaginal cuff dehiscence represents a rare yet nonurgent source of pneumoperitoneum. This differential should be considered in light of the possible intra- and postoperative complications of surgery. Approximately 90% of pneumoperitoneum cases are due to perforation of visceral organs and therefore require operative management . The rem da Vinci® surgical robot system. This procedure was performed for recurrent cervical intraepithelial neoplasia (CIN) III, a premalignant transformation that typically occurs near the cervical os, which had persisted despite loop electrosurgical excision procedures (LEEP). Prior to presentation, her recovery from this procedure was uncomplicated, with all follow-up appointments followed and only her annual physical exam being scheduled. Her surgical history was also significant for a remote cesarean delivery for oligohydramnios using a Pfannenstiel incision.A 38-year-old G3P3023 woman presented to the emergency department with three days of cramping epigastric abdominal pain radiating to the periumbilical area. The pain was constant, not associated with food, and accompanied by nausea but without vomiting. She had no fevers or chills and denied gastrointestinal or genitourinary symptoms, including vaginal bleeding or discharge. Her last time of sexual intercourse was 8 days prior to presentation. Her surgical history was significant for an uncomplicated single site robotic-assisted hysterectomy with bilateral salpingectomy and cystoscopy performed 60 days prior, using theOn exam her abdomen was soft and nondistended without rebound or guarding, but tender to palpation in the epigastric area. She was afebrile and her vital signs were largely normal with pulse 77, respirations 16, blood pressure 119/68, and oxygen saturation of 97% on room air. A complete blood count showed a white blood cell (WBC) count of 11.8 with 78.4% neutrophils and urinalysis showed cloudy urine with 3+ leukocyte esterase and 11–50 WBCs. X-rays of the chest and abdomen were obtained which showed free air under the diaphragm . A folloBased upon these findings the patient was taken urgently for an exploratory laparoscopy for possible perforated viscus. Intraoperative findings were grossly normal with no intraperitoneal free fluid, edema, inflammatory changes and minimal adhesions which were easily taken down with blunt dissection. The stomach, gallbladder, liver, and pelvis were all visually inspected and the large intestine and small bowel were run. These were found to be normal. An esophagogastroduodenoscopy (EGD) was performed, which demonstrated no ulcerations, blood, or other abnormalities. Warm saline was placed into the intraperitoneal cavity and no gas bubbles were seen following insufflation of the stomach and duodenum in the peritoneal cavity.The patient tolerated the procedure well and was admitted to the surgical floor for pain control and observation, as well as continued intravenous antibiotics. However, on postoperative day one, the patient stood up and reported a large amount of blood-tinged, watery discharge from her vagina. The Obstetrics and Gynecology Service was consulted as there was a suspicion for vaginal cuff dehiscence. The patient was then taken to the operating room for exam under anesthesia and a 1 cm vaginal cuff dehiscence was found, which was repaired with 0 Monocryl suture. Her postoperative course was uncomplicated, and she completed a 48-hour antibiotic course with eventual resolution of abdominal pain. She was discharged following routine postoperative monitoring.The case illustrates the importance of investigating gynecological causes as a source of pneumoperitoneum. Numerous case reports have demonstrated pneumoperitoneum following hysterectomy and sexual intercourse. However, a review of the literature reveals that cases of pneumoperitoneum following both genital and oral-genital intercourse are typically associated with onset of symptoms 2–4 hours after sexual activity –5. SimilHere we present a case of pneumoperitoneum following vaginal cuff dehiscence 60 days after robotic-assisted hysterectomy-bilateral salpingectomy, with the most recent episode of sexual intercourse being 8 days prior. Therefore, while both of these are gynecological risk factors for dehiscence, neither is temporally related to this patient's symptoms. This case illustrates the importance of considering these causes of pneumoperitoneum in patients with a relevant gynecological history despite a timeframe that, based on published literature, is not typically associated with this clinical presentation. Such careful investigation could prevent the need for exploratory surgery should a gynecological workup reveal another possible source of air infiltration into the abdomen.This is relevant because surgical intervention for both diagnostic and therapeutic purposes carries a number of rare yet well-established risks. Possible iatrogenic sources of morbidity and mortality include intraoperative complications from surgical manipulations, adverse reactions to anesthesia agents, medication errors, and postoperative infections. Although these risks are low, they illustrate that even a procedure as straightforward as a diagnostic laparoscopy is not entirely benign.Furthermore, abdominal surgery is associated with long-term consequences of intra-abdominal adhesions which can lead to complications such as small-bowel obstruction, pain, and female infertility . Complicfirst, do no harm.”Although this case describes a patient who presented with a potentially acute condition, it highlights the need to balance both short- and long-term risks of operative management against the possible immediate morbidity and mortality of conservatively treated pneumoperitoneum. Although such clinical decision-making is both challenging and often based upon incomplete information, it lies at the core of physician's responsibility to “Free air in the abdomen following months-prior gynecological surgery and/or sexual intercourse represents a relatively rare yet nonurgent clinical picture when compared with the majority of patients who present with this condition. Most cases of pneumoperitoneum are due to hollow viscous perforation, and therefore require operative management. Although urgent exploratory laparoscopy is the standard of care for most patients with pneumoperitoneum, these alternate causes should be taken into consideration when weighing whether a patient would benefit from surgical intervention.


