MEN2A is a hereditary syndrome characterized by medullary thyroid carcinoma, hyperparathyroidism, and pheochromocytoma. Classically patients with a pheochromocytoma initially present with the triad of paroxysmal headaches, palpitations, and diaphoresis accompanied by marked hypertension. However, although reported as a rare presentation, spontaneous hemorrhage within a pheochromocytoma can present as an abdominal catastrophe. Unrecognized, this transformation can rapidly result in death. We report the only documented case of a thirty eight year old gentleman with MEN2A who presented to a community hospital with hemorrhagic shock and peritonitis secondary to an unrecognized hemorrhagic pheochromocytoma. The clinical course is notable for an inability to localize the source of hemorrhage during an initial damage control laparotomy that stabilized the patient sufficiently to allow emergent transfer to our facility, re-exploration for continued hemorrhage and abdominal compartment syndrome, and ultimately angiographic embolization of the left adrenal artery for control of the bleeding. Following recovery from his critical illness and appropriate medical management for pheochromocytoma, he returned for interval bilateral adrenal gland resection, from which his recovery was unremarkable. Our review of the literature highlights the high mortality associated with the undertaking of an operative intervention in the face of an unrecognized functional pheochromocytoma. This reinforces the need for maintaining a high index of suspicion for pheochromocytoma in similar cases. Our case also demonstrates the need for a mutimodal treatment approach that will often be required in these cases. Multiple endocrine neoplasia 2A (MEN2A) is a rare autosomal dominant syndrome caused by missense mutations in the RET proto-oncogene associated with medullary thyroid cancer, pheochromocytoma and hyperparathyroidism. Pheochromocytoma is a rare catecholamine-secreting tumor of the adrenal glands most often presenting with the characteristic symptoms of paroxysmal hypertension, palpitations, diaphoresis, and headache. Acute onset abdominal pain and nausea may be the only presenting symptoms of spontaneous intra-abdominal hemorrhage, a rare and highly lethal complication. We present a case of spontaneous intra-abdominal hemorrhage secondary to a ruptured pheochromocytoma, subsequent management, and a review of the literature.M.J., a 38-year-old man developed sudden severe abdominal pain, nausea, and vomiting after shoveling snow. Prior to this event, he denies having had any episodes of hypertension, tachycardia or diaphoresis, although several months prior he was diagnosed with essential hypertension and was started on lisinopril. In addition, he denied any recent abdominal or flank trauma. Of note, his past medical history is significant for a diagnosis of MEN2A which was made at the age of 18 months, and a prophylactic total thyroidectomy at age 10 secondary to elevated serum calcitonin levels. Since that time he has had no further follow-up, although of his two children, his daughter has been diagnosed with MEN2A and undergone a prophylactic total thyroidectomy 2 years prior to this event. On arrival, paramedics found him near syncopal and diaphoretic with a heart rate of 180 bpm and systolic blood pressure of 64 mmHg. Fluid resuscitation was initiated and the patient was taken to an outside hospital. Initial evaluation at the local level II trauma center was notable for a heart rate of 150 bpm, systolic blood pressure of 70 mmHg, diffuse peritoneal signs, a hematocrit of 34%, INR of 1.0 and PTT of 30.4. Following resuscitation with additional crystalloid and 2 units of packed red blood cells (pRBC), his hematocrit was 34%, INR 2.4 and PTT 66.2. A non-contrast abdominal computed tomogram revealed bilateral adrenal masses and a large amount of intra and retroperitoneal hemorrhage  was then fashioned for temporary abdominal closure. Following closure of the abdomen, the patient suffered cardiac arrest with pulseless electrical activity. Advanced cardiac life support measures were initiated and a perfusing rhythm was obtained shortly thereafter. Given the history of MEN2A and bilateral adrenal masses, the diagnosis of occult pheochromocytoma was entertained. The blood pressure swings were controlled with phentolamine and a sodium nitroprusside infusion with good effect. The patient was returned to the surgical intensive care unit for further management.In the intensive care unit, the patient continued to have a labile blood pressure, a persistent base deficit, decreasing hematocrit and drainage of large amount of blood from the VAC, therefore he was emergently taken to interventional radiology. Diagnostic angiography revealed contrast extravasation from the left adrenal artery which was embolized with 250 micron Embozene™  microspheres and Gelfoam™  slurry to good effect  and 24.3 nmol/L (reference range 0.0-0.89) respectively. Thereafter, his recovery was relatively unremarkable; he underwent two additional procedures to restore bowel continuity and for abdominal wall closure. He was discharged in good condition to a rehabilitation facility on hospital day 25 with instructions to continue taking phenoxybenzamine and labetolol. He returned after approximately 4.5 months for a bilateral retroperitoneoscopic adrenalectomy. Of note, intra-operatively, scarring and adhesions were noted between the left adrenal gland and surrounding periadrenal and perirenal fat. Final pathologic examination revealed a 5 cm right and 4 cm bi-lobed left adrenal  or Sipple Syndrome is an autosomal dominant syndrome, first described by Sipple  and latePheochromocytomas are rare, catecholamine secreting, yellowish-brown tumors composed of chromaffin cells derived from embryonic neural crest cells which were first described by Frankel  in 1886 123 or I131 MIBG scintigraphy or PET may be utilized when CT or MRI fail to reveal the lesion or if malignancy is suspected.Classically patients initially present with the triad of paroxysmal headaches, palpitations, and diaphoresis accompanied by marked hypertension. Of interest, it is estimated that pheochromocytomas are present in 0.1-0.6% of patients with hypertension . In addiAlthough both Roux (Switzerland) and Mayo (US) are credited with concomitantly performing the first successful resections of pheochromocytomas in 1926, neither described any peri-operative hemodynamic instability, and both patients survived . HoweverImplementation of these management principles in the emergent setting can often be challenging as patient presentation can be widely variable, ranging from minor retroperitoneal hemorrhage with hypertension or abdominal pain to shock and impending cardiovascular collapse. In the setting of a contained retroperitoneal hemorrhage, every effort should be made to avoid emergent or urgent surgical intervention. Not surprisingly, review of the literature reveals a mortality of ~25% associated with emergent surgical intervention for contained hemorrhage; in contrast, adequate medical preparation as described above results in a mortality rate similar to that observed for elective adrenalectomy in the absence of hemorrhage. Medical optimization should include adequate blood resuscitation, correction of any coagulopathy to limit continued hemorrhage, hemodynamic support as needed, and ultimately α-blockade followed by volume expansion and β-blockade in an in-patient setting. This simplistic algorithm must be tempered by the recognition that providing supportive care in the setting of cardiovascular collapse mediated by adrenal compression from an evolving retroperitoneal hematoma and the resulting catecholamine excess may tax even the most advanced intensive care unit. Emergent surgical intervention may be considered in cases refractory to maximal medical management as recently described by May and colleagues  with recSpontaneous hemorrhage within a pheochromocytoma resulting in capsular rupture and retroperitoneal or intra-peritoneal hemorrhage has long been recognized as a rare, but catastrophic and highly lethal event. In addition, trauma  and mediIn the present case, we were faced with a unique set of circumstances which dictated an unconventional course of management. Although the patient's medical history notable for total thyroidectomy as a child and the presence of the bilateral adrenal masses raised suspicion for MEN2A and possible pheochromocytoma, given his initial presentation in extremis with hemoperitoneum the decision to undertake an emergent exploratory laparotomy was warranted. Although only partial control of the hemorrhage was obtained at that time, it should be stressed that inaction may have resulted in this patient's demise given the historically observed 100% mortality in the face of conservatively managed intra-peritoneal hemorrhage from pheochromocytoma. Upon arrival to our facility, we were faced with an evolving abdominal compartment syndrome in addition to acute hemorrhage of unclear etiology. In the course of the second laparotomy, hemodynamic instability, the need to address the sequelae of abdominal hypertension, and worsening coagulopathy precluded further exploration of the LUQ for the continued source of hemorrhage. Moreover, given the presence of bilateral adrenal masses in the setting of a history of MEN2A, further exploration of the adrenals without proper α-blockade presented addition significant risk of morbidity and mortality. Therefore the decision was made to proceed with angiographic embolization in the setting of continued bleeding.TAE as a therapeutic option for pheochromocytoma was first described in 1978 by Bunuan  and collSpontaneous intraperitoneal hemorrhage remains a rare complication of pheochromocytoma, though the physiologic consequences present considerable medical and surgical challenges. Unfortunately, the resulting emergent clinical scenario does not allow for optimal pre-operative medical preparation with α-adrenergic blockade, and as such is associated with a high mortality. The present case has demonstrated the importance of multi-modal therapy including the need for emergent surgical intervention and the availability of interventional radiology for control of the hemorrhage. Most importantly, a high index of suspicion must be maintained in similar cases so that the highly lethal hemodynamic sequelae may be anticipated and managed with the appropriate pharmacologic agents to ensure optimal outcomes.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.MEN2A: Multiple Endocrine Neoplasia Type 2A; pRBC: packed red blood cells; FFP: fresh frozen plasma; LUQ: left upper quadrant; TAE: transcatheter arterial embolization; APRV: Airway Pressure Release VentilationThe authors declare that they have no competing interests.JH participated in the surgical and critical care of this patient and drafted the manuscript. PS participated in drafting the manuscript. CS, EK and RA participated in the surgical care of this patient and critical review of the manuscript. All authors have read and approved the final manuscript.


