Peritoneal keratin granulomatosis is a rare condition included under granulomatous lesions of the peritoneum. It can be secondary to neoplasms of the female genital tract and can mimic carcinomatosis intraoperatively. A case of a 40-year-old woman with a history of polycystic ovaries and a chief complaint of vaginal bleeding is presented. She was diagnosed with endometrioid adenocarcinoma with squamous differentiation in endometrial curettings. Intraoperatively, many peritoneal nodules were found, interpreted as peritoneal carcinomatosis. The woman underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy, omentectomy, bilateral pelvic lymphadenectomy, and appendicectomy. Multiple biopsies were taken, as well as peritoneal washings. Microscopic examination revealed multiple keratin granulomas on the serosal surface of the ovaries, fallopian tubes, appendix, and omentum. Lymph node metastasis was not found. Peritoneal keratin granulomas (PKGs) have been reported in cases of endometrioid adenocarcinoma with squamous differentiation of the uterine corpus, ovary, and atypical adenomyoma. It should be noted that the prognosis of cases of peritoneal keratin granulomas without viable tumor cells is favourable and that the histologic examination is essential for its diagnosis. We report a case of PKG in a patient with endometrial carcinoma with squamous differentiation, being the first in a woman with polycystic ovaries.  Peritoneal keratin granuloma is a rare lesion included among reactive tumor-like lesions of the peritoneum. It can be secondary to endometrioid adenocarcinoma with squamous differentiation of the endometrium and ovary and atypical polypoid adenomyoma of the endometrium and in association with ruptured dermoid cysts. The prognostic significance of these lesions is unknown and it seems to have no interference with prognosis, when no viable tumor cells are detected. Here we describe a case of an endometrioid adenocarcinoma of the endometrium, in a woman with polycystic ovaries in which diffuse peritoneal keratin granulomas were found with no viable tumor implants which intraoperatively were misinterpreted as diffuse carcinomatosis.A 40-year-old woman with a body mass index (BMI) of 37 and a past medical history of polycystic ovary syndrome, presented to her gynaecologist complaining of irregular vaginal bleeding. Her menarche was at the age of 16 and her menstrual cycle was infrequent and irregular. Endometrial biopsies (D&C) have been examined at the age of 33 and 38 years. At the age of 38, she was diagnosed with atypical adenomatous hyperplasia of the endometrium and she was put on progestagen therapy. A few months later, she experienced a new episode of irregular vaginal bleeding and after an additional D&C she was diagnosed with endometrioid adenocarcinoma of the endometrium. As a routine pre-op check, tumor markers were requested. Her serum CA125 and serum CA19.9 were elevated to 69.00 U/ml  and 91.60 U/ml , respectively. The magnetic resonance imaging (MRI) of the lower abdomen revealed invasion of more than 50% of the myometrium and of the uppermost uterine cervical stroma. Blurring of the sigmoid fat and prominent inguinal, para-aortic and mesenteric lymph nodes were also described with a maximum lymph node diameter of 1.5 cm. Total abdominal hysterectomy, bilateral salpingo-oophorectomy, bilateral pelvic lymph node dissection, omentectomy, and appendicectomy were performed. Intraoperative peritoneal washings were also carried out. Multiple peritoneal nodules, <0.5 cm in diameter, suspicious of disseminated carcinomatosis, were found during surgery in the pouch of Douglas, over loops of small bowel, and in the mesentery of the small bowel. Multiple biopsies were taken. Due to increased BMI, para-aortic lymphadenectomy was not performed. No frozen section was requested because it was appreciated that a positive result would not affect the overall surgical management.Grossly, the uterine corpus, including both cornua, was filled with a polypoid papillary mass, measuring 11, 5 × 5, 5 cm, extending into the uterine cervix . Both ovHistologically, the tumor of the uterine corpus was a superficially invasive, moderately differentiated, tubulopapillary adenocarcinoma of the endometrium, of endometrioid type with multiple foci of squamous differentiation Figures . ImmunohIntraoperative peritoneal washings showed scattered mesothelial cells, occasional clusters of atypical cells of mesothelial origin, rare anucleate squames, and an occasional keratin granuloma. Overall, the endometrial carcinoma was of UICC/FIGO stage II.Peritoneal keratin granuloma is a rare lesion included under granulomatous lesions of the peritoneum . Such pePeritoneal keratin granulomas refer to the finding of nests of keratinized anucleate squamous cells surrounded by a foreign body type giant cell reaction, either on the peritoneal surface or within subperitoneal connective tissue. These so-called keratin granulomas do not contain any glandular epithelium. The typical histological appearances in previously reported cases were similar to ours.Spontaneous reflux of exfoliated necrotic squamous metaplastic cells or keratin from the squamous element of the endometrial tumor to the peritoneum or its retropulsion through the tubal lumina due to endometrial sampling has been postulated as the pathogenetic mechanism of the 27 cases . The aboThe commonly reported process of keratinization in endometrial carcinomas could be influenced by irradiation, surgical trauma, partial removal of the tumoral mass, hormonal factors, infection, or transfusion, but the aetiology is usually unknown . SometimAfter revision of the literature, only 33 similar cases had been reported till 2012.Tripathy et al. (2010)  and MontChen (1978)  describeThe prognostic significance of keratin peritoneal granulomas with or without viable tumor implants is difficult to assess because of the small number of cases in the literature. Lack of or short follow-up in some cases and postoperative radiotherapy, chemotherapy, or both, which might have influenced the natural course of any postoperative residual peritoneal lesions, makes it more difficult to interpret the real prognostic significance of these lesions. Some authors suggest that they have no prognostic significance when no viable cells are found in the granulomas.The combination of contrast-enhanced T1-weighted and diffusion-weighted magnetic resonance is mentioned to be helpful for the preoperative differential diagnosis .In the current study, we document a very rare case involving a patient with polycystic ovaries syndrome, who presented with a huge endometrial tumor which filled the uterus and protruded through the cervical os. The tumor was an endometrioid adenocarcinoma of the endometrium which was accompanied by multiple peritoneal keratin granulomas attributed to the squamous element of the tumor, transpassing the lumen of the fallopian tubes and eliciting a giant cell reaction. Our findings are in concordance with Chen et al.'s  and WothBecause peritoneal granulomatosis can resemble disseminated carcinomatosis macroscopically, the knowledge of this rare entity is essential to avoid upstaging of the patient. Our patient underwent brachytherapy and whole irradiation and is well after fifteen months of follow-up.Furthermore, the findings of scattered anucleate squames and keratin granuloma in the peritoneal washings constitute the second cytohistologic reference of PKGs .