Two cases of rectal xanthomas are described. One case is that of a 56-year-old Japanese man in whom multiple yellowish spots measuring approximately 3 to 5 mm were observed in the rectum during colonoscopy. The other case is that of a 78-year-old Japanese man in whom colonoscopy showed a whitish plaque of 4 mm in diameter in the rectum. Biopsy examinations performed on both patients revealed the deposition of xanthoma cells within the rectal mucosa. Within the gastrointestinal tract, xanthomas most frequently arise in the stomach, whereas the colorectum is rarely affected. Despite this infrequency, the two cases indicate that xanthomas should be recalled when yellowish to whitish lesions are observed in the colorectum. Xanthomas in the alimentary tract are benign mucosal lesions resulting from the aggregation of foamy histiocytes within the gastrointestinal mucosa . Within We recently encountered two patients with rectal xanthomas that were observed as yellowish to whitish lesions during colonoscopy. In this report, we focus mainly on the macroscopic characteristics of the colorectal xanthoma and review previously reported cases of this disease.A 56-year-old Japanese man was referred to our hospital for investigation of tarry stool. The patient had been consuming lansoprazole, irsogladine, metoprolol, flutoprazepam, and ethyl loflazepate for gastritis, hypertension, and anxiety disorder but had no history of dyslipidemia or diabetes mellitus. A physical examination revealed no abnormalities in his abdomen or xanthomas on his skin, and laboratory findings showed no abnormalities. The levels of cholesterol, triglyceride, and plasma glucose were within normal range. Esophagogastroduodenoscopy showed erosive and atrophic gastritis.During colonoscopy, multiple yellowish spots measuring approximately 3 to 5 mm were observed in the rectum, in addition to hemorrhoids . MagnifyA 78-year-old Japanese man had been treated for remitting seronegative symmetrical synovitis with pitting edema. The patient underwent colonoscopy for screening purposes. He had been taking 2 mg/day of prednisone but had no history of dyslipidemia or diabetes mellitus. A physical examination revealed no xanthomas on his eyelid or extremities and a blood test revealed that his levels of cholesterol, triglyceride, and plasma glucose were within normal range.Colonoscopy showed a whitish plaque of 4 mm in diameter in the rectum . Biopsy In the two cases presented, rectal xanthomas were observed as yellowish to whitish lesions during colonoscopy. As described above, typical gastric xanthomas are well-demarcated whitish plaques or nodules. Similarly, cases with xanthomas in the sigmoid colon and rectum, showing multiple, well-defined, and whitish-yellow lesions, have been previously reported , 5. WeinDiseases other than xanthomas that present with yellowish to whitish lesions in the colon include pseudomembranous colitis, lipomas, and lymphomas. Pseudomembranous colitis is a common cause of antibiotic-associated diarrhea. This disease is characterized by elevated yellow-white plaques that coalesce to form pseudomembranes along the colorectal mucosa and can be easily diagnosed based on their endoscopic appearance . ColonicMicroscopically, foamy macrophages are generally confined to the lamina propria mucosae of the colorectum; the muscularis mucosae or submucosa is rarely affected. Nakasono et al. reported that hyperplastic change was identified in the surface epithelium in 22/28 lesions . In addiAlthough cutaneous and tendinous xanthomas occur in relation to hyperlipidemia, gastrointestinal xanthomas are not associated with dyslipidemia , 7, 13. The etiology of colorectal xanthomas remains unknown. Xanthomas in the stomach are assumed to arise as an inflammatory response to focal mucosal damage and chronic injury such as chronic gastritis , 14–16. In conclusion, we encountered two patients with rectal xanthomas. Both cases showed yellow to whitish lesions in the rectum. Although colorectal xanthoma can present varied morphology, endoscopists should consider this entity when yellowish to whitish lesions are observed in the colorectum.


