Urethral carcinoid tumors are very rare tumors with only four cases described in the literature.We present the case of a 61-year-old man with a primary carcinoid tumor of the verumontanum  portion of the prostatic urethra with a coexisting prostatic adenocarcinoma. In addition to whole mount hematoxylin and eosin staining, special immunoperoxidase staining specific for chromogranin A, neuron specific enolase, synaptophysin, pan-cytokeratin and PSA, and a special combined staining for racemase (α-methyl CoA) antigen and p63 antigen were performed. A review of the literature is included.A single focus of invasive prostatic adenocarcinoma was identified in the periphery of the mid-left, posterior quadrant of the prostate. Approximately 17 mm from this adenocarcinoma, within the verumontanum of the prostatic urethra, there was a 3 mm maximal dimension carcinoid tumor.Based on different histological features and antigenic profiles, we concluded that the two tumors were distinct. Carcinoid tumors of the urethra are exceedingly rare neoplasms, with only four cases reported in the literature -4. GivenWe report the first case of a primary carcinoid tumor within the verumontanum of the prostatic urethra in a 61 year-old man who underwent radical prostatectomy for adenocarcinoma of the prostate. We reviewed the literature for both primary carcinoid tumors of the urethra and for synchronous urethral carcinoid tumors and prostatic adenocarcinoma.Our patient is a 61-year-old Caucasian man who originally presented with urinary obstructive symptoms and a serum prostate specific antigen (PSA) level of 1.5 ng/mL six years before prostatectomy. The obstructive process was treated conservatively, with observation and symptomatics. Over the following three years, PSA of our patient continued to rise, prompting multiple sets of prostatic biopsies that were negative for carcinoma. Ultrasound showed a moderately enlarged nodular prostate consistent with benign prostatic hyperplasia. A few weeks before prostatectomy, his serum PSA increased up to 4.7 ng/mL, at which time additional core biopsies were performed. A single focus of Gleason grade 3+3 (score = 6) prostatic adenocarcinoma was identified, involving 5% of only one of the tissue cores submitted. Our patient was subject to undergo a radical prostatectomy. A conventional suprapubic, radical prostatectomy on our patient was performed. The prostate was submitted for whole-mount processing. Gross examination revealed a 32 gm prostate measuring 4.2 cm apex-base, 4 cm wide, and 3.5 cm antero-posterior. The attached seminal vesicles were up to 2.4 cm in length and 0.4 cm average diameter. Also submitted were dissections of the right and left pelvic lymph nodes. The prostate was fixed in formalin, serially sectioned in 5 mm intervals from apex to the base and paraffin embedded. Histological sections, 5 μm-thick, were cut from the anterior face of each paraffin block, mounted on 2 × 3-inch glass slides and stained with conventional hematoxylin and eosin. Immunoperoxidase staining specific for chromogranin A, neuron specific enolase, synaptophysin, pan-cytokeratin and PSA, and a special combined staining for racemase [α-methyl CoA] antigen and p63 antigen were performed.Digital microscopic pictures were obtained during different magnifications using an Olympus BX51 microscope  and a Macrofire digital camera  connected though a firewire to a Dell Optiplex 745 desktop computer . Low power images from whole mount slides were digitally scanned using an Aperio ScanScope Model T3  and captured using the ImageScope software version 9.x . All images were optimized using Adobe Photoshop CS2 software .A single focus of invasive, variably spaced, small atypical acinar glands without basal cells was identified in the periphery of the mid-left, posterior quadrant of the prostate, involving less than 5% of the prostatic tissue Figure . The cytApproximately 17 mm from the invasive adenocarcinoma, within the verumontanum of the prostatic urethra of our patient, there was a 3 mm maximal dimension tumor with markedly different morphology. From low microscopic power, the tumor of our patient appeared well circumscribed with pushing, rather than invasive, margins Figure . MitoticCarcinoid tumors of the urethra are exceedingly rare neoplasms. Using Pubmed and the search words "urethra" and "carcinoid" only four articles are found, each reporting a single additional case of a urethral carcinoid tumor -4. The fWhile concurrent prostatic adenocarcinoma and urethral carcinoid tumor have yet to be reported, concurrent prostatic adenocarcinoma and prostatic carcinoid tumors are rarely reported ,6. BecauOur case is unique in many ways. It is the first case of a patient with a primary carcinoid tumor in the prostatic urethra, and more specifically within the verumontanum. Secondly, it represents the oldest patient reported with a urethral carcinoid (61 years old). It also represents the first case of a concurrent urethral carcinoid with a primary adenocarcinoma of the prostate.PSA: Prostate specific antigen.Written informed consent was obtained from our patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.MS was the major contributor in writing the manuscript. MSL and PNW reviewed and discussed the manuscript. FGLR performed the original histological examination of the prostate, interpreted and diagnosed the pathology findings, prepared the figures and did the literature review. All authors read and approved the final manuscript.