Tumor-induced osteomalacia is a rare acquired metabolic bone disorder characterized by isolated renal phosphate wasting due to abnormal tumor production of fibroblast growth factor 23. We report the case of a 59 year old woman referred to our department with a long history of progressive diffuse muscle weakness and pain, generalized bone pains and multiple insufficiency fractures of heels, ankles and hips due to a hypophosphatemic osteomalacia. A fibroblast growth factor 23-producing phosphaturic mesenchymal tumor localized in the left quadriceps femoris muscle was identified 7 years after onset of symptoms. Excision of the tumor resulted in normalization of serum phosphate and fibroblast growth factor 23 levels and in complete resolution of the clinical picture with disappearance of all musculoskeletal symptoms. This case illustrates the diagnostic difficulties in establishing a diagnosis tumor-induced osteomalacia and in identifying the responsible tumor. Our case underscores the clinical need to investigate all patients with persistent musculoskeletal symptoms for hypophosphatemia. A systematic approach is of pivotal importance because early recognition and treatment of the metabolic abnormality can prevent deleterious effects of osteomalacia on the skeleton. Tumor-induced osteomalacia (TIO) is a rare acquired metabolic bone disorder occurring as a result of isolated renal phosphate wasting due to abnormal tumor production of fibroblast growth factor 23 (FGF23). Lack of awareness of the clinical manifestations of the disorder often leads to diagnostic delay, failure of diagnosis or misdiagnosis. Once a diagnosis TIO is suspected it is also often difficult to find the responsible FGF23-secreting tumor, since these tumors are often small, slow growing, benign tumors of mesenchymal origin, which can occur almost anywhere in bone or soft tissue –4. SinceT-score −2.2 standard deviations (SD), femoral neck T-score −3.0 SD) and stress fractures of heels and ankles. The patient was also found to have a vitamin D deficiency, low serum calcium and phosphate concentration (respectively 2.13 and 0.45 mmol/L) and elevated alkaline phosphatase (366 U/L). Treatment with phenytoin was discontinued. Supplementation of vitamin D in the form of colecalciferol (vitamin D3) and calcium carbonate was used in a dosage of 400 IE and subsequently 500 mg 3 times per day. Subsequently intravenous treatment with bisphosphonates (pamidronate) was initiated before referral to our center. In view of persistence of complaints, a 99mTc-skeletal scintigraphy was performed, showing multifocal areas of increased radioactive isotope uptake, including at the femoral neck bilaterally, interpreted to be due to multiple insufficiency fractures , computed tomography (CT) and magnetic resonance imaging (MRI), revealed no potential lesion as a source of FGF23 production. The patient was treated with active vitamin D metabolite  and phosphate drink (3dd20 mmol), which partially increased serum phosphate level associated with improvement of symptoms. However, this was insufficient to control hypophosphatemia and associated symptoms in the long-term. There was a clear increase in C-terminal FGF23 (2050 RU/ml) over the following 4 years and it became progressively more difficult to control serum phosphate levels. Her clinical picture also significant worsened. She had difficulty in walking and surgical intervention was required to stabilize the severe insufficiency fractures of her hips. At that stage further radiological evaluation using 111Indium-pentetreotide scintigraphy and also a 18Fluorodeoxyglucose positron emission tomography/CT (FDG-PET/CT) was performed, both demonstrated a suspicious isolated lesion in the soft tissue at the left inguinal region  and vitamin D metabolites. All cases of hypophosphatemia should be further evaluated to establish whether low circulating levels of phosphate are due to renal phosphate wasting, decreased intestinal absorption of phosphate or to redistribution of phosphate from extracellular fluid into cells or into the bone matrix. Hypophosphatemia can also be caused by several medications. The underlying pathophysiological mechanism of medication-induced hypophosphatemia are as mentioned before or resulting from more than one mechanism. Our patient was known with prolonged therapy with anticonvulsants in the form of phenytoin. These drugs are inducers of the cytochrome P450 thereby causing increased vitamin D degradation. They also decrease calcium resorption in the gut . In our 111In-pentetreotide [68Ga-octreotide PET/CT may have an important role in localization of the culprit lesion, and may potentially reduce significant delay in establishing the diagnosis of TIO [The finding of hypophosphatemia in the absence of a family history for bone and mineral disorders, a normal phenotype and relatively recent onset of symptoms which may rapidly progress in an adult with previously documented normal serum phosphate suggest TIO, which may be confirmed by measuring FGF23 levels  depletion in hypophosphatemia can also effect the cardiopulmonary system by impaired myocardial and diaphragmatic contractility and effect the hematopoietic system leading to hemolysis and leukocyte dysfunction, these complications are rare . The seaSurgical intervention is curative when a lesion is identified and fully resected. Radical excision is important to avoid a recurrence. After successful tumor resection, the serum level of FGF23 decreases to a normal level within a few hours to days depending on the magnitude of the initial elevation  . Late reTumor-induced osteomalacia represent a significant diagnostic challenge and should be considered in the differential diagnosis of all patients with musculoskeletal symptoms associated with hypophosphatemia. Association of hypophosphatemia, renal phosphate wasting (low TRP/low TmP/GFR), elevated FGF23 levels and paradoxically low or normal 1,25-dihydroxy vitamin D suggests the diagnosis of TIO after exclusion of the hereditary hypophosphatemic osteomalacia by adult onset of hypophosphatemia in the absence of family history and characteristic phenotype. Awareness of this acquired metabolic abnormality, a systematic approach and evaluation of a hypophosphatemia in a multidisciplinary setting are of pivotal importance because early recognition and treatment can prevent progression of osteomalacia and associated increased risk of fractures. Patients should be prescribed active metabolites of vitamin D with a few requiring additional phosphate supplementation. After the FGF23-producing tumor is located and fully resected, long-term follow-up is advocated for early diagnosis of a potential recurrence.