Adrenal lipomas are rare, small, benign, non-functioning tumors, which must be histopathologically differentiated from other tumors such as myelolipomas or liposarcomas. They are usually identified incidentally during autopsy, imaging, or laparotomy. Occasionally, they may present acutely due to complications such as abdominal pain from retroperitoneal bleeding, or systemic symptoms of infection. We report a giant adrenal lipoma  clinically presenting with chronic mild postprandial pain.A 54-year-old Caucasian woman presented several times over a period of 10 years to various emergency departments complaining of long-term mild postprandial abdominal pain. Although clinical examinations were unrevealing, an abdominal computed tomography scan performed at her most recent presentation led to the identification of a large lipoma of the left adrenal gland, which occupied most of the retroperitoneal space. Myelolipoma was ruled out due to the absence of megakaryocytes, immature leukocytes, or erythrocytes. Liposarcoma was ruled out due to the absence of lipoblasts. The size of the lipoma (16 × 14 × 7 cm) is, to the best of our knowledge, the second largest reported to date. After surgical resection, our patient was relieved of her symptoms and remains healthy six years postoperatively.Physicians should be aware that differential diagnosis of mild chronic abdominal pain in patients presenting in emergency rooms may include large adrenal lipomas. When initial diagnostic investigation is not revealing, out-patient specialist evaluation should be planned to enable appropriate further investigations. Adenomas, pheochromocytomas, and adrenocortical carcinomas represent the most common tumors of the adrenal glands . In contA total of 18 cases have been reported since 1966, as summarized in Table We report the first adrenal lipoma presenting as chronic mild postprandial pain, also the second largest among the 19 cases reported thus far -4,6-14.A 54-year-old Caucasian woman presented to our emergency department complaining of mild postprandial abdominal pain and fullness with a duration of 10 years. Her medical history included hypertension, diabetes mellitus and hypercholesterolemia.2. Abdominal inspection was unremarkable. No murmurs were auscultated and abdominal sounds were normal both in frequency and in tone. There was mildly increased tympany on periumbilical percussion with no dullness. Abdominal palpation was unrevealing. The same applied to bimanual examination of both hypochondric areas. The remainder of the physical examination was normal, as were laboratory tests.On physical examination, our patient was obese with a body mass index of 31 kg/mAn abdominal CT scan was performed with oral administration of contrast. A large retroperitoneal mass (16 × 14 × 7 cm) was found, arising from the left adrenal gland and surrounded by a thin capsule. It exhibited both the density of adipose and solid tissue and was adjacent to but not invading the small intestine, pancreas, spleen, and left kidney. There was no evidence of lymph node or vascular infiltration  were examined by an experienced pathologist. Microscopically, the tumor consisted of large adipocytes without nuclear atypia or mitoses. In several locations, edema or fibrosis by connective tissue with low cell density were observed. Diffuse inflammatory, mostly lymphocytic and occasionally plasmatocytic, infiltrations and occasional depositions of hemosiderin were observed. Small loci of hemorrhage were observed in areas where the cortex was adjacent to the adipose tissue of the tumor. No megakaryocytes were found. Moreover, erythrocytes and leukocytes found were clearly mature, hemorrhagic, with inflammatory infiltrates . Thus, tOur patient had an uncomplicated postoperative course and was discharged on the ninth postoperative day in good condition. She became entirely asymptomatic and follow-up was uneventful for six years postoperatively.Lipomas are benign tumors of uncertain origin that may develop throughout the body. Most originate in the gastrointestinal tract (65% to 75% in the colon). Lipomas of the adrenal gland are very rare -5. The mHistologically, adrenal lipomas consist of bright yellow fat separated by fine fibrous trabeculae. Microscopically, they are composed of mature adipose tissue without cellular atypia. Areas of necrosis, infarct, hemorrhage, inflammatory infiltrates, and calcification may be present. Unlike adrenal myelolipomas, no immature hemopoietic cells exist in adrenal lipomas . It is iThe rising number of adrenal lipomas reported over the past several years may be attributable to the increasing use of improved imaging techniques ,6. CT caThere are two case reports in the literature on adrenal lipomas presenting with acute symptoms. The first describes abdominal pain secondary to hemorrhage  and the In the literature, the diameters of adrenal lipomas range from 1.1 cm to 20 cm (Table Lipomas may rarely be found in the adrenal gland. They are usually asymptomatic but occasionally manifest with acute complications such as hemorrhage or infection. This is the first report of an adrenal lipoma presenting with chronic symptoms of mild postprandial abdominal pain and fullness. Our lipoma is the second largest reported in the literature to date. Physicians of all medical specialties should be aware that the differential diagnosis of mild chronic abdominal pain in patients repeatedly presenting to emergency rooms may include large adrenal lipomas. We recommend that these patients, in whom initial diagnostic investigation is unrevealing, merit further investigation. This holds especially true when empiric treatment is unsuccessful. Such patients should be referred to an out-patient specialist clinic for further clinical and laboratory investigation, including abdominal CT scans.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.SK and AT made the diagnosis and performed the adrenalectomy. ID and SK evaluated the medical literature and prepared the manuscript. AF interpreted the histology and edited the final manuscript before submission. All authors read and approved the final manuscript.


Inflammatory external root resorption (IERR) after orthodontic treatments is an unusual complication. This case report describes a non-vital maxillary premolar with symptomatic extensive IERR (with a crown/root ratio of 1:1) after receiving orthodontic treatment. The first appointment included drainage, chemo-mechanical preparation of the canal and intra-canal medication with calcium hydroxide (CH) along with prescription of analgesic/antibiotic. The subsequent one-week follow-up revealed the persistence of symptoms and formation of a sinus tract. Finally, extraoral endodontic treatment was planned; the tooth was atraumatically extracted and retrograde root canal filling with calcium enriched mixture (CEM) cement was placed followed by tooth replantation. Clinical signs/symptoms subsided during 7 days postoperatively. The sinus tract also resolved after one week. Six-month and one-year follow-ups revealed complete healing and a fully functional asymptomatic tooth. This case study showed favorable outcomes in a refractory periapical lesion associated with orthodontically induced extensive IERR. The chemical as well as biological properties of CEM cement may be a suitable endodontic biomaterial for these cases. Inflammatory external root resorption (IERR), is one of the complications most frequently associated with inflammation of the periradicular tissues stemming from the microorganism-induced intracanal infection; which mostly leads to progressive damage of root structure and finally tooth loss . Other cExperts are of the opinion that vital teeth with orthodontic induced IERR do not need to undergo endodontic therapy, as this approach does not eliminate the etiologic factor of the condition . HoweverExtraoral endodontic treatment (EET) may be performed with a retrograde approach, which includes cleaning/shaping of the root canal system and filling the prepared root-end with appropriate biomaterial. For this purpose, different materials such as amalgam, gutta-percha, zinc oxide-eugenol cements, glass ionomer cement, gold foil pellets, Cavit, composite resin, mineral trioxide aggregate (MTA) and calcium enriched mixture (CEM) cement have been used , 6; amonThis case report represents the diagnosis and treatment challenges of an EIRR lesion around a symptomatic non-vital maxillary premolar, which had previously been under orthodontic treatment; also extraoral endodontic treatment of this tooth using CEM cement and up to one-year follow-up results are presented.º C. Intraoral examination revealed a diffused swelling in the apical area of the involved tooth, class II tooth mobility and severe tenderness to percussion and palpation; periodontal probing was within the normal range (<3mm). Cold test with Endo-Frost cold spray  elicited no response in involved tooth; the adjacent teeth revealed a positive response to cold test. The periapical x-ray revealed a deep restoration adjacent to the pulp and a large radiolucent periradicular lesion surrounding the open-apex and resorbed root , the involved tooth was isolated and the access cavity was prepared. On entering the pulp chamber, a purulent drainage was observed, and patient’s tenderness immediately subsided. After radiographic working length determination, cleaning and shaping of the root canal was performed; the root canal was irrigated and filled with 5.25% sodium hypochlorite for 15 min along with passive instrumentation. CH powder  and normal saline were mixed to prepare a creamy paste which was then placed within the root canal after canal drying with large paper points . A TID pus tract  and clasAfter administering local anesthesia via infiltration of 2% lidocaine with adrenalin 1:80000, the tooth was atraumatically extracted; meanwhile the apical lesion was curetted and sent for histopathological examination. While holding the tooth in a forceps, the root canal was retrogradely prepared using Peeso reamers #2  and copious saline irrigation; finally the cavity was dried with sterilized paper points. Then CEM cement powder and liquid  was mixed according to the manufacturer’s instructions. CEM was retrogradely inserted and packed within the root canal using a measured plugger. The tooth was replanted into its socket and the accurate repositioning was confirmed radiographically . HistopaThe treated tooth was rechecked after 10 days. The clinical signs/symptoms had resolved and the sinus tract had disappeared. The healing process was uneventful; the periodontal examination showed normal sulcular depth and normal gingival status. Clinical examination at the 6-month and 1-year recalls revealed satisfactory clinical function, the absence of pain/tenderness to percussion/palpation, as well as the absence of tooth mobility and signs/symptoms of inflammation or infection. Periapical radiographs revealed healing of the periapical lesion substituted with newly formed bone; external root resorption of involved tooth ceased without radiographic signs of replacement resorption .Extraoral endodontic treatment (EET) is rewarded by some researchers; and they suggest considering EET as the last option after failure of other endodontic treatments . On the It is stated that EET has several advantages over endodontic surgery since it is less complicated, protracted, invasive, and expensive . In the Management of IERR is based on removing bacteria and their byproducts from the root canal space as well as dentinal tubules to stop the inflammatory processes and allow healing of periodontal tissues . The conSurgical endodontic literature supports a direct cause-and-effect relationship between creations of hermetic apical seal by endodontic filling materials and successful outcomes ; an ideain vivo studies specifically in the field of regenerative endodontics [Sealing ability of CEM cement as root-end filling material is comparable with MTA . An inteodontics  and vitaodontics -32, showodontics , 34. Furodontics  or as a odontics .Extraoral retrograde root canal filling with CEM cement in a refractory apical lesion caused by orthodontically induced extensive IERR, may be a suitable treatment choice in current practice.