The case of an atypical hepatic angiocavernoma is referred. The lesion, first described as a hypoechogenic area compared to the surrounding parenchyma, with anechogenic shoots inside, suggestive for vascular structures developed one year later into a totally asonic area with frayed margins. This change is very unusual and uncommon for this kind of lesions.The case of a 74-year old caucasian male, complaining of slight dyspeptic symptoms  is referred. The routine blood tests were all normal. Abdominal ultrasound showed a large, roughly round-shaped lesion (diameter 14 cm) in the VIII hepatic segment diagnosed as hepatic angiocavernoma, which turned unexpectedly in a cystic like lesion one year later.The atypical angioma's degeneration could account for one of the causes of the patient's exitus. It could be related to blood seizure by the large hepatic angioma due to the intratumoural haemorrhage. The angioma represents the liver's most frequent benign tumour, with an approximate incidence of 4% on population. Angiomas can hit any age bracket, but they are more frequent in adults, especially in women (ratio M/F = 1/4-6). Most of angiomas are small, asymptomatic and detected by chance. Large angiomas can appear as palpable abdominal masses, with pain at the right hypochondrium and a bloated loins feeling due to capsular pressure, haemorrhage or thrombus; those ones localised in the sub-capsular area are subject to easy breaking, with subsequent intraperitoneal haemorrhage; the thrombocytopenia caused by the seizure and destruction of platelets within the large cavernous angioma (syndrome of Kasabach-Merritt) is another complication ,3. AngioHistologically, the angioma constists of a vascular proliferation where the vessels are separated by a delicate fibrous stroma. It is possible to find thrombi within the vascular lacunae. The angioma is provided with a fibrous capsule, which at times can appear to be calcific .Its typical sonographic aspect is that of a small roundish area, with hyperechoic definite lips. Most angiomas have a diameter smaller than 3 cm. Circa 30% of angiomas show a hypoechoic, isoechoic or mixed structure, only seldom appearing clearly cystic .A 74 year-old caucasian male complained of light dyspeptic symptoms, characterized by post-prandial repletion and fullness. Moreover, the patient showed a light cardio-respiratory insufficiency. The routine blood parameters were all normal. Abdominal ultrasonography showed the presence of a large, roughly roundish formation, located in the VIII hepatic segment, 14 cm in diameter, basically hypoechoic compared to the surrounding parenchyma. It was chacterised by several anechoic shoots with a serpentine-like course, very suggestive of vascular structures , with non-definite and frayed lips, occupying a large portion of the right lobe (Figure In our case the observed mass was totally different from that of a typical cavernous haemangioma, characterised by a mixed, complex structure, whose solid component is intermingled with hypo-anechoic haematic lacunae. Such lacunae are commonly disomogeneous and with irregular margins, roughly roundish or polygonal , and notUltrasonographic examination performed one year later showed that the angioma had developed and changed its echostructure. The original lesion come out to have turned into a large anechoic formation with irregular and frayed lips.Many authors support that hemangiomas are congenital benign hamarthomas while others have suggested they might originate from hepatic areas of focal necrosis and regeneration. They can grow bigger in the early phases of life due to dilatations of the pre-existing vessels rather than to the development of new vessels, but tend to remain stable as for dimensions in adulthood, especially those ones smaller than 4 cm. Nevertheless, if angiomas get bigger, they can undergo several changes, such as: internal hemorrhage with necrosis, thrombosis, myxomatous modifications, fibrosis and calcifications. Such modifications could account for the different echo-pattern , but genSome reports have documented hemangiomas growing bigger in time. A spontaneous grow of hemangiomas has been suggested and also other causes have been envisaged, for instance a chronic therapy with estrogens. In our Secondarily, the anechoic formation, detected in the last sonographic examination, could be related to the angioma's cystic degeneration ,10.The hepatic cavernous hemangioma with cyst formation is very rare. In literature only three cases of liver's cystic hemangioma have been reported. Two of them have stated the formation of multilocular cysts, whereas the other one has shown a single unilocular cyst. Tumour's cystic degenerations have been attributed to necrotic changes caused by the development of an infarction area due to vessels' occlusion .In our case, considered the ragged shape of the formation detected within the angiocavernoma, the tumour's cystic degeneration seems rather unlikely. In this case the anechoic formation should have been roundish with regular walls.In time, the patient's hemodynamic conditions, well balanced at first, started to worsen. This could be related to blood seizure by the large hepatic angioma due to the intratumoural haemorrhage. In spite of the presence of this gigantic angioma, signs and symptoms typical of the Kasabach-Merritt syndrome were not reported.In order to better study the transformation of the angioma detected in the second ultrasound scanning and indentify a possible relation with the worsening of the patient's cardiac conditions, it would have been necessary to carry out further tests, which couldn't be performed due to the patient's exitus.In any case, we consider this case very interesting, both for the angioma's initial echografic appearance and for its particular and uncommon transformation which probably could be responsible for the worsening of hemodynamic condition that caused the patient's exitus.We maintain that our case represents an interesting communication first for the early aspect of the cavernous haemangioma, that is quite different from other hepatic cavernous hemangiomas reported in the literature. Moreover its interest can be attributed to the peculiar change of the lesion that turned into a liquid concistency. Such a change is rather uncommon, as it involves the whole lesion.We underline that our data result from the ultrasonographic monitoring of the lesion which has been judged, in any way, along with the patient's clinical conditions. We think that the results of our case report may stimulate to closely monitorate all those angiomatous hepatic lesions whose ultrasonographic appearance is closely related to the one we are presenting.CT: computed tomography.The authors declare that they have no competing interests.AMB, GS and CV collected the data and drafted the manuscript. AB and RF took care of the patient during hospitalization. All authors read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Rapunzel Syndrome is an uncommon presentation of trichobezoar, involving strands of swallowed hair extending as a tail through the small intestine, beyond the stomach. This was first described in 1968 and until 2003 only thirteen cases have been reported.'comma'. At laparotomy, the stomach and third part of the duodenum were distended and contained a large mass of dark, foul-smelling hair that occupied the stomach, crossed the pylorus and extended into the small bowel. A retroperitoneal perforation of the third part of duodenum was found and repaired. The large trichobezoar was removed via a gastro-enterotomy and the patient made a slow, but complete recovery.A 55-year old man of East Indian descent presented to the surgical team via the emergency department in Trinidad, West Indies, with an acute abdomen and small bowel obstruction. He had a recent psychiatric history and patchy alopecia as well as a family history of schizophrenia. A supine abdominal radiograph revealed a small bowel obstruction as well as an unusual air shadow in the left hypochondrium in the region of the duodeno-jejunal flexure associated with the stomach bubble, which resembled a the comma sign. To the best of our knowledge this is the first reported case of a spontaneous retroperitoneal perforation of the third part of duodenum associated with Rapunzel Syndrome.We wish to report another case of Rapunzel Syndrome and describe an unusual radiologic sign associated with a retroperitoneal perforation of the third part of duodenum –  Vaughan et al first described it in 1968 with only 13 cases formally reported in the literature to date [the comma sign.Bezoars are collections of indigestible organic or inorganic foreign material in the gastrointestinal tract, which have usually accumulated in the stomach. Classification depends on composition and four types of bezoar have been described; phytobezoars, composed of vegetable matter, lactobezoars, which occur secondary to infant formula and consist of milk curd, pharmacobezoars or medication bezoars and trichobezoars, which are conglomerations of hair or hair like fibres. Rapunzel Syndrome is an uncommon presentation of trichobezoar, involving strands of swallowed hair extending as a tail through the small intestine, beyond the stomach .  to date . We wishA 55-year old man of East Indian descent presented as an emergency to the surgical team via the emergency department in Trinidad, West Indies, with diffuse abdominal pain, fever, nausea and constipation. The abdominal pain was generalised and colicky in nature, and the patient was most tender in the peri-umbilical region. He had a recent psychiatric history and patchy alopecia as well as a family history of schizophrenia.'comma' [Figure On examination the patient was pyrexial and tachycardic with a hard periumbilical abdominal mass as well as generalised tenderness and guarding. Bilateral inguinal hernias were found to be easily reducible. A supine abdominal radiograph revealed a small bowel obstruction associated with an unusual air shadow in the left hypochondrium in the region of the stomach and duodeno-jejunal flexure, which resembled a ' Figure . UnfortuAt laparotomy the stomach, third & fourth parts of the duodenum were distended and contained a large mass of dark, foul smelling hair that had crossed the pylorus and extended into the small bowel. There was also a retroperitoneal perforation of the third part of the duodenum with air in this space. The large trichobezoar was removed via a gastro-enterotomy. Recovery was slow but complete and the patient was eventually discharged.Trichobezoars, although rare, are most common in children and young women. DeBakey and Ochsner looked at 172 cases of trichobezoar and found that almost 90% occurred in teenage females . There iRapunzel Syndrome occurs when the trichobezoar has extended into the duodenum and small intestine and manifests itself with nausea, vomiting, anorexia and weight loss .Duncan et al in 1994 [Rapunzel Syndrome was first reported in the West Indies by  in 1994 . The diaThe use of ultrasound (US), CT scanning and endoscopy have vital roles, and the trichobezoar may appear as a gastric obstruction and palpable mass in the epigastrium. The upper part of a large bezoar may be visible as a mass with a convex upper border projecting into the gastric air bubble. An erect abdominal radiograph and a supine radiograph may also show a prominent gastric outline with an intragastric mottled mass, outlined by gas in the distended stomach, mimicking a food-filled stomach. With a small amount of barium the hair-ball gets coated and becomes visible. Filling the stomach with barium demonstrates a mobile intraluminal filling defect of variable size, which may show extension into the duodenum Figure .Abdominal Ultrasound shows a dense, echogenic rim with sharp, clear posterior acoustic shadowing in the epigastric region. This characteristic appearance persists irrespective of the angulation of the transducer or alteration of position of the patient. This can be attributed to multiple tiny interfaces between the smooth, compressed, compact mass and the entrapped air and food debris. This specific US appearance excludes the clinical possibility of a pancreatic pseudocyst, splenic or renal mass, non-calcified gastric tumour, gastric duplication cyst and gastric outlet obstruction. However a heavily calcified mass such as teratoma, neuroblastoma or impacted mass of faeces may produce a similar US image.Plain abdominal CT usually shows a mobile intragastric mass consisting of "compressed concentric rings", with a mixed density pattern due to the presence of entrapped air and food debris Figure . Often sPerforation resulting from such obstruction appears traditionally as free air under the diaphragm on an erect chest radiograph, with a mottled gas pattern over the stomach resembling pneumatosis intestinalis . CT appearances show pneumoperitoneum and curvilinear, serpiginous, bubbly collections of gas that occur circumferentially or in peripheral or dependent portions of the bowel wall with long segments bowel usually affected.The diagnosis can also be established by endoscopy, where trichobezoars are often found to have a hard, concrete like appearance and enzymatic oxidation of hair gives it a darkened colour. Treatment may include endoscopic removal, chemical dissolution and mechanical fragmentation, for small bezoars. However, surgery is sometimes necessary for large bezoars with extension into the bowel as seen in this case. The bezoar plus its tail can be removed via gastrotomy and sometimes multiple enterotomies to reduce risk of gut perforation, as long bezoar tails are often extremely adherent to the side of the gut wall . In thisKumar et al first reported recurrent trichobezoar in 1996, where it was seen to occur because the underlying emotional stress trigger was not corrected [Memon et al in 2003, the cause being non-compliance of the patient regarding her psychiatric medication [orrected . Recurredication . Indeed,'comma' in the left upper quadrant was actually the stomach bubble (with the bezoar in it) and air in the retroperitoneal space surrounding the perforation of the third part of duodenum and duodeno-jejunal flexure thereby simulating a 'comma'.We report this further case of Rapunzel Syndrome associated with a small bowel obstruction. Additionally, we learnt in restrospect that the radiographic appearance of the 'comma sign' associated with Rapunzel Syndrome for the first time. We also acknowledge the fact that the 'comma sign' may apply to any patient with a retroperitoneal perforation of the third part of the duodenum. However, this by itself is quite a rare occurrence and when identified on a plain supine abdominal radiograph should alert the clinician to rare pathology such as tumour, foreign body or trichobezoar.Of all the cases of Rapunzel Syndrome described in literature with free perforations, these cases presented with acute abdomens and free air under the diaphragm. We would therefore like to describe the We recommend closer attention be paid to the appearance of this sign on a plain supine abdominal radiograph in order to facilitate greater recognition of retroperitoneal perforations in this region and to correlate the findings with the history in order to diagnose bezoars of the Rapunzel Syndrome type pre-operatively.We hope this case report reminds and helps our emergency medicine and surgical colleagues with the diagnosis and treatment of this rare and unusual condition in the future.Written informed consent was obtained from the patient for publication of this case report and accompanying photographic and radiographic images.The authors declare that they have no competing interests.SD reported the case, provided the imaging and helped to draft the manuscript. NB drafted the manuscript, researched the factual content and literature and revised it critically for important intellectual substance. PS oversaw the project and helped draft the manuscript. VN oversaw the project and helped draft the manuscript. All authors read and approved the final manuscript.


A rare case of a thoracic primitive neuro-ectodermal tumor in an adult is presented here. In this case, wide excision surgical excision followed by chemotherapy and radiotherapy were delivered. But due to the rapid aggressive progression of the tumor, which is the characteristic of disease, the patient died within four months after the diagnosis. Primitive neuro-ectodermal tumors (PNET), described originally by Askin and colleagues, are infrA 28-year old male, presented with chest pain and mild dyspnea, of 20 days duration. He was a farmer and nonsmoker with no history of exposure to any occupational or inorganic dusts. The chest pain was retrosternal and pricking in character with no radiation and not related to the meals. Examination revealed averagely built person and no clubbing and lymphadenopathy. Respiratory system evaluation revealed signs of mass in the left hemithorax. Chest radiograph revealed a large mass near the left hilum . Routineet al.[PNET are rare tumors occurring in the posterior sulcus or chest wall of adolescent or young adult patients. They are believed to develop from the peripheral nerves such as an intecoastal nerves. They are highly aggressive neoplasms of the thorax with a median survival of eight months. They are typically painful, invasive thoracic tumors that may develop on and invade chest wall, lung, or mediastinum. They areet al. with thoet al. Results et al. Characteet al.The treatment for PENT must consist, wherever possible, radical resection supplemented with radiotherapy  and aggressive chemotherapy . The average life expectancy is eight months, although occasional long-term survival can be as long as 96 years.[The present case describes one of the few reported cases of PNET in an adult. It is unusual for the age of presentation and a short duration of unique symptom i.e., thoracic pain, which had started only few days before hospital admission. Plain chest radiograph and CT scan were diagnostic for pulmonary tumor and FNAC was diagnostic for a small cell tumor with a high suspicion of malignancy. Open thoracotomy with excision of the removable tumor proved the diagnosis of PNET. The tumor had invaded the great vessels in the thorax and the mediastinal lymph nodes due to the advanced nature of the tumor.Primitive neuroectodermal tumor lesions are aggressive and are usually lethal; they should be considered in the differential diagnosis of thoracic tumors regardless of the age of the patient. Once the diagnosis of the PNET has been made, early wide excision of the tumor, along with multimodality chemotherapy and radiotherapy, should be undertaken to offer any hope of a long-term cure.