Vascular lesions of the solid abdominal viscera may pose diagnostic and management issues. A 16-year old girl admitted to emergency department due to recurrent abdominal pain and diagnosed to have multiple vascular malformations of the spleen on imaging investigations. Littoral cell angioma was preoperative suspicion owing to no response of the vascular lesion to the propranolol. It turned out to be cavernous hemangioma on histopathology. Primary tumors of the spleen are classified as lymphoid tumors, non-hematolymphoid tumors and tumor like lesions. Vascular tumors of spleen are rare lesions and include hemangioma, hamartoma, lymphangioma, Littoral cell angioma, and angiosarcoma. Littoral cell angioma was first described by Falk et al in 1991. It arises from the lining cells of the red-pulp sinuses.[1] Splenectomy has to be done for final diagnosis. Hemangioma is the most common benign neoplasm of the spleen. These are small in size and incidentally diagnosed. Preoperative diagnosis is difficult if these are multiple in number with cavernous characteristic.[1-4] Herein, we present a case operated due to suspicion for Littoral cell angioma but diagnosed as multiple cavernous hemangioma on histopathology.A 16-year old girl was admitted to emergency department due to recurrent epigastric pain for the last six months. Her general physical examination was normal. Abdominal examination was also unremarkable. Laboratory investigations were within normal limits except for mild hyperbilirubinemia. Abdominal ultrasonography revealed multiple vascular malformations of the spleen largest being 15mm in diameter. A computerized tomography with oral and intravenous contrast showed thickening of gastric and intestinal walls, multiple enlarged paraaortic and parailiac lymph nodes; spleen showed 11 hypodense lesions, largest being 17mm in diameter. On MRI, splenic lesions were hypointense at T1 and hyperintense at T2 with minute peripheral contrast enhancement . Preoperative suspicion was of hemangioma and lymphoma . She was consulted with oncology department for suspected malignancy and gastroenterology department for suspected Gilbert syndrome but ruled out on investigations. Upper and lower gastrointestinal endoscopy were performed and biopsies were taken that did not show any pathology. Beta blocker treatment was started with proton pump inhibitors but no improvement was seen on repeat MRI. Splenectomy was then performed with a suspicion of Littoral cell angioma as an alternate diagnosis. Grossly, the spleen had nodular spongy dark lesions, ranging 1 to 2cm in diameter . On histopathological examination, it was reported as multiple cavernous hemangioma. She remained well at the last follow up six months back. Vascular tumors are the most common neoplasms of the spleen. They arise form vascular elements i.e. the red pulp of the spleen. Signs and symptoms may vary from being asymptomatic to splenic rupture or hemorrhage.[1] Diagnosis is supported by preoperative workup, especially imaging investigations such as CT scan and MRI, but is quite challenging. It has no specific clinical features. Our patient presented with recurrent episodes of abdominal pain and diagnosed to have vascular lesions of the spleen as an incidental finding on USG.Ultrasound findings of Littoral cell angioma are nonspecific. On ultrasound it can vary from cystic, hypoechoic mass to a homogenously hyperechoic mass. On CT scan, it is iso-dense or slightly hyperdense (without contrast); with contrast it is hypodense on arterial phase with heterogeneous to homogenous enhancement on venous phase which is again nonspecific. MRI findings of Littoral cell angioma may differ depending on the amount of siderosis which is highly variable.Ultrasound features of hemangioma is a well-defined intrasplenic or pedunculated echogenic solid or complex cystic mass. At computerized tomography, cavernous hemangiomas appear as isodense or hyperdense due to its solid component. Splenic hemangiomas are hypo to isointense when compared with normal spleen at magnetic resonance imaging.[2] In our case, all radiologic work up was non-specific. We could only say that it was a vascular tumor of the spleen. More interestingly thickening of the wall of stomach and intestine were found at computerized tomography which also favored Littoral cell angioma as almost half of the cases have immunosuppression or malignancy. The other possibility was of lymphoma. The laboratory analysis of the patient revealed only mild hyperbilirubinemia which remained for three months. That’s why we also thought of Gilbert’s syndrome but no pathology found at specific work up.Because hemangioma is more frequent than Littoral cell angioma we preferred medical treatment with beta blocker drugs. In case the lesion is hemangioma, it mostly shrinks in few months after the treatment. In our case, three months medical therapy did not reduce the size or number of the lesions therefore Littoral cell angioma became our second differential. Littoral cell angioma can be managed with chemotherapy, partial splenectomy or splenectomy.[4] In our case, splenectomy was the only choice due to multiple lesions involving the entire spleen and moreover true nature of the lesions was also in question. Vascular lesions of the spleen though rare may sometimes become challenging to diagnose.Source of Support: NilConflict of Interest: None declared