Buschke–Loewenstein tumor (BLT) is a giant condyloma acuminatum which is very rare and commonly affects the anogenital region. The malignant transformation and localization in penis is very rare. This article aims to present a case with BLT with malignant transformation in penis.A 59-year-old male patient was referred, who has have been suffering from a cauliflower-like lesion in the penis for 8 years. Biopsies revealed a BLT with malignant transformation. The lesion was excised largely and reconstructed with local flaps and skin grafts.Defined by Buschke and Loewenstein in 1925, giant condyloma acuminatum is a rare and important disorder because of the sexually transmitting capability. The Buschke–Loewenstein tumor (BLT) is a sexually transmitted and very rare giant condyloma acuminatum.A 59-year-old male patient was admitted to our clinic with a lesion starting from the penis and growing slowly for 8 years. The patient's human papillomavirus (HPV) and human immunodeficiency virus (HIV) tests were negative and had no known disease. Multiple biopsies were taken from the depressed and pink-red areas, which differed from the lesion in appearance , all of them were reported as the squamous cell carcinoma arising in the condyloma acuminatum of Buschke and Loewenstein . There BLT was described by Buschke and Loewenstein in 1925 as a giant condyloma acuminatum case of penile BLT.In the literature, it is defined that BLT is commonly seen in the anogenital region.The treatment options for condyloma acuminatum include cryosurgery, topical podophyllin, or trichloroacetic acid; however, these treatments are much more convenient for small lesions.Since there is a possibility of malignant transformation, we recommend both radical surgical excision of condylomas in this area without fear and reconstruction with local flaps for penis and skin grafts for the suprapubic regions.Condyloma acuminata is rarely accompanied by malignancies. Also, reconstruction and management of this rare condition is crucial. A functional repair of a condyloma acuminata defect was presented. We think that this work will contribute to the literature.


Meningiomas are the most common primary nonneuroglial extra-axial neoplasms, which commonly present as spherical or oval masses with a dural attachment. Meningiomas without dural attachment are rare and, according to their locations, are classified into 5 varieties, including intraventricular, deep Sylvain fissure, pineal region, intraparenchymal, or subcortical meningiomas. To the best of our knowledge, intraparenchymal meningioma with cerebriform pattern has never been reported. In this paper, we report a 34-year-old Chinese male patient who presented with paroxysmal headaches and progressive loss of vision for 10 months and blindness for 2 weeks. A thorough physical examination revealed loss of bilateral direct and indirect light reflex. No other relevant medical history and neurologic deficits were noted. Computed tomography and magnetic resonance imaging scans showed an irregular mass with a unique cerebriform pattern and extensive peritumoral edema in the parietal-occipital-temporal region of the right cerebral hemisphere. The initial diagnosis was lymphoma. Intraoperatively, the tumor was completely buried in a sulcus in the parietal-occipital-temporal region without connecting to the dura. The histological diagnosis was intracranial meningioma based on pathological examination. Therefore, when an unusual cerebriform growth pattern of a tumor is encountered, an intraparenchymal meningioma should be considered as a differential diagnosis.  Meningiomas are the most common primary extra-axial neoplasms in adults, accounting for up to 30%  of all pA 34-year-old male patient presented with paroxysmal headaches and progressive vision loss for 10 months and blindness for 2 weeks. Physical examination revealed loss of bilateral direct and indirect light reflexes. No other neurologic deficits and laboratory abnormalities were noted. CT imaging showed an irregular isodense mass with a cerebriform appearance and extensive peritumoral edema in the parietal-occipital-temporal region of the right hemisphere . MR imagIn this report, we presented a case of a large intraparenchymal meningioma that likely developed in a long period of time. Despite the significant mass effect caused by its large size and the extensive surrounding edema, no cognitive deficit was detected at presentation, which was likely due to compensation/accommodation to the slow growth of the tumor. However, the patient had lost both direct and indirect pupil light reflexes which may be secondary to optic atrophy resulting in slowly but progressively elevated intracranial pressure.This case showed an unusual growth pattern of an intraparenchymal meningioma which was characterized by its cerebriform appearance and the involvement of cortical sulci without dural attachment. The etiology of meningiomas without dural attachment is unclear. Some authors presume that such meningiomas arise from arachnoid cells of the pia mater that stretches into the surface of brain or sulcus with perforating blood vessels, while others suggest that the arachnoid cells rest in the brain during the migration progress . The menTo the best of our knowledge, only 31 cases of intraparenchymal meningiomas have been reported . This isDue to the absence of typical features on CT and MRI scans, the preoperative diagnosis of this disease is extremely difficult. Our case showed several findings leading to the diagnosis of lymphoma: the periventricular location, an isodense or slightly hyperdense mass on CT scans, and isointensity with intense enhancement on T1WI and T2WI. It has been reported that intraparenchymal meningioma may be misdiagnosed as glioma , metastaGiven their vastly different treatments and prognosis, intraparenchymal cerebriform meningiomas should be differentiated from lymphoma and glioblastoma multiforme (GBM). Brain lymphoma accounts for 1–5% of all brain tumors . It occuIn summary, in this paper, we present the cerebriform imaging characteristics of a rare intraparenchymal meningioma and review the imaging findings that could help us differentiate atypical meningiomas from other types of brain tumors such as CNS lymphoma and GBM. Advanced MRI techniques such as MRS and PWI can effectively improve the preoperative diagnosis of this tumor.


Fetus in Fetu (FIF) is a rare condition where a monozygotic diamnionic parasitic twin is incorporated into the body of its fellow twin and grows inside it. FIF is differentiated from teratoma by the presence of vertebral column. An eight year old girl presented with an abdominal swelling which by X-ray, ultrasonography and CT scan revealed a fetiform mass containing long bones and vertebral bodies surrounded by soft tissue situated on right lumber region. On laparotomy, a retroperitoneal mass resembling a fetus of 585 gm was removed. It had a trunk and four limbs with fingers and toes, umbilical stump, intestinal loops and abundant scalp hairs but was devoid of brain and heart. Histology showed various well-differentiated tissues in respective sites. FIF is a mystery in reproduction and it is scarce in literature in such well-developed stage.