Aggressive treatment, including resection of both metastasis and primary tumor, has been studied for non-small cell lung cancer patients with synchronous solitary brain metastasis. Involvement of mediastinal lymph nodes is considered a poor prognostic factor and a contraindication to surgical resection of the primary lung tumor after treatment for brain metastasis. Here we present the case of a patient who presented with a Stage IV T1N2M1 non-small cell lung cancer with synchronous solitary brain metastasis. He is alive and without evidence of disease two years after aggressive, multimodality treatment that included craniotomy, whole-brain radiation therapy, thoracic surgery, chemotherapy, and mediastinal radiation therapy. Brain metastases affect approximately 25% of patients with non-small cell lung cancer (NSCLC). For the subset of patients who present with a solitary brain metastasis (SBM), in the absence of other sites of metastatic disease, aggressive local therapy to the primary and metastasis is warranted . CraniotA 52-year-old male presented to medical attention with severe headaches. Magnetic resonance imaging (MRI) revealed an enhancing, intra-axial mass in the left cerebellum, measuring 2.3 × 2.3 × 2.5 cm and accompanied by surrounding edema . A CT ofHe was evaluated in multidisciplinary lung clinic after WBRT for discussion of further management of his NSCLC. His past medical history was significant for only hypercholesterolemia and history of a benign right flank mass that was surgically removed in the remote past. He had a thirty-pack-year history of smoking and chose to quit smoking when diagnosed with NSCLC. On examination, his lungs were clear to auscultation bilaterally. No focal neurologic deficits were discovered on examination. His Karnofsky Performance Status (KPS) was 100%, with an absence of any adverse prognostic factors such as weight loss or laboratory abnormalities.Staging evaluation was completed with whole-body, 18-fluoro-deoxyglucose (FDG)-positron emission tomography (PET)/computed tomography (CT) imaging, which demonstrated a hypermetabolic left upper lobe tumor and two hypermetabolic foci in the left para-aortic and suprahilar regions Figures . The FDGTwo years after completion of therapy, whole-body FDG-PET/CT imaging Figures  have demThis case serves as an example of the aggressive management of oligometastasis, a proposed, clinically significant disease state that exists between locoregional confinement and widespread metastatic disease . The oliThe use of aggressive, local therapy for SBM is supported by prospective trials that demonstrate a benefit for craniotomy or SRS for brain metastasis , 4. WhilThe patient in the current report presented with an excellent performance status and was agreeable to an aggressive, multimodality treatment approach. Abrahams et al., in their report of 70 patients with NSCLC, found that the patients who benefited most from an aggressive treatment approach were those with a KPS of 100% . Perform


Spontaneous rupture of the pancreatic pseudocyst into the surrounding hollow viscera is rare and, may be associated with life-threatening bleeding. Such cases require emergency surgical intervention. Uncomplicated rupture of pseudocyst is an even rarer occurrence. We present herein two cases of uncomplicated spontaneous rupture of a pancreatic pseudocyst into the stomach with complete resolution. Spontaneous rupture of the pancreatic pseudocyst into the surrounding hollow viscera is rare and, whenever it occurs, is associated with life-threatening bleeding.We present a series of two cases of uncomplicated spontaneous rupture of a pancreatic pseudocyst into the stomach with complete resolution.A 44-year-old nonalcoholic male presented with an epigastric mass and postprandial fullness. The patient had been treated as acute pancreatitis by conservative management 2 months earlier. Contrast enhanced computed tomography (CECT) revealed a large retrogastric cystic mass with normal enhancement of the surrounding pancreatic tissue measuring 7.6 × 7.5 cm . Seven dA 70-year-old female with a previous history of pancreatitis presented to the emergency service as a palpapable abdominal mass. Ultrasonography followed by CECT of the abdomen revealed a large pseudocyst measuring 12 × 8 cm at the porta extending into the omental bursa . The patA CT scan showed near-complete resolution of the large pseudocyst in the omental bursa with decompression of the biliary tree . ResiduaMany cases of spontaneous resolution of pancreatic pseudocyst have been reported in the literature. However, all the patients had evidence of acute gastrointestinal bleed, extension into the biliary tree, and pelvicalceal system in one case. Both our patients reporting the above did not develop any such complication.et al.[In a longitudinal study over 30 months, Mehta et al. found thSpontaneous rupture of the pancreatic pseudocysts is known to occur into the stomach, duodenum, biliary tract, renal collecting system, colon, and bronchial tree. However,


Background. Localized granulocytic sarcoma of the uterine cervix in the absence of acute myelogenous leukemia (AML) at presentation is very rare, its diagnosis is often delayed, and its prognosis almost always ominous evolving into refractory AML. Case. We present the case of a 30-year-old woman with vaginal bleeding and a large cervical mass. Further evaluation confirmed the presence of a granulocytic sarcoma but failed to reveal systemic involvement. Results. AML type chemotherapy followed by radiotherapy of the uterus led to a durable complete remission. She remains in complete remission six years after diagnosis. Conclusion. Granulocytic sarcoma of the cervix is a rare entity for which early intensive AML type therapy is effective. Granulocytic sarcoma (GS) is a rare solid tumor consisting of immature myeloid cells first described as a chloroma by Burns in 1823 because its green color due to myeloperoxidase staining –6.  The incidence of GS is estimated at 0.7 per million in children and 2 per million in adults [ It most commonly occurs in bones, the periosteum, soft tissues, lymph nodes, and skin but can occur virtually anywhere. The most commonly involved visceral organ is the kidney.  GS has a poor prognosis. The overall 2-year survival rate for all patients is 6%. No patient surviving more than 5 years has been reported in the literature .  Involvement of the female genital tract in women dying of leukemia is frequent but clinically significant involvement is rare. The most commonly involved organ is the ovary estimated at 36.4% followed by the cervix and uterus . The pre Only a few cases have been reported in which localized GS of the cervix was the only manifestation. Pathak et al. described in their review of 25 patients with GS of the cervix only two cases of confirmed isolated initial cervical involvement. In both cases an AML was subsequently diagnosed. One patient died of the disease, and the other was in remission after acute leukemia chemotherapy in less than 2 years of follow-up .  We present here a 30-year-old female with isolated granulocytic sarcoma of the cervix without evolution into AML and in complete remission after 6 years of follow-up.A 30-year-old,  white female visited her gynecologist with complaints of irregular vaginal bleeding. Four months earlier, she had had an episode of abnormal vaginal bleeding which lasted two weeks. There was no history of abdominal pain or systemic symptoms. Her last PAP smear, one year prior to diagnosis, was normal. A pelvic examination revealed hypertrophy of the cervix, and cervical biopsy showed an infiltration of small, immature myeloid cell. Laboratory studies were unremarkable. A pelvic CT scan and MRI revealed a mass at the level of the cervix measuring 8 × 5 cm. The mass was homogeneous, well defined, infiltrating, but spared the rectum and the bladder wall. No lymph node enlargement was seen. Ovaries, liver, spleen, pancreas, and kidneys were normal in appearance. There was no ascites and no thoracic involvement. The patient was subsequently referred to our department.  She was a social worker with a 2-year-old daughter who had been delivered by cesarean section. The rest of the patient's medical history was unremarkable. 3/mL, and white-cell count 7300 per cubic millimeter, with 62.5% neutrophils, 30.2% lymphocytes, and 5.3% monocytes. Blood chemistry was unremarkable. Cytological examination of cervical smears was composed of 50% of hematopoietic cells, small to medium sized blasts, with high nuclears-cytoplasmic ratio, fine chromatin, and fine granulations in the cytoplasm of myeloid origin. The blasts were myeloperoxidase positive. A biopsy was performed (two fragments of 1.3 × 0.5 × 0.2 and 1.1 × 0.7 × 0.2 cm) which showed extensive infiltration of the squamous epithelium wall by small-sized blast cells. Multiple immunohistochemical analyses were obtained. Myeloperoxidase, Common leucocytic antigen, and CD34 were strongly positive.  and idarubicin (8 mg/m²/day for five days). A postchemotherapy examination of the cervix was normal, and a biopsy performed one month after chemotherapy confirmed the absence of residual blast cells. The patient desired subsequent pregnancies in the future and so the following treatment strategy was adopted: one cure of consolidation chemotherapy with high-dose cytosine arabinoside (3 g/m² × 2/day for 4 days) and idarubicin (12 mg/m²/day for 2 days) followed by laparoscopic protective ovariopexy and external beam radiotherapy of 30 Gy limited to uterus. This was followed by a second cycle of consolidation chemotherapy with high dose cytosine arabinoside (3 g/m² × 2/day for 4 days). At the last follow-up in December 2008, more than six years after the diagnosis, she remains in complete remission. Complete blood counts, abdominal and pelvic ultrasonography, and physical examination were normal.The mean age at presentation of cervical granulocytic sarcoma is 47 years, range from 26 to 75 of age . The two The presenting feature in our case was vaginal bleeding as with the majority of patients: 81–83% of presenting symptoms. Abdominal pain or discomfort present in 17–29% of patients and systemic symptoms in 6–17% were absent in our patient. Finally a combination of other symptoms is responsible for 17% of initial complaints [ The diagnosis is not always easy when GS appears at an extramedullary site in a nonleukemic patient. Most of them are poorly differentiated, and only in 44% of cases the correct diagnosis is made or suspected , 10. The GS of the cervix has a poor prognosis. Treatment is variable but often delayed. Few reports of complete remission have been described after aggressive multimodality treatment of GS in other sites including disseminated GS without evidence of AML .  Our patient was treated with AML type chemotherapy regimen and localized uterine radiotherapy. A complete response was seen soon after treatment, and she remains in complete remission. It appears appropriate to treat GS with AML type chemotherapy protocols even in the absence of systemic manifestations, since acute myeloid leukemia will almost always be present . To our  In the hope of maintaining fertility, she was treated with an LHRH analogue during chemotherapy. She had an ovariopexy before radiotherapy and recovered her menstrual cycles seven months after treatment. Unfortunately she was unable to conceive and even failed several attempts at in vitro fertilization.  Our patient received a total radiation dose of 32.4 Gy. The irradiated uterus suffers somatic damage; it causes endometrial and myometrial atrophy, scar fibrosis, and hypovascularization which interfere with implantation . IndepenGranulocytic sarcoma of the cervix is a rare entity and even in its localized form, early intensive AML type therapy appears to be appropriate. Even though our patient has had a long complete remission, the role of radiotherapy in addition to chemotherapy remains unclear.The authors declare that there are no conflicts of interest.