Ewing sarcoma typically arises in bone and is unrelated to intraparenchymal small blue cell embryonal central nervous system (CNS) tumors previously designated primitive neuroectodermal tumors (PNETs). When the CNS is impacted, it is usually secondary to local extension from either the epidural space, skull, or intracranial or spinal metastases. Primary examples within the cranial vault are rare, usually dural-based, and are largely case reports in the literature. We detail four pediatric patients with solitary, primary intracranial Ewing sarcoma, all manifesting the archetypal EWRS1 gene rearrangement that confirms diagnosis.Neurosurgical Department records, spanning 21 years (1995–2016), were reviewed to identify patients. Demographics, clinical history, pathological/genetic features, and clinical course were retrieved from the medical record and personal files of the authors.EWSR1 mutation and were treated with an Ewing sarcoma regimen. Outcomes were variable, with one patient showing progressive metastatic disease and death 3 years after presentation, one patient with disease-free survival 10.5 years after completion of therapy, and one alive and well at the completion of therapy 1 year after diagnosis. One patient completed therapy recently with post-therapy scans showing no evidence of disease.Four patients, one male and three females, age 5 to 16 years, were identified. One presented in extremis from a large lesion, two with soft tissue masses, and the fourth as an incidental finding after being involved in a motor vehicle collision. Three had clear bony involvement: a 10-year-old girl with a large left temporal lesion had clear origin in the skull, with spiculated calcified striations throughout the mass; a 9-year-old girl presented with a bony left petrous apex mass; and a 16-year-old girl presented with a left temporal mass with extension to the dura and underlying bone erosion. Only the 5-year-old boy had a large left frontoparietal mass traversing the falx with no bony contact. All four tumors manifested the diagnostic EWSR1 mutation confirms the diagnosis of Ewing sarcoma and excludes other types of embryonal CNS tumors. Long-term disease-free survival is possible with adherence to the appropriate therapeutic regimen after gross surgical resection.Testing for the  It is considered a subtype of extraosseous EWS, given the most common origin is dura, and it is thought to account for only 1–4% of extraosseous EWS.EWSR1 rearrangement diagnostic of EWS. Staging work-up, including a CT chest and whole body bone scan, demonstrated no evidence of extracranial disease. She was treated with ifosfamide/etoposide followed by doxorubicin/cyclophosphamide/vincristine for one cycle. She then received focal radiation with daily carboplatin. Following radiation, she received cyclophosphamide for stem cell harvest followed by vincristine/doxorubicin and ifosfamide/etoposide. She subsequently underwent triple autologous transplant with high-dose carboplatin/thiotepa. Complications of treatment included high-frequency hearing loss and mild memory issues and cognitive delay. She remains without evidence of tumor 10.5 years after her initial presentation.A 16-year-old girl with a 2-week history of headaches and daily vomiting presented to the emergency department obtunded, with a fixed and dilated left pupil, and arousable only to pain. Computed tomography (CT) showed an intracranial left temporal mass exhibiting bone remodeling, indicative of probable bony origin Fig. a, and CDEWSR1 rearrangement diagnostic of EWS  translocation. This FLI1 translocation, present in 90–95% of EWS cases EWSR1 gene, which is an EWS-FLI1 fusion; the resulting protein is a known aberrant transcription factor that interacts with TP53 and p21.,EWS-FLI1 fusion gene include dysregulation of cell proliferation, differentiation, apoptosis, angiogenesis, invasion, and metastasis.EWSR1 rearrangement, with 91–100% sensitivity and specificity.EWSR1 rearrangement.Genetically, EWS is characterized by a translocation of the MIC2 gene, is seen in essentially all cases of EWS.MIC2 gene, and thus will not positively stain for CD99.Overexpression of CD99, a cell surface glycoprotein and a product of the Treatment of EWS includes maximal surgical resection with aggressive chemotherapy