Hepatocellular carcinoma (HCC) is a very common cancer. Curative treatments and local ones are well validated. Sorafenib, a multi-kinase receptor inhibitor was introduced in 2007 for advanced HCC in patients with preserved liver function. HCC is known to be resistant to systemic chemotherapy, and there are no validated therapies improving survival for metastatic disease. Herein, we report a case of a 45 years old woman with chronic hepatitis B infection submitted to a right hepatectomy in May 2001 for an hepatic tumor with more than 10 cm wide, confirmed as a HCC moderately differentiated. Three years later, a solitary pulmonary metastasis was documented and a metastectomy was done. In February 2009, the patient started on sorafenib 400 mg twice daily due to an inferior mediastinal metastasis with a vena cava thrombus associated. Computed tomography (CT) scan done 13 months after revealed a consistently mass reduction in more than 50% and a clinically well patient without important collateral effects. HCC is a highly vascularized tumor and sorafenib is known to inhibit both tumor angiogenesis and tumor cell survival. It is already approved for the treatment of advanced and metastatic renal cell cancer. In our case, the combination of two well done surgical procedures and the posterior use of sorafenib when a metastasis was found in an inaccessible surgical place with macroscopic vascular invasion, led to a long survival without important side effects. Liver cancer is the sixth most common cancer all over the world, which is consistently the third most common cause of cancer death in recent years, with a very heterogeneous distribution worldwide depending on the etiology of the subjacent chronic liver disease , 2. In PClinical, radiologic, biochemical and cytological diagnostic HCC criteria are well recognized and reproducible, and treatment modalities vary according to tumor dimension, number of nodules, localization, liver function and subjacent cirrhosis, disseminated disease, and local resources. Surgical treatment has a curative aim, varying from tumor resection with free margins to liver transplantation. In MELD (Model for End-Stage Liver Disease) era, for allocating patients in a waiting liver transplantation list, a change was necessarily made to those with HCC  , 5. DepeLocal HCC treatments exist as neo-adjuvant or down-staging options, including trans-arterial chemo-embolisation, radio-frequency ablation, percutaneous ethanol injection and trans-arterial radioembolisation, with the aim of reducing tumor dimension and serving as a bridge to surgical resection or transplantation .Increased expression of multidrug resistance transporters and active intracellular metabolism in HCC results in an intrinsic resistance to chemotherapy drugs, conducing systemic chemotherapy as to be historically ineffective -11.Successive stages of liver damage, from fibrosis to cirrhosis and HCC, depend on formation of new vessels and the establishment of an abnormal angioarchitecture. It seems that platelet-derived growth factor (PDGF), transforming growth factor-b1 (TGF-b1), fibroblast growth factor (FGF) and vascular endothelial growth factor (VEGF) play a potent pro-fibrogenic and pro-angiogenic role in liver disease . It was Sorafenib was sequentially involved in phase I, II and III studies -17. A suRecently, 15 cases of complete remission attributed to sorafenib treatment in HCC with extrahepatic spread were reported in Japan though it is rarely reported in other countries, even because there is no clear indication on the use of sorafenib in this kind of patients .We report a clinical case of a patient with chronic hepatitis B infection with metastatic HCC disease controlled with sorafenib treatment.In May 2001, a 45-year-old woman was referred to our center due to a liver mass. She had chronic hepatitis B, diagnosed in 1997 after routine blood test. No viral treatment was done. In May 2001, due to abdominal pain localized at right hypochondria, an abdominal ultrasound was done, which revealed a mass with 10 cm wide in the posterior segments of the right hepatic lobe, and the patient was referred to our center. Computed tomography (CT) abdominal scan confirmed a hypodense mass with peripheral contrast enhancement in arterial phase with portal washout, highly suggestible of a HCC. AgHbs, AntiHbc and AntiHbe were positive. Delta virus was negative, as was hepatitis C virus and HIV1/2. Liver analysis revealed an AST of 155 U/L, ALT of 192 U/L, PAL of 130 U/L, GGT of 170 U/L and α-fetoprotein (AFP) of 60.77 µg/L. The HBV DNA was negative. Coagulation was normal. Her ECOG performance status was 1 and Karnofsky performance status scale of 90%. As it was a unique mass in a non-cirrhotic liver (liver function was preserved and there were no signs of chronic liver disease in imagery), a right hepatectomy was performed. Histology revealed a moderately differentiated HCC with a size of 8 x 10 x 14 cm and a trabecular pattern with an immunocytochemistry revealing negativity for AFP and positivity for CAM 5.2. Remnant liver revealed a chronic hepatitis with mild fibrosis. Transaminases and AFP levels returned to normal values after surgery (3.4 µg/L). No antiviral therapy or any adjuvant systemic chemotherapy was started.Three years later (May 2004), in a routine CT scan, a nodular lesion with 2 cm wide with tissue density in the right inferior pulmonary lobe was found. AFP levels were normal (3.9 µg/L). As it was a unique metastasis, a metastectomy was done. Histology was concordant with a HCC metastasis.The patient remained under surveillance. In January 2007, AFP levels started slowly rising . A more A rising AFP contrasted with the absence of demonstration of any tumor in repeatedly imaging techniques. In December 2008, AFP peaked at 365.4 µg/L. At that time, a dynamic CT identified an inferior mediastinal mass of 3.5 x 3.3 cm associated with a vena cava thrombus. Sorafenib (400 mg twice daily) was started in February 2009  resulting in a decline of AFP values and a reduction of more than 50% of the metastatic mass (15 mm wide in March 2010) and of vena cava thrombus size . AntivirOur patient had a chronic hepatitis B infection, recognized in 1997 due to a disturbed hepatic profile, never treated, and a HCC that developed in a non-cirrhotic liver. HCC surveillance benefit is uncertain in this subpopulation of hepatitis B female carriers younger than 50 years old without cirrhosis . In spitThe HBV can cause HCC, directly by HBV DNA integration in the host genome and by persistent expression of viral proteins (such as HBx and LHBs: Large envelope protein) that are capable of oncogenes activation, inducing oxidative stress and genetic instability, or indirectly due to hepatocellular injury induced by chronic HBV infection and hepatocyte regeneration, conferring an annual incidence of HBV-related HCC in patients with chronic hepatitis B that ranges from 2% to 5% and with a lifetime risk for HCC approximately 10-25% , 23. OurAs the patient had no evidence of cirrhosis and no macroscopic invasion of vascular structures, a right hepatectomy was performed as an intention to treat procedure.By the time when the solitary pulmonary metastasis was found, metastectomy was made, as there was no evidence of liver relapse. Surgical resection of 1 or 2 solitary metastasis is recommended in the absence of intrahepatic HCC, improving survival .Nowadays, AFP is not recommended for HCC surveillance, but it is recognized as a risk factor for HCC . It was In our case, prolonged survival was related not only to a proper surgical approach (right hepatectomy and metastectomy) but also to the use of sorafenib, surpassing life expectancy at the beginning and by the time when metastatic disease was documented.The patient is under sorafenib treatment since 17 months ago, who is clinically well, and with a documentation of more than 50% tumor reduction revealed in a CT scan 13 months after sorafenib was started.Hepatocellular carcinoma is a highly vascularized tumor. Sorafenib in known to inhibit both tumor angiogenesis  and tumor cell survival , and is already approved for the treatment of renal cell advanced and metastatic disease . In JapaThe protracted time course of the HCC in this patient was very uncommon. Sorafenib seems a very exciting therapy for advanced HCC, and may also have very important benefits in particular patients with metastatic HCC. Phase III studies in patients with HCC and metastatic disease should come out quickly to put sorafenib (or other molecule with the same properties) in this context in the everyday clinical field.