The diagnosis of deep vein thrombosis of leg is very common in clinical practice. Not infrequently a range of pathologies are diagnosed after excluding a thrombosis, often after a period of anticoagulation.This is a report of three patients who presented with a painful swollen leg and were initially treated as a deep vein thrombosis or a baker's cyst, but later diagnosed as a pleomorphic sarcoma, a malignant giant cell tumor of the muscle and a myxoid liposarcoma. A brief review of such similar reports and the relevant literature is presented.A painful swollen leg is a common clinical scenario and though rare, tumors must be thought of without any delay, in a duplex negative, low risk deep vein thrombosis situation. Painful swollen leg is a common clinical scenario. Deep vein thrombosis (DVT) often presents as a painful swollen leg and prompt management is vital to prevent fatal pulmonary embolism. The common differential diagnoses include cellulitis and a ruptured baker's cyst . Rare paA 70 year old female presented to general practitioner with complaints of pain in left knee and calf. Initial knee radiographs showed early osteoarthritis. As there was associated calf tenderness she was admitted for further investigations. All blood parameters were normal. D-dimers at the time of admission were 440 ng/ml. She was categorized as moderate risk for a DVT on clinical examination. Anticoagulation was initiated suspecting a DVT and the Duplex scan of the leg was inconclusive. The pain settled but swelling persisted and the patient was managing her regular activities. After six months since the initial presentation she was referred to us for increasing swelling of the left leg. There was marked swelling with venous congestion . After full length X-rays of leg Figure , she hadA 59 year female was investigated for a possible venous thrombosis of calf at the emergency department after she presented with a painful swollen left proximal calf. She was categorized as a low risk for DVT on clinical examination. D-dimers were 260 ng/ml and Duplex imaging was equivocal. She was started on treatment dose of heparin.After 3 weeks of anticoagulation there was evidence of a lump in the left popliteal fossa and ultrasound scan of the area revealed a solid soft-tissue mass. She had distal paraesthesia in the foot without any motor weakness. CT scan revealed a soft tissue lump behind knee & proximal calf and she was referred to our centre.On examination she had a diffuse swelling behind the knee with a good range of painless movement. Ultrasound guided biopsy revealed an extra-articular diffuse malignant giant cell tumor arising from muscle. On MR imaging Figure  &3B therA 69 year male was seen for a swollen and painful right calf following a minor trauma. This was treated initially with physiotherapy and the pain settled. Two years later he was further investigated for a similar episode, this time to rule out a DVT. The D-dimers were normal and a Doppler scan ruled out a DVT, but a baker's cyst was diagnosed. Following this episode the symptoms never settled and he was later reviewed for a sudden increase in size of the calf 4 years later. There was an 8 × 8 cm diffuse but discrete swelling over the lateral aspect of his calf.An MR scan revealed a heterogenous soft tissue mass probably of fatty origin in the posterior compartment measuring 10 × 25 cm Figure  &4B. ThiPainful swollen leg is a common clinical scenario for a wide range of pathology. The initial management in the majority is to start anticoagulation and arrange a venous duplex scan, as the priority is to rule out a DVT. But only one third of the first episodes of a venous thrombosis are spontaneous . PatientIn case of the first patient, a moderate risk for DVT clinically along with moderately elevated D-dimers prompted anticoagulation. But after an initial equivocal Duplex, a repeat scan or venography should have been performed to establish or exclude a DVT. There was some relief of pain which made both the patient and physician less concerned. Only after 6 months when the swelling was much worse, an alarm was raised. In the second patient a DVT was unlikely given the low clinical risk and the D-dimer level of 260 ng/ml. Further evaluation should have been done, as an alternate diagnosis was more likely. In her case the lump was much more proximal to be appreciated within 3 weeks of initial presentation. The combination of low pre test probability of a DVT along with an inconclusive Duplex scan in these patients must have prompted further investigations. In the final patient of this series, a Duplex was sensitive enough to diagnose a Baker's cyst but was unable to detect a co-existing solid soft-tissue swelling which was probably small by the time. A general ultrasound in this patient must have a given a better information regarding the underlying pathology.The discovery of nonvascular disease is not an infrequent finding of duplex scan. Baker's cyst is the commonest non-vascular abnormality found in patients undergoing duplex scan for a suspected DVT 3%) . The oth% . The oThe purpose of this case series is to highlight the need to be vigilant before diagnosing a DVT in low risk patients presenting with a painful swollen calf and make clinicians realize that d-dimer levels alone could be misleading as levels could be moderately elevated in other pathologies. A delay in diagnosing tumors could affect the overall prognosis hence further investigations or imaging should be considered without any delay.The author(s) declare that they have no competing interests.BA drafted the manuscript and performed the literature review. VL has compiled the figures and collected the necessary data of patients. AP conceived of the project and coordinated the final draft along with proof reading. All authors read and approved final manuscript.


Primary osteosarcoma is one of the rare tumors affecting the urinary bladder. The occurrence of osteosarcoma in a patient with a long history of transitional cell carcinoma of the bladder is even more uncommon.We present the case of a 74-year-old Greek man who was diagnosed with osteosarcoma 10 years after he had been diagnosed with transitional cell carcinoma of the bladder from which he had been free from recurrences for the past three years. Our patient was treated for the osteosarcoma with transurethral resection of bladder tumor and external beam radiation therapy. He died eight months after the diagnosis, suffering poor quality of life in the last months.Osteosarcoma of the bladder has a dismal prognosis. External beam radiation therapy as an adjunct to transurethral resection of bladder tumor not only provides no benefit to patients with primary osteosarcoma of urinary bladder, but also may be associated with poor quality of life. Osteosarcoma of the urinary bladder is a rare tumor. To date, only 32 cases have been reported using several treatment modalities -3. We prA 74-year-old Greek man presented in June 2008 with gross hematuria. Our patient had been previously treated for non-muscle invasive pTa, G2 transitional cell carcinoma of the bladder (TCC). The first transurethral resection of bladder tumour (TURBT) had been performed ten years before, while the last was performed three years before. The last follow up cystoscopy had been performed twelve months before. Our patient was asymptomatic thereafter, until June 2008.Then, at cystoscopy, a large solitary polypoid, yellow-white tumor was found on the superior surface of the bladder. A computed tomography (CT) scan revealed a heterogeneous mass arising from the bladder dome with evidence of invasion of the bladder wall figure . There wImmunohistochemical analysis performed on paraffin-embedded tissue sections was negative for pan-cytokeratin 7 and 20, epithelial membrane antigen (EMA), as well as a smooth muscle actin, desmin, CD34 and CD68. Vimentin and p53 were strongly expressed in more than 95% of the neoplasmatic cells. No transitional cell carcinoma or carcinosarcoma elements were evident  instead. Our patient declined to receive adjunctive chemotherapy. In the following months, he had two episodes of proven urinary tract infection. Seven months after diagnosis, he developed ascites. A CT scan at that point showed extravesical tumor extension and bowel infiltration figure . Our patOnly 32 cases of primary osteosarcoma of the bladder have been so far reported in the English literature -3. To thOsteosarcoma should be distinguished from other bone-forming tumors, such as carcinosarcoma and transitional cell carcinoma with osseous metaplasia. It is not always easy to distinguish extraskeletal osteosarcoma from other sarcomas, which rarely produce metaplastic bone such as malignant fibrous histiocytoma, synovial sarcoma and epithelioid sarcoma. In most of these neoplasms, osteoid or bone formation is confined in a small portion of the tumor and is relatively well differentiated, without the disorderly pattern and cellular/nuclear polymorphism/pleomorphism of osteosarcoma. The characteristic microscopic pattern of our tumor, that is, the malignant, lace-like osteoid, produced in large amounts by the spindled sarcomatous cells, and the presence of chondroid matrix between these cells along with the above-mentioned immunoprofile and the absence of concurrent epithelial malignancy, supported the diagnosis of primary osteosarcoma of urinary bladder -9.The prognosis of bladder osteosarcoma is very poor, with most patients dying from the disease within six months. The tumor tends to be locally aggressive. Distant metastases are uncommon and are usually confined to the lung. Recommendations to improve survival have included radical cystectomy with radiotherapy and/or chemotherapy, but most patients die of this disease regardless of the type of treatment ,10.However, there are publications that report successful treatment with a long follow- up. One patient was free of such disease 36 months after partial cystectomy  and anotIn our case, our patient had a long history of TCC and also had significant comorbidity. He was thoroughly informed about the disease and finally decided to undergo EBRT. Although he lived few months more than usual, during the last four months, he suffered severe LUTS, abdominal pain and was heavily medicated with opioid analgesics. For the above-mentioned symptoms, there was no obvious etiology other than radiotherapy.Osteosarcoma of the bladder is a rare tumor with a dismal prognosis. External beam radiation therapy as adjunct to transurethral resection seems to provide no benefit for patients with primary osteosarcoma of urinary bladder. Moreover, it may be associated with poor quality of life and thus should be better to be avoided.CT: Computed tomography; TCC: transitional cell carcinoma; EBRT: external beam radiation therapy; TURBT: transurethral resection of bladder tumor; LUTS: lower urinary tract symptoms.Written informed consent was obtained from our patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.CP was involved in patient care, review of literature and writing of the manuscript. AS performed the histological examination and reviewed the literature. GK was involved in radiological evaluation of our patient. HD was involved in patient care and writing supervision. All authors read and approved the final manuscript.