Myxofibrosarcoma has been recently described as a distinct histological entity and commonly affects the extremities and trunk of the elderly. It is, however, rare in the head and neck (2.8%) region, and we are unaware of any reports of it presenting as a parotid mass. The epitheloid variant accounts for less than 3% of myxofibrosarcomas. We report a case of a 78/F presenting with an epitheloid myxofibrosarcoma in the parotid gland. The clinical presentation was of a parotid lump of 9-month duration, and the diagnosis was made following thorough histological assessment. We present what we believe to be the first reported case of a (epitheloid) myxofibrosarcoma affecting the parotid gland and highlight its diagnostic features and outcome of treatment. Myxofibrosarcoma has been recently described as a distinct histological entity and commonly affects the extremities and trunk of the elderly –4. It isA 78-year-old female presented with a lump in the right parotid region of a nine-month duration. Clinical examination revealed a 4 × 3 cm mildly tender, mobile mass with normal facial nerve function. There was no palpable cervical lymphadenopathy. An MRI scan revealed a 3.8 cm well marginated mass largely confined to the superficial lobe of the parotid and involving the lateral edge of the deep lobe, . Fine ne The parotid gland (60 × 40 × 25 mm) was weighing 28 grams. One pole contains a multinodular tumor nodule (36 × 24 × 24 mm) with a gelatinous myxoid cut surface. A second irregular fragment of parotid tissue measuring (75 × 17 × 15 mm) was weighing 6.5 grams. The tumour was well circumscribed and had well-defined outer margin with a pseudocapsule in places . It was Myxofibrosarcoma, often reported in the past as the myxoid variant of the malignant fibrous histiocytoma, was initially suggested as a distinct histological entity by Angervall et al. in 1977 . The lesThe mainstay of treatment is excision of the lesion with wide margins, supplemented if necessary by radiotherapy . Local rThe quality of primary surgery does not only improve local recurrence, but also translates into survival benefit, suggesting that thorough reexcision or adjuvant radiotherapy is warranted for lesions with positive margins , 9.


Dear Editor,The resemblance of multiple sclerosis lesions to brain tumours is encountered in about 1% of cases and differentiation between these diagnoses remains difficult , 10. In We describe the case of a 57-year-old banana plantation worker with right-sided weakness, trouble in finding words and an organic psychosyndrome. Even though receiving corticosteroids, his symptoms aggravated.Magnetic resonance imaging (MRI) disclosed a single, left parietal periventricular lesion with a diameter of 34 mm and displaying rim-like contrast enhancement without significant mass effect Fig. .Fig. 1UDuring resection, clear 5-ala fluorescence was observed, useful for defining resection margins. The ventricular wall was opened and no fluorescence was found intraventricularly nor near to the choroid plexus.Intraoperative histology was compatible with astrocytoma. Final histological examination disclosed lymphocytic cuffs in the Virchow-Robin spaces, reactive astrocytes, partly with multiple nuclei, and abundant infiltrations of macrophages Fig. . A seconFive months after surgery, the patient suffered from slightly spastic hemiparesis, with ability to walk a few steps and to grasp and hold objects with the right hand. MRI control did not detect any sclerotic plaques nor gadolinium-enhancing lesions.Although diagnostic problems between multiple sclerosis and glioma have been described before , this caDifferentiation between multiple sclerosis and acute demyelinating encephalomyelitis (ADEM) at first onset in the adult age is under debate . In our Few reports deal with the relationship between activated lymphocytes and the porphobilinogen pathway , 9. ConcIn our case, the occurrence of intraoperative fluorescence can be explained by functional inhibition of ferrochelatase activity in activated lymphocytes . The act5-Ala-induced fluorescence is not restricted to malignant tumour cells. Cells with an inhibition of the ferrochelatase pathway, such as activated lymphocytes, are prone to accumulate protoporphyrin IX. Solitary multiple sclerosis plaques thus remain a rare, but important differential diagnosis of malignant glioma.


We report a case of embryonal orbital rhabdomyosarcoma (RMS) in a five year old boy. Immuno-histochemistry of incisional specimen confirmed diagnosis. Eight cycles of chemotherapy along with radiotherapy resulted in over 50% reduction in size of the mass. However, increase in size was noted subsequent to completion of therapy and exenteration was deemed prudent. Margins of the excised specimen were free from tumor cells, but after five months, the patient developed multiple metastases, including skeletal muscle involvement, and died nine months after exenteration, despite repeat chemotherapy along with radiotherapy. Orbital RMS with metastasis to skeletal muscle is a rare entity. Marked improvement in RMS survival over the past seen with the use of recent therapeutic protocols including primary radiotherapy and chemotherapy.[Rhabdomyosarcoma (RMS) is the most common primary malignancy of the orbit in children, accounting for 4% of all malignant disease in children.2 Horn anotherapy.A five-year-old boy presented to us with rapid onset of downward eccentric protrusion of right eyeball over a period of one month.  Extra ocThe orbital RMS is usually fast growing an infiltrative and often appears as an enlarging, painless mass. The initial symptoms may mimic differential diagnosis which includes inflammetry process, orbital cellulitis, metastatic neuroblastoma, chloroma, lymphangioma and ruptured dermoid cyst.In orbital RMS metastases are commonly hematogenous in origin; spread most often occurs to the lungs, liver and bones. RegionalIntergroup Rhabdomyosarcoma Study Group (IRSG) whose protocols now from the backbone of modern treatment of rhabdomyosarcoma. The progRecently excision biopsy is preferred more than incisional biopsy as it confirms diagnosis as well as it removes the tumor too, which is significantly helpful for better outcome. ExenteraThough tumor-free margins are thought to be a key element in establishing surgical cure of a patient with any form of neoplasia, which was the case following exenteration in our patient, few published reports,10 highli


Benign cystic mesothelioma of the peritoneum (BCM) is an uncommon lesion with some 130 cases reported since the first case described by Smith and Mennenmeyer in 1979. It is a rare intra abdominal tumor occurring predominantly in women of reproductive age. Due to the rarity of this tumor, similarity of patient presentation, and comparable features on imaging, the diagnosis of this pathology is difficult, and is based on histological findings. This tumor is known for local recurrence. It's agreed that surgery is the only effective treatment, but there are no evidence-based treatment strategies for BCM. Benign cystic mesothelioma of the peritoneum (BCM) is a rare intra abdominal tumor with a strong predilection for the peritoneum of pelvic organs. Symptoms are not specific, and the differential diagnosis is vast, including cystic lymphangioma, mucinous cystadenoma, cystic teratoma and pseudomyxoma retroperitonei. There are no evidence-based treatment strategies for BCM, and even if it is considered as a benign tumor, this tumor has a high local recurrence rate.We report a new case of BCM, which appeared as a surgical emergency.A 71 year-old woman presented to the emergency department complaining of history of abdominal pain since 2 days accompanied by diarrhea. Four months prior to presentation, she noticed an increase in abdominal girth. Moreover, she developed occasional abdominal discomfort, which slowly increased frequency. The patient also developed symptoms of constipation and severe reflux which were not improved by taking laxatives and a proton pump inhibitor. Our patient was hemodynamically stable with temperature at 37.9°C, and blood pressure was 130/80 mmHg. Abdominal examination was marked by diffuse abdominal distension, and tenderness. Computed tomography (CT) scanning showed a large spherical multi-loculated cystic mass in the abdomen which was not-communicating to any abdomen viscera, occupying nearly the entire abdominal cavity, displacing the hole bowel  was described first by Mennenmeyer and Smith . It’s a Benign cystic mesothelioma of the peritoneum (BCM) is a rare tumor with a high local recurrence rate. It requires optimal care in a specialized center especially as there is no evidence-based treatment strategies.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images.All authors declare that they have no competing interests.EBH and AB participated in writing the case report and revising the draft, OM, EB, AO, KM and KAT participated in the follow up. All authors read and approved the final manuscript.


A 13-year-old patient with a complaint of worsening lower abdominal pain during the past 4 months was admitted to the emergency department. An abdominopelvic ultrasound scan revealed a distended uterocervical cavity suggestive of hematometrocolpos. Imperforate hymen was observed on examination of the external genitalia. MRI scan revealed an air-fluid level representing pyometrocolpos within a distended vagina. Posterior vaginal extraperitoneal leakage as the sign of a fistula between the vagina and the rectovaginal space was detected. Although laparoscopic approach was planned, malodorous pus expelled after the insertion of the Veress needle, it was decided to proceed to laparotomy. Pus with peritoneal microabscess formations was observed at laparotomy. The imperforate hymen and TVS were excised vaginally. A more complex anomaly should be suspected in cases with hematometra and concomitant imperforated hymen without any bulging and thorough evaluation using radiological imaging techniques should be performed before surgical approach. The prevalence of Mullerian duct anomalies, although rare, ranges from 0.001% to 10% in general population and 8–10% in women with an adverse reproductive history . TransveA 13-year-old premenarchal girl was admitted to the emergency department with a complaint of worsening lower abdominal pain during the past 4 months. Secondary sexual characteristics had developed normally; she had Tanner stage IV breasts and normal pubic and axillary hair development compatible with her age.3 and C-reactive proteins were low. An abdominopelvic ultrasound scan revealed a distended uterocervical cavity suggestive of hematometrocolpos compressing the urinary bladder without any other pathology of the genitourinary system. For a thorough evaluation, the patient was taken to the operating theater to be examined under anesthesia. After rectal examination, the presence of imperforate hymen was confirmed and a tender soft mass in the upper part of the vagina was detected. The imperforate hymen was excised and a short vaginal blind pouch of 2 cm bearing vaginal rugae was visualized. As the incision did not result in drainage of the collection, presence of a thick TVS was suspected. The patient was scheduled for a magnetic resonance imaging (MRI) scan. While waiting for her appointment for MRI scan at the hospital, the patient had a worsening abdominal pain with vomiting and her leucocyte count was elevated from 5800/mm3 first to 13,000/mm3 and within 6 hours reached to 15,000/mm3. There was only 24 hours between trial of hymenectomy and MRI scan. MRI scan revealed an air-fluid level representing pyometrocolpos within a distended vagina, endometrial cavity, and endocervical canal. Posterior vaginal extraperitoneal leakage was detected and it was interpreted as the sign of a fistula (long arrow) between the vagina and the rectovaginal space (The absence of a hymeneal opening without any bulging was observed on examination of the external genitalia. The abdomen was tender and distended. At admission, her laboratory investigations were within normal limits with a leucocyte count of 5,800/mmal space . The leval space . AlthougThe uterus and bilateral tubes were normal in size and shape for the age of the patient; they were pink and fresh looking. A close observation of the uterus and cervix revealed no possible perforation sites that would explain intraabdominal spillage of the pus. A general surgeon was invited to the operation to rule out a probable rectal fistula but neither a fistula nor a perforation site was observed. As the probable communication or the tract between the abdomen and vagina was not observed, it could not be resected or repaired. In order to provide the drainage of pyocolpos, a 1 cm transverse incision at the superior part of the lower uterine segment was performed and with the guide of a number 8 Hegar dilator that was inserted from the incision site and then passed through the cervix into the vagina blindly, the septum was localized . Then thVarious congenital anomalies of the female tract such as agenesis, failure of vertical or lateral fusion, and failure of canalization occur when normal development of Mullerian duct disrupts in any stage of the developmental milestones. The caudal ends fuse in the midline and are occluded by the Mullerian tubercle that forms the hymen. Failure of this step with degeneration of the epithelial plate results in imperforate hymen. A transverse vaginal septum results from a defect of incomplete vertical fusion between Mullerian tubercle and urogenital sinus with failure of canalization of the vaginal plate . With cuIn the current case, a transverse vaginal septum and imperforate hymen were detected and the level of TVS was the middle third of the vagina. When hematometrocolpos was firstly diagnosed, clinical presentation of the patient seemed to be restricted to an obstructive pathology only. The pathology was complicated with infection and development of pyometrocolpos with a fistula between the vagina and the rectovaginal space. There was only 24 hours between trial of hymenectomy and the laparotomy that revealed the presence of abscess, so the abscess formation was not thought to be related with the initial procedure. The mechanism of fistula formation was not clear due to its unique nature. As the patient was 13 years old and she had the onset of complaints 4 months ago, presumably her menarche began only 4 months ago and accumulation of the menstrual blood and causing the described entity took months. We are not sure whether this duration is enough for formation of a chronic fistula with an epithelialized tract. As this was not present, it might well be due to spontaneous rupture of the abscess. Therefore, we addressed the probable mechanism that might explain the formation of the fistula or the tract as increased intracavitary pressure due to gradually accumulating collection of the infectious material and failure of drainage because of the resistance of thick TVS. The probable mechanism for Escherichia coli growth from the culture was thought to be related to the close proximity to the bowel.Although it is well known that hematometra is a good medium for infectious development, occurrence of severe, extensive infection in the abdomen with a healthy looking uterus and tubes in this case was hard to explain.To our knowledge, the present case is the first reported case of a fistula formation between posterior fornix and rectovaginal space associated with pyocolpos and obstructive multiple Mullerian anomalies. A more complex anomaly should be suspected in cases with hematometra and concomitant imperforated hymen without any bulging and thorough evaluation using radiological imaging techniques should be performed before surgical approach. In these pathologies, other female reproductive tract anomalies and abnormalities of the renal and skeletal systems are often related because of the embryogenetic origin. The evaluation of these anomalies should be done regarding these coexisting defects and the right treatment should be planned after defining the anatomy of the pathology as definitely as possible with proper imaging techniques such as an ultrasound scan and MRI.