Teratomas are the most common germ cell tumors of childhood. Head and neck teratomas, however, account for less than five percent of all teratomas. Considered rare at an incidence of 1 in 20,000 to 40,000 live births, they may occur in the cervical region, nasopharynx, brain, orbit, or oropharynx. Teratoma presenting as an isolated intranasal mass is extremely rare. In this report, we describe a case of a mature teratoma arising from the roof of the nasal cavity presenting as an isolated intranasal mass, the first of its kind from our literature review. The tumor was resected endoscopically with no recurrence detected. Teratomas are the most common germ cell tumors of childhood , consistA full-term newborn boy presented with noisy breathing and mild subcostal retractions at birth. There was no stridor or desaturations on room air. On examination, there were no external nasal deformities or swellings. Bedside nasoendoscopy revealed a soft fleshy mass occupying the left nasal cavity. An attempt to pass a 6-French catheter beyond the mass into the nasopharynx was successful, which suggested that there was no associated choanal atresia. Examination of the right nasal cavity, apart from a septal deviation as a result of mass effect from the left nasal mass, was otherwise unremarkable. The oral cavity and oropharynx were also clear with no evidence of cleft palate.Computed tomography (CT) and magnetic resonance imaging (MRI) scanning of the nose were performed, which revealed a left nasal mass lesion arising from the roof of the nasal cavity with extension into the posterior choana . There wThe patient subsequently underwent an examination of the nose and endoscopic resection of the nasal mass under general anesthesia. Intraoperatively, a large left nasal mass was noted , which ePostoperatively, the patient had an uneventful recovery and was discharged well on postoperative day 3. Final histology confirmed the diagnosis of a mature teratoma, with presence of tissues from all three germ layers , skeletal muscle (mesoderm), and ciliated respiratory epithelium (endoderm)). On follow-up at three weeks and three months postresection, there was no evidence of tumor recurrence on examination under general anesthesia . The patient is currently being followed up every six months.The nasopharynx is a common site of origin for congenital head and neck teratomas, which is often associated with cleft palate formation . In compAn intranasal mass in a newborn often triggers the consideration of a congenital midline nasal mass such as a nasal encephalocele or a nasal glioma . This is2-laser resection of an extensive nasopharyngeal and oral teratoma [As this was an isolated intranasal teratoma, resection was performed via an endoscopic approach. A Skimmer microdebrider was chosen for its small size, which permitted the debulking of the teratoma within the confines of a neonatal nasal cavity. Similar successful cases of endoscopic resection have also been previously described for nasopharyngeal  and nasateratoma .In general, the long-term prognosis of head and neck teratomas following surgery is excellent , as teraIn summary, we report the first case of a congenital teratoma arising from the roof of the nasal cavity that has been successfully resected via an endoscopic approach.


Perineal hernia is a protrusion of intra-abdominal viscera through a defect in the pelvic floor and is a rare but challenging complication after extensive abdominoperineal surgery. There have been small series published after colorectal exenteration, but no cases have been reported after radical cystectomy and urethrectomy.A 68 years old woman developed an anterior perineal hernia, with no vaginal prolapse, after an anterior exenteration for bladder cancer. A perineal approach with the use of a synthetic polypropylene mesh was chosen to resolve the condition. After 6 months of follow-up, the patient has no symptoms or recurrence of the anterior perineal hernia.To our knowledge, this case is the first report of perineal hernia after radical urethrocystectomy. Although being a case report, this article describes a potential and challenging complication after extensive anterior pelvic surgery, that could increase its incidence in the future. Literature review shows that whether perineal, abdominal or combined approach is chosen, surgery must respect hernia repair principles. Perineal hernia is a protrusion of intra-abdominal viscera through a defect in the pelvic floor. Symptomatic perineal herniation is a rare but challenging complication after extensive abdominoperineal surgery. A Mayo Clinic publication, reported a prevalence of 0.34% 8 of 2732) in patients operated on between 1990 and 2000 with a minimum 5-year follow-up  was prescribed. No pelvic irradiation was necessary.A robotic radical cystectomy, hysterectomy with bilateral adnexectomy, extended pelvic lymphadenectomy and urethrectomy with heterotopic Bricker urinary diversion was performed. Due to the locally advanced disease prior to chemotherapy a broad resection was performed including a complete urethrectomy.The postoperative recovery was marked by a delayed vestibule healing and an inguinal hematoma that required surgical drainage. The pathologic examination retrieved a high-grade muscle-invasive bladder urothelial carcinoma, and a final TNM score of pT2 No M0 with negative surgical margins.After one month of the surgery, an anterior perineal bulge appeared during efforts, increasing its volume during the following months. The patient had severe discomfort, despite not complaining of pain or digestive symptoms.Upon physical examination, an anterior perineal hernia was observed between the ischiopubic rami, at the level of the vestibulum with labia majora bulging but without vaginal prolapse . The conA magnetic resonance scan showed a small bowel content at the level of the perineal hernia, without vaginal prolapse . No radiA multidisciplinary meeting was held and, based on the patient's discomfort, possible complications, and after patient's consent, surgery was scheduled.Under general anesthesia, with the patient placed in a lithotomy position with Allen stirrups, broad-spectrum antibiotic provided, an infiltration of the perineal skin with a solution of lidocaine and 1% adrenaline was performed. Sub - clitoridian vestibular and lower portion of the anterior vaginal wall incision. Dissection of the voluminous enterocele and opening of the peritoneum sac .Dissection was extended behind the fat tissue of the labia majora and to the level of the ilio-pubic rami, where two polypropylene 2/0 sutures were placed bilaterally on the inferior pubic ligaments. The peritoneum was closed with a purse-string polydioxanone 1 suture, and reinforcement of 2/0 poliglecaprone interrupted sutures. The urogenital diaphragm and bulbo-spongiosus muscles were then sutured in the midline using 2/0 poliglecaprone interrupted sutures to cover the peritoneum sac.