Primary intraosseous meningiomas are uncommon. The osteolytic variants of these tumors are even rarer. When one reviews a bony skull lesion, the differential diagnosis is very wide and includes both malignant and benign diseases. In case of primary skull meningioma, correct diagnosis and total resection of the lesion ensures curative therapy. Presented is a case of osteolytic skull lesion not invading the dura that was proved to be a fibrillary meningioma with regions of syncitial pattern. Of a rarer prevalence are lytic skull meningiomas that are not readily suspected because of their radiological appearance; rather they are investigated first for a primary source of malignancy elsewhere in the body or are thought to be other lytic lesions of the skull. Yet, osteolytic meningiomas, although rare, should be considered when approaching to a lytic skull lesion since their natural history and treatment outcome are much different from other more common diagnoses in the skull such as metastatic cancer.Primary meningiomas of the skull without involvement of the dura and intracranial cavity comprise an uncommon entity. These lesions often appear as hard osteoblastic tumors which appear as hyperdense areas of the calvarial bones in x-ray studies . They arA 62-year-old right-handed male Iranian farmer presented with a soft enlarging mass in the left fronto-parietal region. The mass had come to the patient's attention over last 8 months but was ignored until it was of a considerable size. Its largest diameter was about 9 centimeters and it was a fluctuating mass. The patient had undergone scalp radiotherapy for treatment of ringworm about 45 years ago. He had no remarkable medical history. Drug history was negative and he did not smoke. Skull x-rays and head computed tomogram (CT) showed a lytic lesion of the skull in the left fronto-parietal region.An extensive physical examination and laboratory/radiologic work-up including a whole body radionuclide scan of bone showed no other lesion elsewhere in the body. Levels of tumor markers such as prostatic specific antigen (PSA) and evaluation of chest and abdomen were normal.Magnetic resonance imaging of head showed an extradural intradiploic enhancing mass lesion without intracranial extension .The patient underwent surgery for resection of the mass lesion. Skin was easily reflected and the mass was not adhered to it. The lesion appeared as a soft lobulated gray mass that had destroyed the calvarial bone. It was easily suctioned and had extension to the peripheral diploe. The infiltrated bone around the lesion was resected so that a rim of healthy bone with normal strength was reached. The dura was intact and the lesion was easily peeled off.Pathologic examination of the lesion revealed fibrillary meningioma with areas of syncithial differentiation . RegardiExtradural meningiomas constitute 1-2% of all meningiomas : tumors Primary intraosseous meningiomas are usually of osteoblastic subtype. More rarely, these lesions may present as an osteolytic skull lesion . The dif


Combined malignant naevi are characterised pathologically by the association of a melanoma with one or more different types of benign melanocytic naevi in a single lesion.We show here a case of malignant combined naevus made up of a blue naevus and a melanoma, presenting as a slowly progressing and asymptomatic pigmented lesion on the trunk of a 35-year-old man. Dermoscopic examination was not conclusive for a malignant lesion, showing only an atypical brown pigment network. The definitive diagnosis was reached only at the hystopathological examination.This finding suggests that combined naevi should be always excised and histologically examined to achieve a correct diagnosis and avoid risk of misclassification. Combined naevi are unusual pigmented skin lesions that represent less than 1% of all biopsed melanocytic naevi . HoweverA 35-year-old man presented with a 1-year slowly progressing and asymptomatic pigmented lesion of the trunk. Physical examination revealed an irregular, oval, blue-brown plaque with the dimensions of 5.5 × 3.3 mm. Dermoscopic examination revealed a multicomponent pattern made up of an homogeneous central steel or blu-gray pigmentation with an irregular border surrounded by an atypical brown pigment network Figure . This pade novo melanomas and are also less specific for malignancy [The clinical and pathological significance of melanocytic naevi lies principally in their relationship with melanoma. In the case of combined naevi the problem of a correct diagnosis is complicated by the presence of two or more different types of melanocytic naevi in a single lesion . Overalllignancy . This coThe authors declare that they have no competing interests.AB and ED performed the surgical procedures and contributed to the analysis of the clinical data. PM performed the histological examination of the lesion. RM and PP contribuityed to the interpretation of the data and to the discussion of the manuscript AB performed the histological examination of the lesion and was a major contributor in writing the manuscript together with PM. All authors read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Overall, epsmcc accounts for only 0.1%–1% of all gastrointestinal tract (git) malignancies, which mostly affect the esophagus and colon. It is frequently metastatic at presentation, with high-grade pathologic features and aggressive clinical behaviour. Management strategies are varied, but chemotherapy remains the cornerstone of treatment. Response to platinum-based chemotherapy is between 50% and 100%, but relapses are almost universal, and prognosis is poor.Extrapulmonary small-cell carcinoma  is rare and represents a clinicopathologic entity distinct from pulmonary small-cell carcinoma. It has been described in all anatomic sites outside the central nervous systemgit), the genitourinary and reproductive systems, the salivary glands and sinuses, and lymph nodesepsmcc represents 0.1%–1% of git malignanciesExtrapulmonary small cell carcinoma , esophagus (30%), and stomach (8%); only about 3% involve the small bowelepsmcc of the duodenum in 2004, only 9 cases had been reported in the literatureWithin the ct) imaging of the chest, abdomen, and pelvis, and git endoscopy. Diagnosis is confirmed when biopsy shows small cells with scanty cytoplasm and round nuclei combined with histologic features typical of high-grade neuroendocrine tumours. Staging can be defined by the tumour–node–metastasis (tnm) system, but more commonly uses the Veterans Administration Lung Study Group  system which identifies two distinct stages: limited stage (ls) or extensive stage (es), based on whether the disease is confined to a locoregional anatomic location or whether it is not.Clinical presentation often involves weight loss and anorexia, or site-specific symptoms such as abdominal pain, obstruction, bleeding, or mass. Staging workup depends on the site of the primary, but often involves computed tomography , for which an endoscopy and colonoscopy were performed. Those tests failed to reveal any cause for the anemia. Physical exam demonstrated a firm, slightly tender, palpable mass in the upper abdomen, but no hepatomegaly or splenomegaly. There was also no jaundice or systemic evidence of liver disease, and no palpable lymphadenopathy. No rales, wheezes, or areas of reduced air entry were observed on auscultation. He had a performance status of 2.ct showed a large heterogeneous mass appearing to arise from the small bowel mesentery, measuring 15.7 × 26.3 × 22 cm, and partly encasing loops of small bowel [ct demonstrated a single 2.2 × 2.1-cm subpleural lesion in the left lower lobe of the lung, abutting the inner aspect of the thoracic wall, appearing to arise from the abdomen, with no intrathoracic adenopathy. A ct-guided biopsy of the abdominal mass demonstrated a poorly differentiated neuroendocrine carcinoma with features of a small-cell carcinomact were negative for bone and brain metastases respectively. A diagnosis of epsmcc was made, and after prompt placement of a peripherally inserted central catheter, treatment with etoposide , but preliminary investigations did not reveal a source. On day 6, he developed worsening abdominal pain, somnolence, tachycardia, and hypotension. Peritoneal signs were present on physical exam. Imaging of the abdomen by Broad-spectrum antibiotics were instituted, and surgical consultation was requested. Surgical intervention was refused because of a high perioperative risk and impending neutropenia anticipated from the chemotherapy.Over the following 4 days, the patient’s clinical picture remained tenuous. He developed acute renal failure (creatinine rose to 188 μmol/L from a baseline of about 110 μmol/L) and anasarca. Antibiotics were added, ultimately including imipenem, vancomycin, metronidazole, ciprofloxacin, and fluconazole. With these interventions, the patient’s hemodynamics and overall clinical picture normalized by day 10.The patient recovered well from the foregoing events and received his 2nd cycle of chemotherapy on schedule. In light of the renal failure and gross edema, to which the cisplatin may have contributed because of its nephrotoxicity and the large fluid volumes given with the drug, cisplatin was replaced with carboplatin (area under the curve: 6). Antibiotics were discontinued, adequate diuresis was achieved, and the patient was discharged home on day 8 of cycle 2.ct imaging of the abdomen was requested to evaluate tumour response. On the patient’s return to the clinic 1 week later (day 22), his symptoms had not improved. Fever and worsening abdominal pain were now present. The abdominal ct demonstrated a large 25 × 16-cm cystic abdominal mass with air–fluid levels at the site of the previous tumour [ct-guided abdominal drainage, which removed more than 4 L purulent material, and intravenous antibiotics were administered. Symptoms improved immediately with drainage. The abdominal exudate grew Klebsiella, Bacteroides, Enterococcus, and Staphylococcus aureus.The patient returned to clinic on day 16 of cycle 2 with complaints of fatigue and dyspnea. He received 2 units of packed red blood cells because of a hemoglobin level of 85 g/L, and repeat ct on the day before drain removal showed complete resolution of the abscess and significant reduction in tumour size , receipt of systemic treatment, and for esophageal epsmcc, age and tumour size Like its counterpart in the lung, Treatment strategies are varied and include surgical, pharmacologic, radiotherapeutic, and combined approaches. However, chemotherapy, either alone or as part of a combined approach, has emerged as the cornerstone of treatmentepsmcc to platinum-based chemotherapy was complicated by intestinal perforation. Perforation is evidenced by the temporal correlation between chemotherapy administration, radiologic evidence of tumour necrosis, clinical manifestations of sepsis and acute abdomen, and subsequent polymicrobial intra-abdominal cultures that grew mostly enteric organisms. This sequence supports a pathophysiologic mechanism in which wall integrity of the small bowel is lost as the tumour rapidly necroses in response to chemotherapy. It is also possible, however, that the result could be related to an agent-specific risk, as is seen with bevacizumabIn the present case, rapid response of a large, intestinal-associated git—namely, lymphomas. Perforation is a common complication in this group of diseases, frequently seen at disease presentation and portending a poor prognosisBowel perforation has been extensively described in metastatic lung cancergit is rare and portends a poor prognosis. Response to chemotherapy is strong and often rapid, but relapses are almost universal, with few survivors beyond 2 years. In the case reported here, high chemosensitivity resulted in treatment-related perforation of the small bowel, sepsis, and abscess recurrence during a subsequent cycle of chemotherapy. Based on the literature of git lymphomas, we suggest that surgery be considered part of the management approach in some patients with epsmcc of the small bowel. As a theoretical framework, surgery should be advised if the risk of fatal complications such as perforation or hemorrhage outweigh the surgical mortality and morbidity. Given the lack of feasibility of prospective trials for this rare entity, large retrospective analyses are needed to better characterize the risk factors for perforation among patients with epsmcc of the git and to help guide future clinical management.Extrapleural small-cell carcinoma of the