Malignancies of the anal margin and perianal skin are relatively uncommon, and account for approximately 2% to 3% of all anorectal malignancies. Despite the fact that gastrointestinal presentation of lymphoma is not uncommon, primary localization of mantle cell lymphoma to the perianal region is rare.We present the case of a 64-year-old Turkish man with a rapidly progressive perianal mass. Our patient had previously required medical treatment on multiple occasions for hemorrhoidal disease; however, the treatment was ineffective and gross lymph nodes were noted in his left inguinal region. Following excision of the mass and his lymph nodes, the pathological diagnosis of both tissues was mantle cell lymphoma.Although gastrointestinal presentation of non-Hodgkin lymphoma is common, the literature includes only a few cases of perianal localization. Our case illustrates the importance of suspicion and complete examination of perianal masses. In practice, examination of the inguinal region should be a part of routine proctological examination. Malignancies of the anal margin and perianal skin are relatively uncommon, and account for approximately 2% to 3% of all anorectal malignancies. The most common anorectal malignancies are squamous cell carcinoma, Bowen’s disease , adenocarcinoma, basal cell carcinoma and malignant melanoma. A Buschke-Lowenstein tumor, or verrucous carcinoma, is a benign lesion of the perianal margin and is not considered an anorectal malignancy .Mantle cell lymphoma (MCL) is a subtype of B-cell non-Hodgkin lymphoma and comprises about 7% of all cases of adult non-Hodgkin lymphoma. Approximately 25% of patients with MCL present with extra-nodal disease, of which 20% occur in the gastrointestinal (GI) tract. Multiple lymphomatous polyposis is a rare primary GI manifestation of MCL [A 64-year-old Turkish man presented to our surgical clinic with a rapidly progressive perianal mass Figure a,b. Our Following excision of the mass and lymph nodes, a pathological diagnosis of MCL was made for both tissues Figure . No evidPatients with perianal lesions often present with common perianal complaints, including itching or burning, bleeding, pain, drainage or a mass . HoweverMCL is an aggressive B-cell lymphoma with a propensity to involve extra-nodal sites, including the colon. Secondary colonic involvement of MCL occurs in 88% of patients. Primary GI lymphomas are relatively uncommon, representing 1% to 4% of malignant tumors of the GI tract . EndoscoPerianal masses have a variable etiology and most are benign; however, perianal presentation of unusual disorders such as MCL should always be a consideration. Although colorectal presentation of MCL is rare, a high index of suspicion among surgeons can prevent delays in diagnosis and treatment.Written informed consent was obtained from the patient for publication of this case report and all accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.GI: gastrointestinal; MCL: mantle cell lymphoma.The authors declare that they have no competing interests.BG analyzed and interpreted the patient data regarding characteristics of the disorder. BG and AO were the major contributors in writing the manuscript. EO was the primary surgeon and had the main role in literature researching. HON was responsible for the pathological review. TY was the mentor in all processes and determined the pattern of case presentation. All authors read and approved the final manuscript.


Chordomas are rare tumors of the skeletal system that arise from an intra-osseous benign precursor of notochordal cells. They are mainly locally aggressive. However, metastases to other sites, including the humeri, resulting in pathological fractures have been reported. We report the case of a patient with a metastatic chordoma that produced a pathologic fracture of the humerus.We report the case of a 60-year-old Iranian woman who presented with a fracture of her right humerus following a minor trauma. She had a history of a sacrococcygeal chordoma. Histological and immunohistochemical studies of the fracture site suggested the diagnosis of a chordoma.Chordoma is a rare tumor and rarely metastasizes, but it should be considered in the differential diagnosis of epithelioid bone tumors. The only current effective treatment for this type of tumor is carbon ion therapy. There is currently no effective medical therapy available for advanced chordoma, and this type of tumor is not very responsive to radiotherapy. Chordomas are rare, low-grade, primary malignant bone tumors arising from primitive notochord remnants of the axial skeleton. The sacrum is the most common anatomical site of origin, accounting for 50% to 60% of all cases, followed by the skull base region , accounting for 25% to 35% of cases; the cervical vertebrae, accounting for approximately 10% of cases; and the thoracolumbar vertebrae, accounting for approximately 5% of cases .These tumors are mainly locally aggressive, with a high incidence of local recurrence and a poor long-term prognosis. Metastatic lesions have been reported in the liver, lungs, lymph nodes, peritoneum, skin, heart, humeri, brain, and distant regions of the spine -7.3, platelets 200 U/L, sodium 141 mEq/L, potassium 4 mEq/L, glucose 11.6 mmol/L, blood urea nitrogen 15.1 mmol/L, and creatinine 250 mmol/L. A plain X-ray showed a pathologic fracture in the mid-shaft of her right humerus and a large, ill-defined soft tissue mass (Figure A 60-year-old Iranian woman presented to our hospital with pain and swelling in her right arm following a minor fall. Our initial physical examination revealed her to be in a good general condition. She was normotensive, with a pulse rate of 92 beats/minute. Her other laboratory values were as follows: body temperature 37°C, hemoglobin 12.2 g/dL, leukocytes 22 cells/mmChordomas arise from the remnant of the fetal notochord and grow slowly. The most common location is the sacrococcygeal region. This tumor is generally reported in adults; most patients are in the fifth to seventh decades of life -12. ClasThe biological behavior of chordomas differs from patient to patient. Some patients harbor tumors capable of aggressive behavior both locally and distantly, while others have tumors that behave more indolently. There is no good method for predicting the behavior of individual chordomas ,11. TherChordomas are rare tumors. They are relatively radioresistant, and currently there is no effective medical therapy for treating them. Complete local excision is the most effective treatment for achieving a long-term cure. Although these tumors rarely metastasize, they need to be considered in the differential diagnosis of metastatic epithelioid bone tumors.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.NA participated in the histology-related issues and drafted the manuscript. SSo contributed to all of the surgical aspects of patient care and revised the respective sections in the manuscript. SSe participated in the radiological aspects of the case and provided input to the case discussion. RM contributed to the review of the literature and provided clinical insights. All authors read and approved the final manuscript.