®, Aspide Medical Laboratoire, La Talaudière, France) was placed, fixed to the iliopubic rami with the 2/0 polypropylene sutures and with one arm placed through the transobturator foramen on each side (A transobturator light weight polypropylene mesh (Surgimeshach side C and D. ach side .After a follow-up of 6 months, there was no evidence of recurrent hernia and no scar defect .Perineal hernia is an infrequent but challenging event after extensive abdominoperineal surgery, mainly in the posterior compartment. The publications on this subject are scarce and usually based on single or small case series. Incidence ranges between 0.6% and 7% –6. To ouUsual presentation is that of a soft, reducible mass, along with discomfort and bulging in the perineum, which was the case of our patient. Complications may include skin breakdown, bowel obstruction due to incarcerated small intestine, entero-cutaneous fistula or the extreme presentation of hernia rupture with small-bowel prolapse and/or evisceration .As previously stated, risk factors for perineal hernia  may inclAlthough diagnosis might be evident, the use of imaging studies, as computed tomography or magnetic resonance imaging may provide information on hernia content, soft tissue surroundings and exclude recurrent malignancy before deciding a reconstructive surgery.Based in digestive surgery literature , 3, 8, aThe basic principles of hernia repair must be followed: exposure and mobilization of the hernia sac, reduction of its content, excision of the sac, and repair of the defect. There is no accepted consensus as to the preferred approach, perineal, abdominal or combined.Regardless of chosen surgical approach, the main goal is to close the hernia defect, that can be achieved by reapproximation of the tissues with non-absorbable suture or in poor quality autologous tissues, by using a mesh support –11. WhenIn our case, due to the urinary diversion, the perineal approach was chosen. Furthermore, since the patient had no previous radiotherapy, a polypropylene mesh was used in order to ensure a correct and permanent support of the pelvic floor defect, thus diminishing the risk of hernia recurrence.Although being a rare condition, anterior perineal hernia should be suspected in patients who complain of perineal bulge after extensive abdominoperineal surgery. To our knowledge, this is the first case reported of anterior perineal hernia after radical cystectomy and urethrectomy.Informed consent was obtained from the patient for publication of this article and accompanying figures.


Pleomorphic adenoma directly arising in the neck is thought to originate from heterotopic salivary gland tissue. In this article, we present the case of a 55-year-old female patient with a histologically proven pleomorphic adenoma located at the left mandibular angle, anteriorly to the sternocleidomastoid muscle and posteroinferiorly to the submandibular gland. As the patient also had an ipsilateral thyroid nodule with coarse calcifications, clinical and radiological features suggested a possible level II metastatic lymph node. However, ultrasound-guided fine needle aspiration cytology and postsurgery histopathological examination revealed a pleomorphic adenoma arising from heterotopic salivary gland tissue unrelated to a benign thyroid nodule. In this article, we provide a review of the existing literature on heterotopic salivary gland tissue and related neoplasms and discuss their imaging presentation. Heterotopic salivary gland tissue (HSGT) is defined as normal salivary tissue located at sites other than the major and minor salivary glands. Though HSGT has been described in the temporal bone, lymph nodes, and mandible, thyroid, and parathyroid tissues, its presence in the upper and lower neck is very rare, with neoplasms arising from HSGT in the neck being even rarer –5. To thA 55-year-old female patient, with known sleep apnea and chronic tobacco use, was addressed to the Otorhinolaryngology–Head and Neck Surgery Department of our hospital due to increasing dyspnea upon exertion. Nasofibroscopic exploration revealed leukoplakia on the right vocal cord and bilateral Reinke's edema. On physical examination, a mass located along the anterior border of the left sternocleidomastoid muscle and 2 cm below the left mandibular angle was palpated. The lesion was elastic and not painful, but partly fixed to deeper neck structures.To further assess this lesion, a contrast-enhanced computed tomography (CT) of the neck was performed. It revealed a 23 × 17 × 25 mm solid nodule with coarse calcifications located in the left thyroid gland and an ipsilateral 24 × 25 × 35 mm well-delineated hypodense mass located in the level IIA lymph node group . AlthougThe patient underwent surgery under general anesthesia, and histopathological examination confirmed the FNAC findings. Histopathology indicated the level IIA mass to be an encapsulated classic pleomorphic adenoma with a predominant myoepithelial component, surrounded by clusters of normal salivary gland tissue embedded in fatty tissue . Three rAt the head and neck level, salivary tissue is present in the major salivary glands , minor salivary glands (upper aerodigestive tract), and accessory salivary glands (immediate anterior periparotid region), and this is present in association with branchial cleft anomalies, or more rarely, as heterotopic salivary gland tissue (HSGT). HSGT is defined as salivary tissue outside the major, minor, and accessory salivary glands, with no clinical or histological features of branchial cleft anomalies . Though HSGT is susceptible to the same disorders than those affecting the major and minor salivary glands, namely, infectious or inflammatory disorders, as well as neoplastic diseases. Both benign and malignant neoplasms can occur in HSGT. The literature suggests that pleomorphic adenoma is the second most common neoplasm arising from HSGT after Warthin's tumor . AlthougMost pleomorphic adenomas arise in the parotid glands, in which case the diagnosis is usually straightforward upon imaging. Pleomorphic adenomas have mainly well-defined borders and most often display a myxoid matrix on histopathology examination, with typically high signal intensity on T2-weighted sequences, low signal intensity on T1-weighted sequences, and moderate contrast enhancement. On diffusion-weighted sequences, there is no restricted diffusivity. On CT, the tumors display only minimal enhancement; on US, they are strongly hypoechoic, while being hardly vascularized on Doppler-US. The combination of typical location in the parotid gland, well-defined borders, and abovementioned imaging features enables a correct presumptive diagnosis in most cases. Nevertheless, in the presence of atypical features  or atypical location, US-guided FNAC is recommended prior to surgery. In the current