Presacral masses are unusual growths that have a limited differential diagnosis, typically not including neuroendocrine tumors (NETs). Classically, NETs are well-differentiated gastroenteropancreatic tumors of probable benign behavior. These tumors are associated with a typical morphologic pattern and involve the distal colon, rectum, and genitourinary tract; they are considered less aggressive, frequently asymptomatic, and rarely cause carcinoid syndrome, even when metastatic. Neuroendocrine tumors of the presacral region are extremely rare and few have been described in the literature. They have not been previously reported as being associated with imperforate anus.We present an interesting case of a woman with a history of imperforate anus that was found to have a primary neuroendocrine tumor of the presacral region with no rectal wall involvement.We argue that this is a primary gastroenteropancreatic neuroendocrine tumor which likely originated from cells of hindgut origin that underwent an abnormal migration during embryonic development. Neuroendocrine tumors are rare with an incidence rate of two to five per 100,000, with a higher incidence rate for African-American men and women. The age distribution ranges from the second to the ninth decade, with peak incidence between the ages of 50 and 70. They are the most common neuroendocrine tumor of the gastrointestinal tract. Around 55% are located in the gastrointestinal tract and 30% in the bronchopulmonary system. In the gastrointestinal tract, they tend to arise most commonly in the small intestine (45%), followed by rectum (20%), appendix (16%), colon (11%), and stomach (7%) [Neuroendocrine tumors of the presacral region are rare and usually represent direct extension or metastasis from primary rectal tumors. Only a handful of primary presacral NETs have been reported in the literature and are felt presumably to derive from hindgut rests, especially given their histopathologic similarity with primary rectal NETs . We repoA 58 year old African-American female reported symptoms of abdominal pain and diarrhea. Physical examination including abdominal, vaginal, cervical and rectal exam were not contributory. She was found on computerized tomography scan of the abdomen/pelvis to have a 3.2 cm heterogeneous mass anterior to the coccyx, with central hypodensity and peripheral rim enhancement; the mass appeared separate from the posterior rectal wall. The pelvic organs were unremarkable, as no other abnormalities were identified on CT scan. Subsequent CT-guided biopsy and pathology was consistent with a neuroendocrine tumor. Colonoscopy was negative for primary tumor of the rectum. Transanal endoscopic ultrasound confirmed a heterogeneous and hypoechoic mass at 13 cm from the anal verge, clearly separate from the posterior rectal wall, with no discernible lymphadenopathy  declare that they have no competing interests.TK- Participated in the design of the study and helped to draft the manuscript. SRG- Conceived of the study and participated in its design and coordination and help to draft the manuscript. SNH- conceived of the study, participated in its design and coordination and helped to draft the manuscript, performed the revisions. All authors read and approved the final version of the manuscript.


The pyogenic granuloma is thought to represent an exuberant tissue response to local irritation or trauma.Clinically these lesions usually present as single nodule or sessile papule with smooth or lobulated surface. These may be seen in any size from a few millimeters to several centimeters. Pyogenic granuloma of the oral cavity is known to involve the gingiva commonly . Rarely it may present extragingivally. Here, we report a case of Pyogenic granuloma in the palate of a 16 years old man which is very rare location for this lesion. Pyogenic granuloma is a relatively common, soft tissue tumor of oral cavity that is belived to be reactive and not neoplastic in nature ,2 the naThe pyogenic granuloma is thought to represent an exuberant tissue response to local irritation or trauma ,4,5,8Clinically these lesions usually present as single nodule or sessile papule with smooth or lobulated surface. -3,5-7 ThThe increased incidence of these lesions during pregnancy may be related to the increasing levels of estrogen & progesterone. -3 PyogenA 16 years old male patient was referred to our department with a chief complaint of a lesion on his hard palate.The lesion was of negligible size when the patient first noticed it (3 months ago), but had grown rapidly over the past 20 days to attain the present size.The patient's medical history was unremarkable. Clinical examination revealed an exophytic, pedunculated lesion that measured 0.7 cm in diameter, and in it's surface pseudomembraneuse with some areas of erythema was seen.The lesion was firm in consistency and non tender fig , with miDue to the relatively small size of the lesion, an excisional biopsy, along with histopathologic evaluation was recommended as the diagnostic approach.The histopathologic examination revealed granulation tissue with non neoplastic proliferation of endothelial cells with blood cells formation and infiltration of acute and chronic inflammatory cells in a few collagenous matrix fig . SurfaceThese findings were consistent with a histopathological diagnosis of pyagenic granuloma.In the oral cavity pyogenic granulomas show a striking predilection for the gingiva, with interdental papillae being the most common site in 70% of the cases. They are more common in the maxillary anterior area than any other area in the mouth. Gingival irritation and inflammation that result from poor oral hygiene, dental plaque and calculus or over-hanging restorations may be precipitating factors in many cases. ,8 PyogenAlthough pyogenic granuloma an be diagnosed clinically with considerable accuracy, radiographic and histopathological investigations, aid in confirming the diagnosis and treatment. Radiographs are advised to rule out bony destruction suggestive of malignancy or to identify a foreign body.All clinically suspected pyogenic granulomas must be biopsied to rule out more serious conditions as mentioned previously. The histopathological picture of the extra gingival pyogenic granuloma is quit similar to the ones occurring on the gingival. Microscopically, it consists of many dilated blood vessels in a loose edematous connective tissue stroma. There is typically a dense acute inflammatory infiltration but this may be scanty or absent. ,5,9Treatment of pyogenic granuloma consists of conservative surgical excision which is usually curative. There is a relatively high rate of recurrence (about 15%) after simple excision.  RecurrenWritten informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal written informed consent was obtained from the patient for publication of this reportThe authors declare that they have no competing interests.MA and FF carried out the clinical study and reviewed the manuscript, followed the patient and collected the documents. NM carried out the histopathologic examinations. PMM assisted in clinical study and drafted the manuscript.


An aneurysmal bone cyst is a benign, but expansile tumor like lesion that generally occurs in the long bones including the vertebral column. An aneurysmal bone cyst arising from the rib, especially in the elderly, is extremely rare.We report a 58-year-old Turkish woman with an aneurysmal bone cyst of the right 3rd rib treated with chest wall resection. The pathologic findings confirmed the diagnosis of aneurysmal bone cyst. The patient has been followed up for 5 years with no evidence of recurrence.En bloc resection can be curative and provide good results for this rare type of chest wall tumor. Aneurysmal bone cyst (ABC) was first reported by Jaffe and colleagues in 1942. They used this term to describe the "blow out" radiographic appearance and blood filled contents of the cystic spaces -5]. We . We 5]. A 58-year-old Turkish woman was admitted to our hospital complaining of pain from a swelling on the right side of her chest for the previous 4 years. She had no history of trauma and her past medical history was unremarkable. Physical examination revealed a 10 cm × 7 cm tender palpable mass in the midaxillary line with otherwise normal findings.Her routine laboratory test results were normal. Posterior-anterior (PA) chest X-ray demonstrated a purely lytic expansile lesion originating from the right 3rd rib with ballooning of the cortex. Computed tomography (CT) of the lesion showed a multiloculated, expanding mass with fluid levels in the right 3rd rib. The mass was protruding into the hemithorax and the adjacent soft tissue Figure . The corThe microscopic examination demonstrated blood filled multiple cavernous spaces separated by fibrous connective tissue, with hemosiderin pigment laden macrophages; multinucleated osteoclastic giant cells and well-developed vascular spaces with hemorrhages in the septa lining the cystic spaces, confirming the diagnosis of ABC Figure .Our patient has been followed up for 5 years with no evidence of recurrence or functional disturbance Figure .Primary rib neoplasms are uncommon. They comprise 5% to 7% of all primary bone tumors ,4. ABC pABC is not a true cyst or aneurysm . The etiThe typical radiographic appearance of ABC is that of an eccentric, lytic, "blow out" type of lesion, demarcated by a thin shell of subperiosteal new bone ,6. ThereOnce the lesion is diagnosed, treatment should be initiated as soon as possible since local extension and rapid growth can cause pathologic fractures, paralysis due to spinal cord compression, compression of the vital organs and malignant transformation ,6. The cABC is a rare, benign pathology, which should be considered in the differential diagnosis of chest wall tumors. Complete surgical excision can be the best treatment for cure.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.MY and HD performed the operation. BK was the major contributor in writing the manuscript. MY performed the histological examination of the mass. ID was the departmental chair.


Malignant esophageal stenosis with complete obstruction and esophagorespiratory fistula (ERF) is difficult to treat with standard endoscopic techniques.We report a patient in whom with local recurrence of esophageal carcinoma an esophagotracheal fistula occurred. Initially the patient had undergone esophageal resection with interposition of a gastric tube. Due to complete obstruction of the lumen by recurrent tumor conventional transoral stent placement failed. For retrograde dilatation a laparotomy was performed. Via a duodenal incision endoscopic access to the gastric tube was achieved. Using a guidewire the esophageal obstruction was traversed and dilated. Then it was possible to place an esophageal stent via an antegrade approach.Open surgery enables a safe access for retrograde endoscopic therapy in patients who had undergone esophageal resection with gastric interposition. Esophageal cancer is an aggressive tumor with unfavorable prognosis. Despite the radical surgery, local recurrence occurs in up to 21% of the cases . The endHowever in patients who underwent esophageal resection and transformation of the stomach into a small gastric tube for esophageal reconstruction, retrograde access is far more challenging, since not even radio guided procedures seem applicable. To our knowledge a suitable therapeutic approach in this difficult palliative situation has not been described before. We recently encountered a patient with local recurrence after esophageal resection and interposition of a gastric tube. Due to complete obstruction and ERF, he required a laparotomy for retrograde passage and subsequent stent placement.A 66-year-old man presented with severe dysphagia, weight loss and recurrent pulmonary infections due to an esophago-tracheal fistula. Due to squamous cell carcinoma of the esophagus 17 month ago, he had undergone esophageal resection and interposition of a gastric tube with cervical anastomosis. This treatment was followed by adjuvant radio-chemotherapy (50 Gy with 5-FU and Cisplatin). The first signs of dysphagia developed 8 weeks before admission to our hospital. Initial endoscopic therapy revealed local tumor recurrence beginning at 21 cm from front incisors, but failed to provide palliation of dysphagia. The distal end of the stenosis could not be measured precisely due to high grade stenosis which could not be passed endoscopically. Though intravenous hyperalimentation was administered, the patient kept on losing weight. Furthermore recurrent pulmonary infections occurred and swallowing of salvia, without coughing became impossible. For palliative surgical treatment the patient was transferred to our institution. Unfortunately we found the esophageal lumen to be completely obstructed by recurrent tumor. Moreover the tumor had invaded the trachea and had caused an esophago-tracheal fistula. The fistula itself could not be seen endoscopically, but was found by gastrographin swallow and CT-scan  declare that they have no competing interests.RA: Reviewed the current literature, drafted the manuscript and made substantial intellectual contributions to the article; CK:Initiated the publication of this case, helped to draft the manuscript and revised it critically; SM:Performed the endoscopic procedures, helped to draft the manuscript and revised it critically. KP: Performed the X-ray examinations and supplied digital artwork. RMA: Performed endoscopic procedures and helped in drafting the manuscript; IÖ: Participated in the design of this article and coordinated and helped to draft the manuscript. KWT: Performed the surgical treatment of the patients, helped to draft the manuscript and revised it critically.All authors read and approved the manuscript.