To report a case of keratitis and a case of conjunctivitis caused by synthetic fibers from toy teddy bears.Case stories with histopathological analysis. 1) A two-year-old girl developed a severe case of keratitis and corneal ulceration. The initial treatment with various antibiotics gave no improvement and eventually the patient developed spontaneous perforation of the cornea. The corneal swabs contained no bacteria or fungi. Corneal grafting was performed and the corneal button was sent for histopathological examination. 2) A five-year-old girl presented with ocular irritation in her left eye. Examination revealed a conjunctival granuloma in the inferior fornix. The lesion was excised and histopathologically examined.Microscopy revealed synthetic fibers embedded in the cornea and in the conjunctival granuloma. The diagnosis was confirmed by demonstration of marked birefringence of the synthetic fibers. Microscopical examination of synthetic fibers from two different types of fur (whiskers and face hairs) from the two-year-old girl's teddy bear was performed. Hairs from the face of the teddy bear were morphologically and microscopically identical with the fibers causing the severe corneal ulceration in the two-year-old girl.Doctors should especially in small children be aware of the risk of ocular consequences of close exposure of synthetic fibers from stuffed toy animals. Corneal ulceration, clinically presenting as corneal infection with negative culturing and staining, should lead to a different clinical strategy and treatment. The treatment of conjunctival synthetic fiber granuloma is excision and antibiotic eye drops. A close exposure of the eyes of stuffed toy animals and blankets made of synthetic material may cause penetration of the conjunctiva or the cornea by synthetic fibers ,2. UsualFifteen cases of conjunctival teddy bear granuloma have been published , but so We report a unique case of keratitis and a case of conjunctival granuloma caused by synthetic fibers from toy teddy bears.A two-year-old girl was referred by her general practitioner. Three weeks previously the patient presented with ocular irritation and corneal opacities of her left eye. Topical treatment with ciprofloxacin and chloramphenicol had no improvement of the condition. According to the mother there had been no trauma or foreign body complaints in the patient. The girl was born with oesophageal atresia, and had mild asthma. Apart from this she was in a healthy medical condition.Examination in general anaesthesia revealed a 4 × 5 mm oval ulceration infero-centrally of the left cornea with purulent exudate, stromal oedema and a couple of large "mutton fat" precipitates Figure . No foreFor the next two weeks there was a remarkable improvement of the corneal ulceration. However, suddenly a relapse with a new hypopyon and worsening of the corneal ulceration occurred. The treatment was supplemented with fortified gentamicin and fortified cefuroxin eye drops every hour. One week later the results of the staining and the cultures were proved negative for bacteria and fungi. The condition worsened and a spontaneous perforation occurred centrally in the ulceration. Furthermore, a new but smaller ulceration was observed more laterally. Corneal transplantation was performed and the corneal button was sent for microscopical examination.In addition, microscopical examination was performed on synthetic fibers from two different types of fur (whiskers and hairs from the face) from the child's toy teddy bear.A five-year-old girl was admitted to the eye department because of irritation and foreign body sensation in her left eye for two days. She was otherwise in a good general health.Slit lamp examination showed a 7 × 5 mm granuloma in the left inferior conjunctival fornix embedded with hairs and synthetic fibers. The lesion prolapsed easily with gentle pressure on the left lower lid. The examination was otherwise normal.The lesion was excised under general anaesthesia and sent for microscopical examination. Postoperatively, the eye was treated with chloramphenicol three times daily for five days. The wound healed within two weeks and the postoperative course was uneventful.The specimens were fixed in 4% buffered formaldehyde and embedded in paraffin. Sections were cut at 4 μm and mounted on glass slides.2 and Gordon & Sweets' method for reticular fibers.Deparaffinized sections were stained with haematoxylin-eosin (HE), periodic acid-Schiff (PAS), colloidal iron (CI), Alcian blue at 1.0 and 0.2 mol MgClThe light microscopy showed a heavily inflamed and ulcerated cornea Figure . Bowman'To verify whether the synthetic fibers belonged to a toy teddy bear, we performed microscopical examination of two different types of synthetic fibers (whiskers and face hair) from the girl's toy teddy bear. Hairs from the face of the teddy bear were morphologically and microscopically identical with the fibers causing the severe corneal ulceration in the two-year-old girl.Investigation of the teddy bear fur demonstrated that the fiber was made of 100% polyethylene . The azo-dye , applied to the fibers is not restricted by EU and is not known to cause inflammation.Microscopical examination of the conjunctiva revealed a normal differentiated conjunctival epithelium with goblet cells Figure . The subIncreased tear flow, redness and foreign body sensation are the initial symptoms associated with foreign body in the eye, followed by pain, photophobia and ciliary injection. Signs of possible deeper ocular penetration include decreased vision and clouding of the cornea .The majority of patients with conjunctival granuloma (caused by synthetic foreign bodies) were referred to an eye department after the granuloma was visible . This maEven though the patient with the corneal lesion was examined with slit lamp, the examiner was unable to identify the corneal foreign body. A main reason might be that the synthetic fibers were bluish brown similar to the colour of the hypopyon and the iris. However, a slit lamp examination combined with negative bacterial and fungal staining and culturing should lead to a diagnosis of foreign body granuloma.Cases with tarantula hairs and caterpillar setae penetrating the cornea and giving rise to serious conditions like chronic kerato-conjunctivitis, intracorneal hairs with granuloma formation and chronic iritis are well documented -9. HowevIn a recent review of the literature, Schmack et al demonstrated the histopathological and ultrastructural features of conjunctival granuloma caused by synthetic fibers . The diaThe ocular surface epithelium not only acts as a physical and mechanical barrier against harmful substances, but also participates actively during allergic inflammatory processes . PatientSurgical removal of the conjunctival granuloma and postoperative treatment with antibiotics is recommended and has shown to be successful ,18. The The authors declare that they have no competing interests; there was no research funding and no proprietary interests in the materials described.MKF performed the literature review and drafted the manuscript. SH and JUP performed the microscopical examination of the cornea and the conjunctival granuloma and assisted in the final preparation and submission of the manuscript. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2415/11/17/prepub


Objective. Krukenberg tumors in pregnancy are very rare and their management can present a dilemma for the obstetrician gynecologist. Case Report. We present the case of a G3P2002 who presented to us and the 38 weeks gestation with bilateral massive Krukenberg tumors. Despite at surgery and chemotherapy she died 3 months postpartum. Conclusion. Early detection followed by surgery and chemotherapy could possibly result in a favorable outcome with such patients.  The presence of adnexal masses during pregnancy ranges from 1 to 81 to 1 in 2,500 pregnancies, but only 3% of these masses are malignant. Krukenberg tumor is an ovarian metastasis of a gastric tumor and accounts for 1%—2% of all ovarian tumors [μg/L, normal range: <0.62 μg/L), progesterone , and 17-OH-progesterone , and the tumor markers levels were 43.4 U/mL (CA 19–9), 1.7 ng/mL (carcinoembrionic antigen) 12.8 U/mL (CA72–4) and 33.9 U/mL (CA 125), respectively. A 30-year-old woman, gravida 3 para 2, from a very remote community was referred at 38 weeks to our tertiary care center due to the presence a bilateral ovarian masses and shortness of breath. The patient complained of history of 1 year before the pregnancy with mild bloating and early satiety that was attributed to gastritis and gastroesophageal reflux, but despite the symptoms she never saught for medical attention. A pelvic sonogram suggested that the ovarian tumors were malignant. Decision to proceed with exploratory laparotomy and cesarean section was taken. Circulating levels were as follows: testosterone  and oxaliplatin (50 mg/m2) in a weekly schedule. Despite all our efforts the patient succumbed to disease in 3 months.In the postoperative period the patient was started on chemotherapy with 6 cycles of chemotherapy using 5-fluorouracil  the presence of a tumor in the ovary, 2) evidence of intracellular mucin secretion by the formation of signet cells, and (3) diffuse infiltration of stroma giving a sarcoma-like appearance [ evidenceA search in English, Spanish, and French literature revealed four reports of bilateral ovarian Krukenberg tumors, with our case being the fifth in the literature. The cornerstone of the management of these tumors is the diagnosis of the gastrointestinal primary tumor, and also the prognosis worsens when the primary tumor is identified after the metastasis to the ovary is discovered . Due to the rare nature of these tumors, there is no current standardization for the diagnosis and the treatment. Also due to the rarity of the condition it is not appropriate to comment on the effect of tumor on pregnancy and vice versa. There are only 3 cases of pregnancy-related Krukenberg the tumors in the literature; of these also 2 were detected postpartum, and this is the only tumor that is not associated to virilization. Some authors are of the opinion that there might be an increased risk of acute abdomen secondary to tumor torsion or rupture . The role of debulking surgery and chemotherapy with platinum-based chemotherapy can be reasonable, and even relatively safe to be administered during pregnancy. But despite the interventions, usually the discovery of the presence of masses of the size that our patient exhibited the prognosis is poor as it generally represents an advanced stage disease, However, possible early detection with debulking surgery, possible hysterectomy with/without delivery, and platinum-based chemotherapy may improve the survival of these patients , 6.