case, the presence of a thyroid nodule with coarse calcifications, along with a hypoechoic mass on US located in the ipsilateral level II lymph node group, suggested a possible malignant tumor with lymph node metastasis. In this case, the difficulty in establishing the correct diagnosis arose from the unusual location of the pleomorphic adenoma, as well as from its association with a thyroid nodule.In this case, US-guided FNAC yielded the correct diagnosis. US, whether with or without FNAC, is generally recommended as the first preoperative radiological examination for any mass of the head and neck regions . DespiteThough the FNAC samples of pleomorphic adenoma of any location are often highly reliable, they prove to be nondiagnostic in the event of complex tumor composition . It is wAlthough pleomorphic adenoma is associated with good prognosis, the treatment of choice is surgical excision with free margins in order to prevent local recurrence and malignant transformation into carcinoma ex pleomorphic adenoma (CEPA), carcinosarcoma  , or metaMultifocal local recurrence of pleomorphic adenomas, a typical feature, is well described in the literature, probably accounted for by close margins, pseudocapsular tears, or tumor spillage upon surgical resection. Hypocellular tumors, younger age at initial presentation, female gender, and tumor recurrence are considered critical factors for an increased risk of recurrence . The recEctopic pleomorphic adenoma is one of the differential diagnoses to be taken into account when facing any level II cervical mass with well-defined borders at imaging, hypoechoic and hypovascular US features, and with minor but homogeneous contrast enhancement at CT. As FNAC is a fairly accurate preoperative procedure for the diagnosis of pleomorphic adenoma, it should be carried out under US guidance to confirm the diagnosis.


Malignant gastrointestinal neuroectodermal tumor (GNET) is an extremely rare entity that was first described by Zambrano et al. in 2003 as “Clear cell sarcoma-like tumor of the gastrointestinal tract”. It shares some of the histological features of clear cell sarcoma (CCS) but lacks the immunohistochemical reactivity for melanocytic markers. We report a case of GNET that was initially misdiagnosed as gastrointestinal stromal tumor (GIST). Recognizing this entity is important to avoid misdiagnosis.A case of an 18-year-old male presented with a small intestinal tumor. Histologically it was characterized by polygonal cells arranged in pseudoalveolar pattern and situated in the muscularis propria. Scattered osteoclast-like multinucleated giant cells were also noted. The neoplastic cells were positive for S-100 protein and negative for HMB-45, Melan A, smooth muscle actin, desmin and CD117. EWSR1 gene rearrangement was detected by fluorescence in situ hybridization (FISH) analysis. The patient returned with recurrence after 36 months’ management by surgical resection and died one year later.GNET can be mistaken histologically for other non-epithelial gastrointestinal tumors. Awareness of its existence and diagnostic criteria by the pathologist is necessary to avoid misdiagnosis, particularly as GIST, CCS or malignant peripheral nerve sheath tumor (MPNST). Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLTGT) or as recently designated, malignant gastrointestinal neuroectodermal tumor (GNET) is an extremely rare and controversial entity . It was An 18-year-old college student was referred from the University Clinic for having low hemoglobin (Hg 4.7 g/dl). He was completely well till one month back when he presented with easy fatigability, postural dizziness, palpitation and dyspnea. He gave history of 30 kg weight loss over the past 6 months.On physical examination, he looked pale, not jaundiced or cyanosed. His vital signs were all within normal limits, except for an elevated heart rate (98/min). Cardiovascular, respiratory and abdominal examinations were unremarkable. Primary investigations in our hospital revealed low hemoglobin level (5.1 g/dl), low iron (<10 ug/dl), and a positive occult blood test. A provisional diagnosis of microcytic hypochromic anemia for further workup was made.Bone marrow aspirate and trephine biopsy revealed normocellular marrow, depicting normal trilineage hematopoiesis. Upper GI endoscopy showed erosive antral gastritis with patchy ulcerative inflammation. Abdominal ultrasound showed a slightly enlarged liver with a rounded echogenic lesion in the anterior wall of the right lobe suggestive of hemangioma. The spleen was slightly enlarged and normal in echogenicity with no focal lesions.Computed Tomography (CT) scan of the abdomen and pelvis showed a fairly large well defined soft tissue mass located in the anterior upper pelvis and engulfing jejunal loops causing bowel wall thickening. The patient underwent an exploratory laparotomy and excision of the jejunal mass. Macroscopic examination revealed an 11 × 8 × 2 cm annular mass located within the jejunal wall ulcerating through the mucosa and extending to the serosal surface.Microscopic examination revealed a tumor situated in the muscularis propria and extending to the mucosa and the serosa. The neoplastic cells were arranged in predominantly pseudoalveolar pattern Fig. . They weFluorescein in situ hybridization (FISH) analysis for EWSR1 break apart probe on paraffin-embedded tumor showed evidence of a 22q12 rearrangement in 197 out of 205 (96%) of interphase nuclei scored. The native state of EWSR1 break apart probe will be seen as two adjacent or fused (overlapping) red/green (yellow) signals. However, EWSR1 gene rearrangement presented as one red and one green separated signal Fig. .The patient did not receive chemotherapy or radiotherapy. During the clinical follow up, the patient remained disease free for 3 years until he presented with local recurrence. The treatment plan was surgical resection but the patient sought medical advice in a different institution where he died of the disease a year later.GNET tends to occur mainly in young to middle-aged adults. The patients present with abdominal pain, weight loss, intestinal obstruction or an abdominal mass. Some patients have metastatic disease at the time of diagnosis. Most of the cases have been described in the small intestine in addition to the stomach and the colon , 3, 5. TGNET is centered in the wall of GI tract with extension to the mucosa and/or serosa. The tumor cells grow in solid sheets with pseudopapillary, alveolar and nest formation. The neoplastic cells are predominantly epithelioid with oval or round nuclei with variable amount of eosinophilic or clear cytoplasm but a case featuring an oncocytic cytoplasm has been reported. Fascicles of spindle cells have been also described. The nuclei display irregular nuclear contour. The nucleoli are inconspicuous but occasionally prominent and basophilic. Mitotic activity is