Primary myofibroblastic sarcoma of the bone is a rare spindle cell tumour with, to the best of our knowledge, only eight cases reported in the available English language literature. The disease's rarity and its low-grade features make an accurate diagnosis difficult in most cases. The differential diagnoses of this unusual tumour include various benign entities as well as other sarcomas. Due to the difference in prognosis, a precise pathologic diagnosis is essential, which requires a combination of thorough morphologic examination, immunohistochemistry and electron microscopy wherever available.We report the case of a 38-year-old Indian woman with a lytic lesion in her left femur. The tumour was associated with cortical destruction and soft tissue extension. A biopsy from the soft tissue component showed features suggestive of a low-grade malignant mesenchymal tumour. Excision of the tumour was performed and histopathological examination showed a low-grade spindle cell sarcoma with collagenous stroma. Expressions of vimentin and smooth muscle actin were also noted. Ultrastructural examination confirmed its myofibroblastic nature. A final diagnosis of low-grade myofibroblastic sarcoma of the left femur was thus rendered.Low-grade myofibroblastic sarcoma is one of the rarer osseous spindle cell sarcomas depicting a favourable prognosis in the cases reported so far. Its diagnosis requires ancillary techniques like immunohistochemistry and electron microscopy. To the best of our knowledge, we report the ninth case in the literature and the first case from our subcontinent. In addition to its role in wound healing, they have been described in soft tissue tumours like myofibroblastoma, angiomyofibroblastoma, myofibromatosis and inflammatory myofibroblastic tumour ,2. Myofiet al. . Primaryet al. -8. To thThe histopathological differential diagnoses of this rare neoplasm include benign myofibroblastic proliferations and sarcomas such as well-differentiated intraosseous osteosarcoma, leiomyosarcoma, fibrosarcoma and malignant fibrous histiocytoma of the bone -11. An aWe describe a case of low-grade myofibroblastic sarcoma occurring in the femur of a 38-year-old woman. This rare entity is briefly reviewed with a discussion of various differential diagnoses.A 38-year-old Indian woman presented in our hospital with a two-year history of swelling and pain in her left thigh and hip. The swelling was progressive in nature. She had no history of trauma prior to the swelling. No significant personal or family history was present. On examination, there was a 20 × 10 cm soft tissue swelling involving the anterolateral aspect of her left thigh and extending to her hip. There was mild tenderness over the swelling and movements at her hip joint were painfully restricted. Results of systemic examination, however, were unremarkable.Routine haematological and biochemical investigations, including alkaline phosphatase, were within reference ranges. Radiological investigations, such as computed tomography (CT) scan and magnetic resonance imaging (MRI), revealed a diffuse, irregular, ill-defined heterogeneous altered marrow signals in our patient's left upper femoral metaphysis and extending into the epiphysis and diaphysis. The altered marrow signals were seen to extend up to the lower shaft diaphysis and metaphysis. Similar signals were also noted in her left pelvic bone (acetabulum and pubic). There was anterior and posterior cortical disruption in her upper femoral metaphysic with extension into the adjacent soft tissues. The altered marrow signals were hyperintense on T2-weighted and short inversion recovery (STIR) images. On the other hand, the signals were low on T1-weighted images  and vimentin [Myofibroblasts have been characterized in the last three decades, as mesenchymal spindle cells sharing features of both fibroblasts and smooth muscle cells. These cells have been shown to play an important role in wound healing . In addivimentin .Histopathologically, myofibroblastic sarcomas (MFS) are composed of slender spindle cells with variable nuclear pleomorphism and mitotic activity. The spindle cells are arranged in interlacing fascicles and have eosinophilic cytoplasm, which may be occasionally wavy. In addition, collagenous stroma is also seen ,6,12. LoPrimary MFS of the bone is an extremely rare neoplasm. An extensive literature search yielded only eight previously reported cases of osseous MFS -8. Of thTwo patients (a 71-year-old woman with iliac bone tumour and a 49-year-old man with tumour in the right maxilla) died with distant metastasis. These two patients showed a high mitotic count (6 to 8 per 10 HPF) and necrosis. However, these histopathological features did not correlate well with prognosis, since some cases with high mitotic activity and marked atypia pursued an indolent course. Both patients who died of their disease were treated by wide resection with radiotherapy and/or chemotherapy. Due to the unpredictable behaviour of their disease, these patients had to be kept under close follow-up, especially because the histopathological features of the disease do not seem to predict accurately its biologic behaviour. The reported cases of osseous MFS are summarised in Table MFS needs to be differentiated from tumours like conventional osteosarcomas, chondroblastoma and solitary fibrous tumour, which may show focal or extensive myoid differentiation. These tumours can be differentiated by their clinicopathological features . AnotherOther neoplasms that enter the list of differential diagnoses include leiomyosarcoma, fibrosarcoma, and malignant fibrous histiocytoma of the bone. Leiomyosarcoma, whether primary or metastatic, can be differentiated by its lack of prominent collagenous stroma and expression of h-caldesmon . FibrosaLow-grade myofibroblastic sarcoma is an unusual osseous neoplasm. It needs to be differentiated from other spindle cell tumours of the bone using immunohistochemical expression pattern and/or electron microscopy. A close radiological and pathological correlation is mandatory to exclude other entities and to make an accurate diagnosis. The biologic behaviour of this rare neoplasm is difficult to predict because only a few cases have been reported so far. Reports of more cases in the literature would help clinicians define this entity better.Written informed consent was obtained from our 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.RA reported the histopathological specimen and collected the clinical data. RG reviewed the literature and drafted the manuscript. AS assisted in drafting and revising the manuscript. AKD was in-charge of signing out the case and critically revised the manuscript. All authors read and approved the final manuscript.


Multicentric myxoid liposarcoma is a rather infrequent tumour that tends to behave aggressively.We herein report two further cases of this tumour that have been managed in our Hospital. Both were young men with multiple sites of involvement at the moment of diagnosis and both have shown a bad prognosis with frequent recurrences after treatment and rapid death in one case.We comment on the diagnosis of this entity and on the therapeutic options available for these patients. Liposarcomas are among the most frequent malignant soft tissue tumours. Most of them arise in the extremities or the retroperitoneum, affect middle-aged and old patients and tend to follow a relatively indolent clinical course with local recurrences after resection and occasional distant metastasis, mainly to the lungs . PrognosA 44-year-old man consulted on pain affecting his left leg. His previous medical record was uneventful with no previous hospital admissions or surgical interventions. The patient reported no weight loss and had no other symptoms rather than pain, which had progressively worsened in the last 2 months and prevented the patient from sleeping at night. The physical examination was unremarkable, but the MRI performed showed a huge soft tissue mass, affecting the adductor muscles of the left thigh and measuring 30 × 20 cm. Radiological diagnosis were liposarcoma or haemangioma. An incisional biopsy of the mass revealed a diffuse growing neoplasm. The predominant areas were of myxoid nature Figure , but theA 35-year-old man consulted on the appearance of a right gluteal mass. He had noticed the mass some months before, but he was not concerned about it for he had considered it the result of the intensive training due to his job as a bodyguard. However, the mass had grown so much that he could no longer sit and he decided to seek medical assistance. The patient was in good general health, had no other symptoms and reported no weight loss. The physical examination confirmed a 40 × 15 cm mass in his right gluteal area and the MRI suggested a possible adipose nature for it. Incisional biopsy showed a similar histological image to that described in the previous case with myxoid and round cell areas. Diagnosis was myxoid liposarcoma and material from the biopsy was sent for cytogenetic analysis and subsequently confirmed the translocation t  typical of myxoid and round cell liposarcomas. During the extension study the CAT showed a 7-cm extrapulmonary mass in the posterior mediastinum. The core needle biopsy showed the same histological image as the gluteal mass. As surgical therapy would imply amputation of the leg including the hip and the pelvis (hemipelvectomy) and the patient had a second mass in the mediastinum, neodajuvant chemotherapy was initiated with iphosphamide, adriamycin and DTIC. The control MRI indicated partial response of the mass to chemotherapy and two months after diagnosis simple resection of the gluteal mass was attempted. The histological analysis of the enucleated mass showed less than 10% of tumour necrosis related to chemotherapy and confirmed the existence of widespread areas of round cells, comprising almost 40% of the tumour. After surgery local radiotherapy was initiated with good tolerance. For one year the patient remained well and apparently disease-free, but 13 months after initial diagnosis he consulted on the appearance of a right laterocervical mass. Fine-needle aspiration suggested an adipose tumour and a cervical lymphadenectomy confirmed involvement by myxoid liposarcoma in one of twelve identified lymph nodes, without any evidence of disease elsewhere. No further therapy was initiated and the patient remained well, but 8 months after this last surgery he suddenly developed abdominal pain and the imaging techniques revealed a 18 cm adipose mass involving the left kidney. The patient was operated again for bulk reduction and underwent wide resection of the retroperitoneal mass including the left adrenal gland and kidney  declare that they have no competing interests.MJFA diagnosed the first case, reviewed the literature on this issue and written the clinical report; PLC was the oncologist in charge of both patients and has written the therapy section; MLF operated both patients and helped in preparation of draft manuscript; TM diagnosed histologically the second case and helped with pathological section of manuscript; and CC established the radiological diagnosis in both cases and helped with radiological section of manuscript. All authors read and approved final manuscript