Background. In 1897, Dieulafoy was the first to characterize a gaping arteriole within the gastric mucosa causing massive hematemesis, designating it as “exulceratio simplex.” A hundred years later, this vascular abnormality, now commonly referred to as a “Dieulafoy lesion,” has been identified through the entire gastrointestinal tract and the bronchus. Objectives and Methods. As the original findings have been subjected to revisions and modifications by modern authors, Dieulafoy's original paper was reviewed and analyzed. The evolution of the current usage of “Dieulafoy's lesion” in the literature has been summarized with comparisons to the original report. Additionally, an index case of a 10-year-old female with a gastric “exulceratio simplex” is reported with a review of previously reported paediatric Dieulafoy lesions. Conclusions. The term “Dieulafoy lesion” in modern literature no longer adheres to the initial conclusions with regards to its origin, demographics, and presenting symptoms. Dieulafoy lesions remain a rare cause of gastrointestinal bleeding that can cause life-threatening haemorrhages in children. Paul Georges Dieulafoy 1839–1911), a professor of pathology at the Faculty of Medicine in Paris, France, was the first to describe a series of 10 patients who presented with massive hematemesis (approximately 4 L of blood in less than 24 hours) due to a bleeding gastric vessel, without any evidence of ulceration in the first three lessons of the 1897-1898 edition of “Clinique Médicale de l‘Hôtel-Dieu.” At autopsy, a superficial ulceration with a gaping arteriole was found in the gastric submucosa. The lesion's borders were not hardened or projecting, and the remainder of the mucosa was in perfect health. Dieulafoy concluded that this lesion was not a typical gastric ulcer and named it an “exulceratio simplex,” which in time became known as a “Dieulafoy lesion” [839–1911,Modern literature has broadened the original definition of a “Dieulafoy lesion.” Initially only described in the stomach, such findings have been additionally reported in the rest of the gastrointestinal tract such as the esophagus , duodenuDieulafoy's original treatise published in French was reviewed, analyzed, and translated into English for discussion as we suspected that the original findings have been subjected to revision with modification by many current authors. Using an index case of a 10-year-old female who presented with recurrent upper gastrointestinal bleeding that necessitated a partial gastrectomy, the pathological abnormality of the vascular anomaly in the submucosa—“Dieulafoy lesion”—is analyzed from a historical viewpoint of “then and now.” All published paediatric cases of Dieulafoy lesions as available in PubMed and Medline have also been retrieved and analyzed providing a comprehensive review of paediatric Dieulafoy lesions.We obtained a copy of the original first three lessons of “Clinique Médicale de l‘Hôtel-Dieu” treatise written in 1897-1898 by Paul Georges Dieulafoy. The original French paper was read and analyzed in its native language and translated into English for detailed review and study.The definition and usage of a “Dieulafoy lesion” in modern literature was explored. The most widely recognized definition of a “Dieulafoy Lesion” was retrieved from Wikipedia, an extensively accessed free online encyclopaedia. We then researched the definition, epidemiology/aetiology, clinical presentation, detection, and treatment of this lesion using Google Scholar, PubMed, and Medline. Finally, a search of PubMed and Medline limited to the paediatric population (ages 0–18) was conducted in the English and French languages. All references were read, reviewed, and analyzed.A 10-year-old female presented to the Emergency Room with melena, hematemesis, and generalized abdominal pain. At presentation, her haemoglobin was 6.5 g/dL with a blood pressure of 99/64. Gastroscopy identified four polypoid lesions on the lesser curvature surrounded by an erythematous and friable mucosa that was oozing blood. Multiple mucosal biopsies showed no significant pathological abnormality. Imaging including an MRI showed no significant findings, and a repeat gastroscopy was unremarkable. She was discharged home on prevacid.A year and half later, she was admitted to the paediatric intensive care unit (PICU) after vomiting approximately 1 L of bright red blood preceded by a one-day history of general malaise and fatigue. She was hypotensive at presentation, with a systolic pressure of 70 mmHg and haemoglobin of 7.5 g/dL. She was stabilized by transfusion with fluids and two units of packed red blood cells (PRBCs). The abdominal ultrasound was normal. A repeat gastroscopy rediscovered the 4-5 cm region of erythematous and friable gastric mucosa and raised the possibility of an underlying vascular abnormality. A second repeat MRI revealed diffuse thickening of the greater curvature with several enlarged mesenteric lymph nodes; the largest measured 5-6 mm.On laparoscopic examination, prominent, tortuous vessels were seen on the lateral aspect of the greater curvature and extensively on the posterior wall of the stomach. The greater omentum inhibited a full visualization, and it was decided to carry out an exploratory laparotomy to determine the full extent of this vascular abnormality. At intraoperative examination, mild handling of the stomach caused extensive bleeding, which required vascular clamps and two units of PRBCs to maintain haemostasis. No “normal” gastric wall was visible on the posterior aspect  resultinHistopathological examination of the specimen showed a hemorrhagic area measuring 3.3 × 2.2 cm with a central 0.4 × 0.4 cm vascular polyp. On microscopic examination, a large dilated tortuous vessel with evidence of fibrin thrombi and recent haemorrhage through the overlying mucosa was identified Figures . This suPostoperative recovery was uneventful. As she had no further complaints at her followup at the outpatient clinic 6 weeks later, her care has now been transferred to her family physician.All information provided in this paper has been completely deidentified and is thus exempted from a formal review by the Ethics Committee as per our institutional policy.sur l'une des petites érosions cratériformes, due à la disparition de la muscularis mucosae, apparaît làrtériole béante” . He concludes the three lessons with eight major learning points about the “exulceratio simplex” (pages 42-43) [Though in 1884 Gallard wrote the first description of a patient with a “Dieulafoy's lesion” , this ep“à l'estomac, une perte de substance très superficielle et assez étendue” ;the exulceratio simplex is “très superficielle et ne dépasse pas en profondeur la tunique muqueuse” ;the “exulceratio simplex” is oval, elliptical, or star shaped and has the dimensions of a 50-centime piece, of 1 franc or 2 francs. It is n'étant ni indurés ni surelevés”  and the gastric walls maintain their softness;edges of the exulceratio “est un processus ulcéreux aigu”  that reaches a voluminous and superficial submucosal arteriole;the exulceratio ““silencieusement, sournoisement”  in a healthy patient who may develop nausea, vertigo, syncope, and large haematemeses;rarely the exulceratio simplex presents with gastric symptoms, but more often it evolves “acquérir la certitude que la lesion … siège bien à l'estomac” ;to diagnose exulceratio simplex, the physician must identify a “true” haematemesis and then the exulceratio simplex is extremely serious due primarily to the substantial haematemeses;the future risk of recurrence remains unknown.siège bien à lèstomac”  [l'exulceratio à une predilection pour les jeunes gens”  [In the current literature, alternative names, descriptions, and criteria for Dieulafoy lesions include “caliber-persistent artery,” “gastric aneurysm,” “gastric arteriosclerosis,” “submucosal arteriole malformation,” and “cirsoid aneurysm” , 12. Thepage 43)  and the page 43) . This co“les hémorragies stomacales dues à léxulceratio simplex sont … pas annoncées par de petits vomissements de sang”  [ressemblent à des gens bien portants”  [Dieulafoy lesions classically present as intermittent and massive GI haemorrhage with symptoms associated with this blood loss. 44% of patients report melena, 30% haematemesis, 18% with both hematemesis and melena, 6% with haematochezia, and 1% with iron-deficiency anemia  with thepage 32) . Intereselle passerait assez facilement inaperçue sans un examen attentive et sans l'idée préconcue qu'on va la trouver”  [Though over a hundred years have passed since Dieulafoy first described this lesion, the mechanisms causing the tortuosity and the persistence of the large-sized submucosal arteries remain unknown. Many reports have attributed this lesion to the pathological structure or the size of the artery with a persistent musculoelastic mantle of Wanke. Debate continues regarding the exact pathology and linkage of this artery to the overlying mucosa . Furtherpage 24) . The thepage 24) . The thrpage 24) , 17, (b)The advent of endoscopy has marked a drastic change in the diagnostic evaluation and therapeutic management of Dieulafoy lesions. Up to 71% of initial endoscopies are diagnostic only if there is active haemorrhage of at least 0.5 mL/min . HoweverShould endoscopy fail to determine a source of bleeding, angiography and capsule endoscopy may provide useful information. On an angiogram, extravasation of contrast into the GI system from an eroded artery is indicative of this lesion, as is the presence of tortuous ectatic vessels in the arterial phase without early venous return thereby distinguishing this unique lesion from arteriovenous malformation . This diSince 1986, endoscopy in addition to being the primary diagnostic tool has also become the first-line method of treatment . TraditiDieulafoy lesions as a cause of massive gastrointestinal bleeding are uncommon occurrences in the paediatric population. The most common cause of massive GI bleeding in children is usually related to oesophageal varices secondary to portal hypertension and chronic liver disease , 30. TheAs listed in Unlike Dieulafoy lesions in the adult population, there is no clear preponderance of these lesions in pediatric males. Such strong male predominance in adults is perhaps hormonally related and can be attributed to the lack of estrogenic protective effects in males. It is also postulated that complex hormonal dysfunctions at the submucosal arteriole arcade are involved in the pathogenesis of the caliber-persistent artery as described by Dieulafoy's illustrations on pages 15 and 16 [The location of Dieulafoy lesions reported in children includes ten in the stomach, five in the jejunum, five in the ileum, and two in each the duodenum, and rectum and one in each of the sigmoid, anorectal junction, and anal canal. Twelve children (46%) were treated surgically, a much higher percentage than in the adult population already described, while 14 were managed endoscopically by injection therapy, band ligation, and thermocoagulation . RecentlThe usage of the term “Dieulafoy lesion” in the current literature no longer fits within the confines of the conclusions drawn by its namesake over a hundred years ago. Dissidents contest with Dieulafoy's original theory that the “exulceratio simplex” though of congenital origin represents an acute process. Other digressions now include an aging population, nongastric and extraintestinal locations, and its recognition in children as well as in adults. Though massive haematemesis—“gastrorrhagias”—was the sine qua non of the “original” Dieulafoy lesion, the inclusion of nongastric  and extraintestinal (bronchus) sites of this anomaly has completely altered the clinical presentation. Additionally, endoscopic vascular criteria have replaced the diagnosis of this lesion in these widespread locations.Nevertheless, the name “Dieulafoy lesion” is entrenched in the literature and contains historical significance; therefore, we suggest that this eponym may still be used with the understanding that the diversity of the lesion it describes no longer conforms to the original definition of the term. Dieulafoy lesions remain a rare and most dangerous form of gastrointestinal bleeding that can result in life-threatening haemorrhages in children.