and focal radiation.Three of the four patients in our series received initial maximal surgical resection, followed by the either standard of care interval-compressed chemotherapy  or high-dose chemotherapy with autologous stem cell transplant (Case #1) and focal radiation. Two of these patients have had one and 10.5-year disease-free survival, respectively. The third patient pursued alternative therapies including homeopathy, diet, and IV vitamin C in addition to interval-compressed chemotherapy and succumbed to her illness after relapsing with widespread extracranial bony metastasis. The final patient has completed therapy and end of therapy scans demonstrated no evidence of disease. This patient did not undergo gross total resection as it was felt that total resection at the petrous bone was unlikely and the surgical risk was not justified.,,,Previous case studies of primary EWS involving the central nervous system have been reported.Salunke et al. examined a series of 10 cases of primary osseous intracranial EWS, six of which occurred in pediatric patients.Within our case series, none of the four patients presenting with primary intracranial EWS had metastases at time of diagnosis. However, one patient did develop metastases during her third relapse following interval compression chemotherapy, re-irradiation, and high-dose vitamin C therapy. Two patients have shown disease-free survival  and one patient is too early in her post-therapy monitoring to assess outcome, although end of therapy scans demonstrate no signs of disease.Our case series is small with some patients still relatively close to completion of therapy making it difficult to make recommendations for therapy based on this experience, but important considerations for therapy can be gleaned from larger studies of extracranial EWS. As discussed above, large collaborative studies have shown that interval-compressed chemotherapy results in improved survival outcomes and is now standard of care.With an increased understanding of the molecular diagnosis of CNS tumors, the importance of molecular studies for the definitive diagnosis of tumors is becoming more apparent. This has resulted in new classifications as outlined by the WHO Classification of Tumors of the Central Nervous System.EWSR1 gene rearrangement for accurate diagnosis, to ensure all patients are started on the most appropriate treatment to optimize outcomes.In conclusion, Ewing sarcoma typically presents as a PNET most commonly arising from the long bones or axial skeleton. Primary intracranial Ewing sarcoma is extremely rare and can easily be mistaken for CNS embryonal tumors. The cytogenetics, treatment regimen, and disease-free survival vary considerably between primary intracranial EWS and other more commonly seen CNS embryonal tumors. All supratentorial small round blue cell tumors should be tested for the


Objectives. Duplication cysts are rare benign lesions usually arising in the gastrointestinal tract. We report a case of a 52-year-old woman with an incidental gastric mass found on computed tomography during a pregraft workup for a familial cardiomyopathy. Methods. The mass was completely excised by partial gastrectomy and gross examination revealed a cystic lesion containing two small solid nodules in its wall. Microscopic evaluation and immunohistochemistry study were performed to further characterize the cyst and the nodules. A comprehensive literature review of the NCBI database PubMed was also carried out. Results. While the cyst was diagnosed as a foregut duplication cyst, the solid nodules proved to be concomitant gastrointestinal stromal tumor (GIST) and leiomyoma. Both morphologic features and immunohistochemistry stains, including CD117, smooth muscle actin, and CD34 supported the diagnosis. Clinical course was benign and the patient had no clinical evidence of relapse ten months following the surgical procedure. The literature search did not reveal any other published case of a foregut duplication cyst presenting in combination with a GIST and a leiomyoma. Conclusions. To our knowledge, this is the first case of a composite lesion comprising a foregut duplication cyst of the stomach along with a leiomyoma and a GIST. Gastrointestinal duplication cysts are rare tumors that can arise anywhere in the digestive tract . StomachMesenchymal tumors of the stomach are also infrequent neoplasms. Gastrointestinal stromal tumors (GIST) are the most common among them, with an incidence of 0,32 per 100 000 person-years . Sixty pLeiomyomas are benign neoplasms arising from the smooth muscle layer present along the gastrointestinal tract . GastricIn the present report, we describe the case of a 52-year-old asymptomatic woman presenting a foregut duplication cyst of the stomach in association with a low-grade GIST and a leiomyoma.A 52-year-old woman known