A 12-years-old girl followed for recurrent urinary infections was admitted to the pediatric department complaining of a 1-year history of constipation and pelvic pain. The child also reported of low spinal pain without any sensorymotor disorder. There were no events of rectal bleeding. Clinical examination revealed a suprapubic sensitivity with no perceptible abdominal mass. Digital rectal examination revealed a spherical, renitent and painless retrorectal mass. However, no intraluminal lesion was found. The anteroposterior and lateral X rays of both the thoracolumbar spine and the pelvis did not show any particularities. The abdominopelvic ultrasonography revealed a right laterouterine cystic mass measuring 4.5 cm in the largest diameter. The pelvic magnetic resonance imaging (MRI) showed an ovoid mass measuring 65 × 50 mm, with a regular wall thickness of 2 mm. The lesion had a hypersignal content in both the T1- and the T2-weighted images, pushing the rectum and the respective neighbouring structures . The uteWhat is your diagnosis?What are your therapeutic options?Rectal duplication with gastic mucosa heterotopy.The surgical exploration through a sagittal posterior access found a retrorectal mass with a thick wall and greenish contents remaining in the meconium. Resection of the mass was performed. The histological examination revealed a digestive cystic duplication with a gastric body type of mucosa and submucosa . The posDuplication of the digestive tract is a rare malformation and the rectal localization represents 1–8% of this pathology. ThereforRectal duplications usually present as a cystic mass in the retrorectal space but anterior localizations are also found. Rectal dThe most frequently reported symptoms are pain and discomfort, constipation, urinary retention, recurrent perianal fistula, perianal infection and more rarely rectal prolaps. The gastic mucosa heterotopy can cause ulcerations and bleedings.24 Cases 2MRI allows precise diagnosis and its exact association. Scintigraphy, using TC99, is another useful diagnostic tool when gastric heterotopy is suggested.–4The appropriate treatment of rectal duplications is a complete surgical excision. It allows complete recovery and prevents potential malignant degeneration.5Rectal duplication with gastric heterotopy is a full entity with variable presentation and complications. The diagnosis is suggested by MRI and confirmed by histological investigation. Total excision is the preferred form of treatment.


Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease (RDD) is an uncommon but well-defined benign self-limited clinicopathological entity. It mainly involves lymph nodes. Extranodal involvement is seen in up to 43% of cases, with the most common location in the head and neck region. Primary RDD occurring in the bone is rare with only twelve cases reported in the literature to date, all diagnosed on histology except one by fine needle aspiration (FNA) cytology. We report a case of RDD diagnosed by FNA cytology in a 28 year-old female presented as an osteolytic lesion of superior pubic ramus where the differential diagnosis included a sarcoma and lymphoma. Based on the cytologic findings, a diagnosis of a RDD was considered during the rapid FNA on site with no clinical history provided. The diagnosis of RDD was further confirmed by immunohistochemical stains and histology diagnosis. Our findings show that even in the absence of a clinical history, FNA is a less invasive and a very reliable tool for the diagnosis of SHML (RDD). Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease (RDD) is a rare but well-defined benign self-limited clinicopathological entity. It commonly involves lymph nodes. Extranodal involvement is seen in up to 43% of cases, the most common location is the head and neck region (75%), where the skin, orbit, nasal cavity and paranasal sinuses are the most common sites.2 PrimaryA 28-year-old woman presented with progressive hip girdle pain for several months and underwent imaging studies which revealed a destructive lytic bone lesion around the right acetabulum and proximal femur involving the superior pubic ramus. The pain was worsening with weight bearing and was controlled fairly well with pain medications. The patient had no significant constitutional symptoms. She denied any weight loss. Review of systems was unremarkable. Her past medical history was also unremarkable.3; platelet count of 244,000 mm3 and erythrocyte sedimentation rate of 50 mm/hr.The patient’s laboratory values at the time of admission included hemoglobin of 12.2 g/dl; hematocrit of 35.3%; white blood cell count of 5,630/mmAn anterior-posterior pelvis radiograph showed an osteolytic bone lesion in the right superior pubic ramus and acetabulum . A compuA CT guided FNA of the right superior pubic ramus was performed using a 22 gauge needle. Air-dried smears were stained with Diff-Quik . The needles from each pass along with remaining FNA samples were rinsed with Hank’s solution and used for making cell block. Rapid interpretation was given based on the finding noted on the Diff-Quik stain. The specimen was hypocellular and revealed varying numbers of large histiocytes with prominent emperipolesis. Those large histiocytes contain numerous phagocytosed intact lymphocytes, neutrophils and rare plasma cells within the cytoplasm . Fine vaImmunoperoxidase stains for antibodies to S-100 protein and CD1a were performed on the cell block. The histiocytes were intensely positive for S-100 protein  and negaA follow-up biopsy confirmed the diagnosis of RDD histologically. Due to progressive pain and concern for a potential future pathologic fracture, the patient was advised to undergo radiation therapy and chemotherapy that she was to pursue in a different city from where she lives. Follow- up had been lost since then.SHML or RDD was first described by Rosai J. and Dorfman R.F. in 1969.10 Extran1811Our findings show that even in the absence of a clinical history, FNA is a less invasive and a very reliable tool for the diagnosis of SHML (RDD).No competing interest to declare by any of the authors.http://www.icmje.org/#author. Each author has participated sufficiently in the work and take public responsibility for appropriate portions of the content of this article.Each author acknowledges that this final version was read and approved. All authors of this article declare that we qualify for authorship as defined by ICMJE As this is case report without identifiers, our institution does not require approval from Institutional Review Board (IRB) .To ensure integrity and highest quality of CytoJournal publications, the review process of this manuscript was conducted under a double blind model (authors are blinded for reviewers and reviewers are blinded for authors) through automatic online system.


Liposarcoma is one of the most common soft tissue sarcoma of adult life, usually occurring in the retroperitoneum and the extremities. Primary liposarcoma of the colon is very rare. The optimal treatment has not been established due to the small number of cases reported. We report a case of primary liposarcoma of the colon presenting as a massive intraluminal lesion.A 79-year-old woman presented with abdominal pain, progressive constipation and weight loss. A CT scan and a colonoscopy revealed an intraluminal mass in the transverse colon and multiple intraperitoneal lesions. The patient underwent surgical resection of the lesions. Pathologic examination was consistent with pleomorphic liposarcoma of the colon.Although no guidelines are available for the management of liposarcoma of the colon, surgical resection should be performed when feasible. Our patient's overall survival was satisfactory in spite of the multiple negative prognostic factors. Liposarcoma is one of the most common soft tissue sarcomas of adult life. Histological classification includes five main subtypes: well-differentiated, myxoid, round cell, pleomorphic and dedifferentiated ,2. LiposA 79-year-old woman, in good general conditions, presented with a 3-month history of episodic abdominal pain and progressive constipation associated with weight loss. Her past medical history and family history were unremarkable. Physical examination revealed a palpable, tender mass in the right upper quadrant of the abdomen. Laboratory tests on admission were within normal values, including tumour markers carcinoembryonic antigen (CEA), CA 125, CA 19.9 and CA 72.4. As a first approach, an abdominal ultrasonography (US) was performed, showing a hypoechoic ovoid mass in the right mesogastrium (max Ø 10 cm) and another hypoechoic round mass (max Ø 4.5 cm) in lumbo-aortic region. A computed tomography (CT) scan showed multiple intraperitoneal lesions: two solid, homogeneous masses located behind the proximal transverse colon, measuring respectively 9 and 6 cm, showing extensive vascularization following contrast injection; a solid homogeneous mass involving the distal transverse colon and left colic angle walls; multiple hypodense lesions with defined borders located in the hepatic hilum, Morrison's space, omentum and para-aortic region. A colonoscopy was performed, showing an intraluminal, yellowish lesion with clear margins in the transverse colon which didn't allow a further progression of the endoscope (Fig. Laparotomy revealed an intraluminal mass at the right colic angle and a dissemination of the tumour at stomach, omentum, mesentery and mesocolon Fig. . Frozen Twenty eight months after surgery, the patient developed progressive dysphagia with radiological evidence of tumour progression. Thirty months after surgery she died for an acute cardiac event. No autopsy was performed.th and 6th decades, and an equal sex distribution [Liposarcomas of the large intestine usually present with nonspecific gastrointestinal symptoms such as bleeding, obstruction, diarrhea, abdominal pain and weight loss. It is thus difficult to distinguish them preoperatively from other colon cancers. Primary colonic liposarcoma tends to occur in adults, with a peak incidence between 5ribution . WHO claribution .The GI tract does not represent a typical location for primary liposarcoma. However it can secondarily be involved as a site for metastases from liposarcomas located in the retroperitoneum and extremities ,15. CaseThe small number of cases reported has not allowed the determination of an appropriate diagnostic and therapeutic approach to this rare tumor. Complete surgical excision should be considered the gold standard. In our case, despite wide local excision was performed, the complete removal of the tumour was technically unfeasible due to the numerous extraluminal lipomatoid lesions. Our patient showed macroscopic evidence of residual disease and was thus candidate to further therapy. The role of chemotherapy in the management of liposarcoma has not been yet well established . ConversPrognosis for colonic liposarcoma is still difficult to predict. It may be affected by a variety of factors, including location, size, dissemination of the disease, as well as histological type ,26. No sOnly a few cases of primary liposarcoma of the colon have been reported and optimal treatment strategies have not been established yet. However, surgery should be performed whenever feasible. The choice of therapeutic options should be based both on the natural history of the disease and on the patient's individual clinical status. The collection of a larger number of cases is needed for the definition of guidelines for the management of this rare tumour.Patient consent could not be obtained as the patient died. Written informed consent was obtained from the patient's son 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.MDA, the operating surgeon, conceived the design, collected data and participated in critical review. MC participated in data collection. RC assembled data and reviewed pathology. ES assembled data, reviewed literature and drafted the manuscript. All authors read and approved the final manuscript.