Hemangioendothelioma is a rare vascular tumor of intermediate malignant potential. Though epithelioid hemangioendothelioma (EH) is commonly found in soft tissues, it has been known to be present in skeletal tissues. The authors present a case of a 50-year-old woman diagnosed with EH of the iliac bone and acetabulum, who experienced pathological fracture at presentation. This report describes a multidisciplinary approach to the management that includes initial incisional biopsy, curettage, and bone grafting, followed by Intensity Modulated Radiation Therapy. The patient finally underwent hemipelvic resection with allograft reconstruction after recurrence. Histopathological study revealed osseous EH of low mitotic activity that stained positively for CD31, CD34, vimentin, and Factor VIII. Herein, the authors discuss the imaging characteristics, histopathological aspects, cytogenetic findings, and the radiobiological behavior of osseous EH. After an aggressive multidisciplinary intervention, the patient is able to achieve local control with no evidence of distal metastatic disease. Hemangioendothelioma is a rare tumor that exhibits intermediate malignant potential, clinically behaving between benign hemangioma and malignant angiosarcoma. Epithelioid hemangioendothelioma (EH) is the most common histologic subtype that arises from vascular tissue  and repr50-year-old African American woman initially presented to the emergency department with a two-week history of vague right hip and lower pelvic pain. Her past medical history was significant for rheumatoid arthritis, diabetes mellitus type 1, and hyperthyroidism status after radioactive iodine ablation. No history of trauma or systemic etiology was found. Physical exam was unremarkable for motor or sensory neurological deficits. No functional impairment was detected. CT of abdomen and pelvis obtained at an outside facility revealed an enhancing mass identified within the medullary space of the right iliac bone which extends from the level of sacroiliac joint to the roof of right acetabulum . PatientPostoperative MRI of the pelvis  noted miThe initial biopsy revealed focal fibrosis and marrow that are slightly hypercellular with mild increase in plasma cells that are mostly scattered and comprise approximately 10% of the marrow cells. Immunohistochemical stains for CD 138, kappa, and lambda react positively, suggesting that they are polyclonal. Therefore, no clear evidence for plasma cell neoplasm is found. CD31 and CD34 immunostains marked for endothelial cells show focal vascular hyperplasia.The curettage specimen consists of multiple fragments of soft tissue and bone measuring 5.5 × 5.5 × 0.8 cm in aggregate. Histological section reveals proliferation of well-formed thick walled vessels, anastomosing vascular channels lined by epithelioid cells in hyalinized and inflamed stroma. Neoplastic cells show moderate nuclear pleomorphism, abundant eosinophilic cytoplasm, and intracytoplasmic lumen formation . MitoticGiven the extent of the disease involvement revealed at the time of curettage, an upfront complete resection with negative margins may result in morbidity and high probability of debilitation. Hence, it was the decision of the multidisciplinary tumor board that the patient undergoes upfront radiation therapy to reduce the tumor size and the extent of disease to facilitate surgical resection. She underwent CT simulation using vac loc bag to maintain daily treatment reproducibility prior to the plan generation. In order to reduce the potential dose to surrounding normal tissues such as ovary, bowel, bladder, rectum, and femoral heads, a five-field Intensity Modulated Radiation Therapy (IMRT) plan was utilized . MRI fusAt approximately one year after completion of therapies, the patient remains free of malignancy.Epithelioid hemangioendothelioma falls under the broad histological category that also includes papillary intralymphatic angioendothelioma, spindle cell, retiform, and composite hemangioendothelioma. Historically, EH has shown similarities to histiocytoid hemangioma, cellular hemangioma, low grade anaplastic sarcoma, and angioendothelioma. EH has been correlated with translocation of 1; 3)  and   as well as deletions in chromosomes 11 and 12 [  which represents the ratio of the rate of cell loss to the rate of new cell production or proliferation as exhibited by the equation φ = 1 − Tpot/Td, where Tpot is the potential tumor doubling time and Td is the actual tumor doubling time [G2/M cell cycle arrest in preparation for mitotic catastrophe and apoptosis, results in greater percentage of cell kill in carcinoma than sarcoma or endothelioma [Outcome is most favorable for low grade lesions when complete excision can be achieved. However, treatments range from simple curettage to en bloc resection to radical resection of the tumor and surrounding structures or organs . In the ing time . The celing time –21. On ting time , 23. Thethelioma . As a reOsseous EH involving the femur and ilium is rare and can be difficult to diagnose from the clinical and histopathological perspective. Depending on the anatomical location of the primary tumor, local control and cure may be difficult to achieve with a single modality of therapy. As such, a multidisciplinary team of radiologists, pathologists, surgeons, medical oncologists, and radiation oncologists are required to generate a plan of care that produce the highest chance for cure and the lowest morbidity. Given the rarity of this disease, further studies are needed to establish guidelines and elucidate the best sequence of therapeutic interventions. This case highlights a multidisciplinary collaborative approach to the management of osseous EH in a difficult anatomical location to be adequately addressed by a single modality. In this case the patient is able to achieve cure and local control.


Cardiac PEComa is very rare. We reported two cases of epithelioid PEComas, one in an adult and one in a 2-year-old child. Both tumors were composed of sheets of epithelioid cells with coagulation necrosis. In addition, the adult case showed marked nuclear atypia and high mitotic activity with atypical mitosis and the pediatric case showed unusual clear cell features. Immunohistochemically, both tumors were positive for HMB-45 and SMA and negative for S100 and cytokeratin. Electron microscopy was performed in the pediatric case and showed premelanosomes. The adult patient developed extensive metastasis indicating malignant behavior. Prior to the two cases, only 5 other cases of cardiac PEComa were reported and the literatures are reviewed. PEComa was first proposed by Bonetti et al.  and is aThe specimen was fixed in a 10% neutral-buffered formalin solution and processed as a routine surgical pathology specimen. Representative sections were embedded in paraffin, processed in the usual manner, and stained with hematoxylin-eosin.Immunohistochemical stains were performed on formalin-fixed, paraffin-embedded 4 um tissue sections with the avidin-biotin immunoperoxidase complex method  with diaminobenzidine as the chromogen and hematoxylin as the nuclear counterstain. Information about the antibodies used was summarized in A 2-year-old girl presented with dyspnea and poor sleep for 10 days. Physical examination showed that the patient had cyanosis around her lips, decreased movements, edema of lower extremities, and hypourocrinia. The patient had no fever and her growth and development were normal. Cardiovascular system examination revealed lower heart sounds with irregular rate (heart rate 200 times/minute), and no heart murmur was identified. The examinations of hemogram, urine, and blood biochemical analyses were within normal ranges.Echocardiography showed irregular-shaped occupying lesions measured 5.4 cm × 3.7 cm in posterior of atrial septum (mainly in left atrium), left heart dilatation with left ventricular systolic dysfunction, medium tricuspid regurgitation, and mild mitral regurgitation. The clinical impression was most compatible with a left atrial myxoma. The patient underwent thoracotomy to excise the mass. At surgery, a globular solid mass was seen arising from the posterior aspect of left atrium. The lesion pushed pulmonary vein to the anterior and compressed inferior vena cava and infiltrated into coronary vein sinus. The enlargement of the right atrium and the right ventricle were observed and the pericardium was normal. Eight days after surgery, ultrasound showed that the patient had massive pericardial effusion. No adjuvant chemotherapy was performed for the patient. Postoperative evaluation did not reveal stigmata or family history of tuberous sclerosis complex (TSC). The patient was discharged after recovered from pericardial effusion. The patient was regularly followed up for 32 months and was doing well.The patient was a 33-year-old African American female with no family history of tuberous complex. She first presented with left atrial mass and that was resected. 8 months later, she underwent a more extensive excision of recurrence and radiation therapy. 2 years later, the patient was found to have a large retroperitoneal mass involving the liver with extension into the inferior vena cava to right atrium. The mass also encased the right kidney. The patient underwent an en bloc resection. The excised globular mass measured 6.0 cm × 4.4 cm × 3.5 cm and had a firm and friable consistency , and wasThe patient underwent en bloc resection for the retroperitoneal metastasis. The specimen measured 25.0 × 22.5 × 19.0 cm, was tan-white and included portion of right liver, right kidney, and inferior vena cava. A well-circumscribed subcapsular mass in the liver, measuring 5.5 × 4.5 × 4.5 cm was identified in the resected portion of liver. Pathological examination showed that the tumor encasing and invading into inferior vena cava, invasive into the liver and impinged upon but did not invade the kidney. The patient was alive with disease at 33-month status postinitial cardiac surgery.The histologic features for the two cases were summarized in Both of the tumors showed extensive positivity for HMB45 , focal tPEComa is a tumor composed of epithelioid cells with clear and acidophilic cytoplasm and having a perivascular distribution. PEComas can occur in a wide variety of sites such as kidney, lung, liver, pancreas, common bile duct, jejunum, rectum, falciform ligament of the liver, urinary bladder, prostate, breast, bone, soft tissue, and female genital tract organs. PEComas occurring in kidney are also termed angiomyolipoma (AML). One rare variant of AML is epithelioid AML and is defined as tumor composed of predominantly epithelioid cells by World Health Organization . Here weThe cardiac PEComas are very rare. To date, only 5 cases of cardiac PEComas were reported , 14–17. The main differential diagnosis for cardiac PEComas is cardiac myxoma clinically and rhabdomyoma histologically, especially in children. Rhabdomyomas are the most common pediatric heart tumors and considered as hamartomas with no malignant potential. Cardiac rhabdomyoma is highly associated with tuberous sclerosis complex (TSC). Morphologically, cardiac rhabdomyomas are well demarcated nodules of enlarged cardiac myocytes with cleared cytoplasm. In some cells, strands of eosinophilic cytoplasm stretch from a central nucleus to the cell membrane, giving rise to cells that resemble a spider (“spider cell”). The majority of cells show vacuolization with sparse myofilaments. There is a strong reaction with periodic acid-Schiff reagent, reflecting the glycogen content of rhabdomyoma cells. Immunohistochemical studies show the striated muscle characteristics of rhabdomyoma cells, which express myoglobin, desmin, and actin. Moreover, it has been reported that aberrant HMB45 expression was detected in cardiac rhabdomyomas . NeverthBecause of their rarity, optimal treatment for PEComas is not determined. Surgical resection represents the only curative approach for primary PEComa as well as for local recurrences and metastasis, as chemotherapy and radiotherapy have not demonstrated significant benefits , 23.In summary, we have documented herein two cases of epithelioid PEComas in the heart, one of which developed extensive metastasis. Cardiac PEComa tends to occur in young adult and pediatric patients with slightly predilection to the left atrium and female gender. Necrosis is common features in cardiac PEComa and presence of nuclear atypia and high mitotic activity could be associated with aggressive behaviors. Additional cases and longer followup are needed to gain more insight into tumor biologic behavior in those cases lacking marked nuclear atypia and high mitotic activity.