for a familial dilated cardiomyopathy was evaluated in the context of a pregraft workup. A chest CT scan was performed and a lesion was incidentally detected in the stomach next to the gastric fundus. The lesion was described as exophytic and suspicious but was otherwise difficult to characterize on imagery. The patient had no gastrointestinal clinical symptoms and the physical examination was normal. An endoscopic ultrasound (EUS) was done and showed a 2 cm hypoechogenic and well-encapsulated nodule that seemed to originate from the gastric muscularis. No adenopathy was identified. Because of the unknown nature of the lesion and the likely need of immunosuppressant medication in the future for cardiac graft management purpose, it was decided to remove the mass for further characterization. A laparoscopic partial gastrectomy was performed. A minimally adherent 2 cm lesion located at the gastric fundus near the gastroesophageal junction and the left crus of the diaphragm was completely removed and sent for pathology examination see .The resected specimen consisted of a small portion of the stomach measuring 3.7 × 2.5 × 2.0 cm and showing a 2.5 cm cystic lesion. The cyst contained a brownish mucoid substance and showed a smooth regular lining except for two distinct areas of nodularities measuring, respectively, 0.5 and 0.2 cm. The largest nodule was partly submitted for frozen examination and a diagnosis of low-grade spindle cell neoplasm was made.Histologically, the cystic lesion showed a benign pseudostratified ciliated epithelium consistent with a foregut duplication cyst of the stomach. The 0.2 cm nodule was histologically characterized by proliferation of plump spindle cells without significant mitotic activity (less than 5 mitoses/50 HPF). As illustrated in http://dx.doi.org/10.1155/2016/1537240.In order to identify similar cases, an extensive literature review was performed using NCBI database PubMed in June 2015. The terms ((“Bronchogenic Cyst”[Mesh]) OR foregut cyst) AND (stomach) were used because the search using Mesh terms for bronchogenic cyst, leiomyoma, and GIST retrieved no results. The research was limited to English language. 70 citations were identified. Of those, 31 articles were available for reading and were retained. Five additional articles were identified in the references of the initially selected articles. A total of 36 articles with the description of 37 gastric foregut cyst cases were included in the present literature review. Thirty-seven cases of gastric foregut cyst were identified. None of them were associated with GIST or leiomyoma. Another literature review using the terms ((“Leiomyoma”[Mesh]) and ) showed one case of metachronous occurrence of GIST and leiomyoma at different locations. The summary of literature review is provided in Table 1 in the Supplementary Material available online at The present case report describes a very unusual location of foregut duplication cysts presenting in association with a GIST and a leiomyoma. To our knowledge, there is no known association between duplication cyst, GIST, leiomyoma, and idiopathic cardiomyopathy, as seen in our patient. Also, such a composite lesion has never been reported. The literature search for leiomyoma and GIST revealed only one published case of a 22-year-old girl with Carney triad who presented with metachronous gastric GIST and oesophageal leiomyoma . Unlike Thirty-seven cases of gastric foregut cyst have been identified in the literature review . There were 21 women and 16 men (22 to 81 years old). While the vast majority of patients were asymptomatic, some were investigated for symptoms resulting from the compression of surrounding organs. The sizes of the lesions ranged between 1.6 and 40 cm and they were located anywhere in the proximal stomach. All of them presented a pseudostratified columnar ciliated respiratory epithelium and some showed other features of the normal bronchopulmonary tree such as cartilage and glands. In most cases, diagnosis was established after complete excision. Cytological examination of fine needle aspirates permitted accurate diagnosis in only three patients. There were no associated tumors found.Foregut cysts are the result of abnormal pinching of the foregut during embryogenesis; however, their exact pathogenesis remains unknown . They usIn summary, we report an unusual case of a 52-year-old woman presenting a bronchogenic foregut cyst of the stomach in association with a leiomyoma and a GIST. To our knowledge, no similar cases have been reported. The present case provides new lines of evidence of neoplastic processes occurring within the bronchogenic cyst wall and contributes to the justification of their complete excision and of a thorough pathological examination.Comprehensive litterature review on gastric bronchogenic cysts using NCBI database PubMed.