Dermoid cysts of the head and neck are rare congenital benign tumors. According to the literature they represent about seven percent of all dermoids and less than one percent of all intracranial neoplasms. Extradural dermoid cysts are very rare. We report a case of intracranial extradural dermoid cyst of mastoid bone. We believe that this is the second documented extradural dermoid cyst, the first case reported in the literature  was in close relation to the petrous apex but ours is in close relation to mastoid antrum. Hearing loss was the only clinical presentation in this case, while neurological symptoms were the main presenting symptoms in the first reported case. We present our management of this rare case with respect to the clinical, radiological, histopathological, and surgical aspects and conclude that dermoid tumors, though rare, need to be included in differential diagnosis of middle ear lesions. Dermoid tumors are not true neoplasms but are inclusion cysts composed of ectodermal elements. They are uncommon lesions, accounting for approximately 0.3% of all brain tumors and about seven percent of all dermoids of the head and neck .Dermoid cysts are derived from both the ectodermal and mesodermal elements. A keratinizing squamous epithelium is typically present along with dermal derivatives including hair follicles, smooth muscle, and apocrine and sebaceous glands. Fibroadipose tissue is also present. The exact etiology of these neoplasms is unknown though the most likely theories are incomplete closure at lines of fusion or traumatic implantation of skin elements . We report a case of intracranial extradural dermoid cyst of mastoid bone, with respect to the clinical, radiological, histopathological, and surgical aspects.An 18-years-old girl diagnosed at the age of three years to have right otitis media with effusion (OME) was treated medically. She presented again at the age of 8 years with the same complaint but did not respond to the medical treatment and underwent right myringotomy with ventilation tube and adenoidectomy. Two years later, her symptoms recurred and underwent right T-tube insertion. Symptoms had recurred again after removal of the T-tube 2 years later.She presented at the age of 18 years with right hearing loss and intermittent nonpulsatile tinnitus. There was no history of otorrhoea, otalgia, or vertigo, and there were no abnormal neurological signs or symptoms.Physical examination revealed retracted right tympanic membrane, while the left was normal. Audiogram showed right moderate conductive hearing loss (CHL)  with shaComputed tomography (CT) of the temporal bone  showed rMagnetic resonance imaging (MRI) of the temporal bone and brain with and without gadolinium  revealedThe patient underwent a combined approach: right temporal craniotomy with middle cranial fossa approach to excise this mass by the neurosurgeon, associated with canal wall down mastoidectomy by the otologist, which was necessary to eradicate the whole mass. The mass was encapsulated and adherent to the dura of the middle cranial fossa. Incision of the mass produced yellowish cheesy content with hair. A whitish mass was occupying the antrum and mastoid air cells. No incus or stapes suprastructure was seen, and the proper middle ear cavity was intact. The patient tolerated the procedure well and recovered with no complications.Histopathological report  showed dCongenital dermoids are benign developmental anomalies rather than true neoplasms. They originate during early embryogenesis and are derived from both ectodermal and mesodermal elements (4). The exact etiology of these lesions is unknown, though the most likely theories are incomplete closure at lines of fusion or traumatic implantation of skin elements .Dermoid cysts of head and neck are rare. They represent about 7% of all dermoids , 4 and lTwenty-four cases of dermoids of the temporal bone were reported in the English literature . MultiplGrossly, dermoids are usually polypoid, pedunculated and rarely sessile masses. They are grayish white or pink in color, covered by skin often containing hair. Microscopically the surface epitheliod layer is of stratified squamous epithelium that contains epidermal appendages. The stroma is fibrofatty material and may contain smooth and stratified muscle, cartilage, bone, minor salivary glands, nerves, and lymph nodes.Clinical presentation varies and usually depends on the location of the tumor. Dermoid tumors of the middle ear may present as unresolving serous otitis media or recurrent otitis media as in our case. This could be caused by the obstruction of the Eustachian tube by the dermoid. Negative pressure is created within the tympanic cavity and in mastoid air cells associated with low oxygen tension resulting in alteration in middle ear mucosa with an increase in mucus secreting cells.The present case is unique because it represents extradural, intracranial dermoid tumor of mastoid antrum and was associated with well-defined osseous erosion. We believe that our case is the second documented case of extradural dermoid cyst. The first one was adjacent to the petrous apex .Dermoid cysts appear hypodense on CT scan as a result of high lipid content. On MRI imaging dermoid tumors are hyperintense on T1-weighted sequences and are variable from hypo- to hyperintense on T2-weighted sequences due to their high fat content. Differential diagnosis of these tumors includes teratoma, temporal lobe meningocele herniating into mastoid antrum, meningiomas, lipoma, and congenital cholesteatoma.The recommended treatment is complete surgical excision, and this can be achieved safely by the cooperation of multidisciplinary teams as in our case, the dermoid was totally excised without complications. These tumors have a limited growth potential and when completely removed they do not recur.The prognosis of dermoids in the head and neck region is favorable. Malignant transformation in a long-standing dermoid cyst is a rare complication. Tsugu et al.  reportedThe authors describe an unusual case of an extradural dermoid tumor within the mastoid antrum, and middle cranial fossa who presented with hearing loss proceeded by unresolving serous otitis media. The tumor was successfully and totally removed. Distinction from congenital cholesteatoma is impossible pre-operatively.


Pericardial effusion as a complication of malignant gynecological disorders is rare. Few cases of endometrial cancer, squamous cell carcinoma of the cervix, ovarian cancer and uterine carcinosarcoma have been previously reported. We report the first case of cardiac tamponade secondary to a cervical adenocarcinoma.A 54-year-old Caucasian woman, without any relevant medical history and no gynecological aftercare, was admitted to our hospital emergency room with severe dyspnea. Echocardiography revealed severe pericardial effusion with a swinging heart. An emergency pericardial drainage was performed through a pericardial window, which permitted the draining of 700 mL of bloody fluid and a pericardial biopsy. Cytological examination of the fluid revealed atypical cells, and the biopsy specimen showed tumor emboli suggestive of adenocarcinoma. Magnetic resonance imaging showed a 35 mm cervical lesion indicative of an endocervical tumor. Exploratory laparoscopy revealed diffuse peritoneal lesions and histological examination of cervical curettage showed a poorly differentiated micropapillary adenocarcinoma of the cervix.Carcinomatous pericarditis as the first symptom of a malignant gynecological adenocarcinoma has not, to the best of our knowledge, been documented before. This case highlights the extreme severity of pericardial effusion secondary to cervical adenocarcinoma, a sign of advanced disease. Gynecological malignancies have to be considered in cases of neoplastic pericardial effusion. Cardiac tamponade as a complication of a malignant gynecological disorder is a very rare occurrence. Very few cases of malignant pericardial effusion due to endometrial cancer -4, squamA 54-year-old Caucasian woman, without any relevant medical history and no gynecological aftercare, was admitted to our hospital emergency room with dyspnea that had progressed over 10 days and was worsening. Upon admission, we noted blood oxygen saturation of 89%, tachycardia (125 beats per minute) and hyperthermia (38.9°C). Her blood pressure was 120/80 mmHg. Electrocardiography showed sinus tachycardia and echocardiography revealed severe pericardial effusion with a swinging heart. An emergency subxiphoid incision with pericardial drainage was performed through a pericardial window, which permitted draining of 700 mL of bloody fluid and a pericardial biopsy. Cytological examination of the fluid revealed atypical cells, and the biopsy specimen showed tumor emboli suggestive of adenocarcinoma. Immunostaining indicated elevated levels of tumor marker, cytokeratin 7 (CK7). Her blood concentrations of carbohydrate antigen (CA) 125 and CA 15.3 were 4667 IU/L and 451 IU/L, respectively, suggesting ovarian malignancy.When stabilized, our patient was transferred to our department for further investigations. A gynecological examination only found a large uterus. A physical examination of her cervix was normal. An ultrasound examination showed uterine myomatosis, but no endometrial abnormality. Her ovaries were not seen. Magnetic resonance imaging (MRI) showed a 35 mm cervical lesion indicative of an endocervical tumor Figure . A thoraet al. [Malignant neoplasia from any organ can potentially metastasize to the pericardium , but theet al. , a totalet al. , and is When symptomatic, pericardial metastases tend to occur as secondary manifestations of the cancer ,3,6,11. Where medium-term follow-up has been reported, the outcome of patients with endometrium adenocarcinoma with pericardial effusion was progression to death in two of the four reported cases ,3. In thAccording to our findings and previous reports, cardiac tamponade can be considered as indicative of a very poor prognosis when it complicates the progression of a cervical or endometrial adenocarcinoma.This case highlights the extreme severity of pericardial effusion secondary to cervical adenocarcinoma, a sign of advanced disease. Gynecological malignancies have to be considered in cases of neoplastic pericardial effusion.Written informed consent for publication could not be obtained from any immediate relatives despite all reasonable attempts. Every effort has been made to protect the identity of our patient and there is no reason to believe that any of her immediate relatives would object to publication.The authors declare that they have no competing interests.EA and MD wrote the manuscript. PF analyzed and interpreted the imaging of the patient and was a contributor in writing the manuscript. FW performed the histological examination and was a major contributor in writing the manuscript. DL critically reviewed the manuscript and provided significant help in writing. All authors have read and approved the final version of the manuscript.


Actinomycosis can involve all parts of the urogenital system. Urachal actinomycosis rarely reported and was mistaken with urachal adenocarcinoma. We report a case of urachal actinomycosis that presented with abdominal pain and underwent laparotomy with the diagnosis of urachal malignancy pathology reviewed the diagnosis of urachal actinomycosis. Patient had no problem in two years follow up. Actinomycosis is an infectious, chronic and granulomatous disease caused by gram-positive non-aerobic bacteria. These bacteria are te natural flora of mouth cavity especially teeth and tonsil, GI especially appendix and colon, and genital system in human that rarely causes disease. The prevalence of clinical actinomycosis is cervicofacial 50%, abdominal 20%, and thoracic 15%, respectively  was positive. He had no fever and the examination of other parts of his body was normal.Sonography showed the heterogeneous mass with size of 124 × 61 mm expanding from the umbilicus to the bladder . AbdominThe patient underwent cystoscopy due to suspected urachus adenocarcinoma. Urothelium was intact but mass pressure on bladder was observed. The patient underwent laparotomy with the incision of under umbilicus middle line. The mass had adherence to bladder and omentum and posterior rectus sheath which was removed with suitable margin along with partial cystectomy. The mass was solid, irregular, and brown. Bilateral lymphadenectomy was performed due to gross lymph nodes on iliac vessels.Microscopic evaluation of mass reported the actinomycosis without involvement of bladder urothelium  and 4. TActinomycosis is a chronic, indolent and recurrent disease which can involve all urogenital sections such as kidney, bladder, prostate, and testicle . AbdominUrachus actinomycosis is rarely reported and the patients usually refer with symptoms of tangible mass under umbilicus, abdominal pain, weight loss, appetite decrease, fever, and laboratory signs such as leukocytosis and anemia . Our patCorrect diagnosis before surgery is reported in only lower than 10% of cases. Abdominal actinomycosis may be mistaken with malignancy, bowel tuberculosis, crohn disease, diverticular and rectus sheath pathologies. Malignancy and abscess are in differential diagnosis . In our Imaging findings are non-specific in urachal actimycosis. Sonography determines the solid or cystic mass but may underestimate the expansion of inflammatory reaction. According to most reports, CT scan can better determine the status of mass than MRI (magnetic resonance imaging). The findings of PET/CT of actinomycosis are as intense hypermetabolism which is similar to malignancy , 12. LowIn a study about pelvic actinomycosis in 33 cases, cystoscopy was performed on 12 patients and the findings of cystoscopy reported the pressure effect of external mass on bladder, bullosis edema, and vegetative proliferation in one case showing chronic inflammatory changes . FurthurSince the clinical laboratory and radiologic signs are not special, in most cases, the diagnosis is determined after surgery . If the In most reported cases, the patients didn’t have any problem at long follow-up after surgery , 9; our