variable and ranging from 0 to 20 mitoses per 10 HPF. Necrosis and surface ulceration can be seen. Osteoclast-like multinucleated giant cells is a frequent and consistent finding in most of the tumors. Rosette-like structures and myxoid stroma have been described. Metastatic tumors resemble the primary tumor in morphologic features including the presence of osteoclast-like multinucleated giant cells , 7, 9. OImmunohistochemically, the neoplastic cells are characterized by strong and diffuse staining for S100 protein. They also stain with SOX10. Variable reactivity is described with CD56, synaptophysin and neuron-specific enolase. All cases reported were uniformly negative for HMB-45, Melan-A, tyrosinase and MiTF. They also did not express GIST markers . Desmin and SMA were also negative. Epithelial markers were negative except in one case that expressed CAM 5.2, but only focally and weakly [Of the few cases examined ultrastructurally, no melanocytic, myoid or any other specific differentiation was revealed. Instead, the neoplastic cells showed slender or bulbous interdigitating cell processes in addition to dense-core secretory granules , 2.Antonescu and his team studied 3 cases of CCS of gastrointestinal tract and claimed to be the first to describe a recurrent translocation of EWS 22q12) and CREB1 (2q32.3) resulting in EWS-CREB1 fusion. They concluded that these cases may represent a gastrointestinal neuroectodermal tumor that express neuroectodermal markers and lacks melanocytic differentiation. However, the existence of rare cases of CCS of the gastrointestinal tract with EWS-ATF1 gene fusion that also lack melanocytic differentiation argue in favor of common histiogenesis between the two tumors . In one 2 and CRESome authors regard this entity as CCS in gastrointestinal tract and explain the lack of melanocytic differentiation as being a variant of CCS especially after detecting EWSR1 rearrangement which is involved in CCS , 17. OthThe differential diagnosis of GNET includes GIST, melanoma, CCS, MPNST and synovial sarcoma. GIST needs to be excluded first because of the location of the tumor and histologic features. GISTs feature spindle and/or epithelioid cells which makes the distinction from GNET difficult. However, GIST is not characterized by osteoclast-like multinucleated giant cells and it expresses CD117, DOG-1 and CD34 immunohistochemically. Clear cell sarcoma and melanoma need to be considered because of some shared histological features with GNET and S100 protein reactivity. The melanocytic markers in this situation are invaluable to rule out these diagnoses. Cytogenetic study for EWSR1 may not help to separate GNET from CCS but electron microscopy may have a role in revealing the melanocytic differentiation which is not present in GNET. MPNST can enter in the differential diagnosis because of similar histology (spindle and/or epithelioid cells) and S100 protein reactivity. However, MPNST usually doesn’t show strong and diffuse reactivity for S100 protein seen in GNET and it lacks the genetic translocation involving EWSR1 gene .In conclusion, GNET is an extremely rare malignant tumor of the GI tract that can be mistaken for other non-epithelial GI tumors. Awareness of its existence and diagnostic criteria by the pathologist is necessary to avoid misdiagnosis, particularly as GIST, CCS or MPNST.


Total colonic aganglionosis occurring together with malrotation is a rare occurrence and may pose diagnostic and management dilemmas for the paediatric surgeon. We report a case of a neonate that presented with extended total colonic aganglionosis and malrotation, along with a spectrum of central nervous system and renal abnormalities. The clinical and radiological features and potential diagnostic and management pitfalls are discussed along with a literature review of this exceptionally infrequent association.  Hirschsprung's Disease (HD) and malrotation can arise together in a small proportion of cases [1]. Symptomatic malrotation has been reported to occur in 1 in 6000 live births. However, malrotation may be an incidental finding in up to 1 in 500 live births during surgery or radiological investigations [2]. Malrotation is frequently diagnosed in association with other congenital pathologies of the bowel, diaphragm and abdominal wall [2]. The incidence of HD is 1 in 5000 live births. Total colonic aganglionosis (TCA) accounts for approximately 10% of cases of HD, and some of these patients have aganglionosis extending into the small bowel. Patients with extended aganglionosis encompass a rare and severe phenotype and present a difficult surgical challenge with higher rates of morbidity and mortality [3]. We recently managed a neonate presenting with bilious vomiting for whom the diagnosis of malrotation was made radiologically and surgically. This ultimately led to a significant delay in definitive diagnosis and appropriate surgery for the true obstructive bowel pathology. This case illustrates an important lesson in the surgical management of neonatal bowel obstruction.A female neonate was born at 36+4/40 weeks gestation with a birth weight of 2.76 kg by caesarean section for breech presentation to a 15 year old primigravida mother. Antenatal anomaly scanning at 25 weeks gestation detected a cystic abnormality of the left kidney. The patient had passed a small amount of meconium within the first 24 hours of life. She presented to our centre on day 4 of life with feed intolerance and bilious vomiting. Clinical examination revealed moderate abdominal distension but was otherwise unremarkable. An urgent upper gastrointestinal contrast study (UGI) was diagnostic for malrotation . The patient subsequently underwent emergency laparotomy which confirmed malrotation without volvulus. The bowel was otherwise normal in appearance and a Ladd procedure was performed. The post-operative course was complicated by failure to establish feeds, total parenteral nutrition (TPN) dependence and ongoing large volume bilious aspirates from the nasogastric tube. Associated anomaly workup during this period revealed bilateral optic nerve hypoplasia, bilateral peri-sylvianpolymicrogyria, absent septum pellucidum and septo-optic dysplasia on MRI brain and polycystic left kidney with ectopic left ureter on renal ultrasonography. Parents were non-consanguineous and genetics workup was normal. Ongoing obstructive symptoms  beyond the 3rd postoperative week including feculent vomits and failure of spontaneous bowel opening prompted further evaluation for alternative pathology including a rectal suction biopsy which revealed an absence of ganglion cells on calretinin staining, indicating a diagnosis of HD. On Day 40 of life a second laparotomy was undertaken. Following adhesiolysis (from the previous Ladd procedure) a calibre change at the mid-small bowel was noted. Levelling frozen section biopsies confirmed total colonic aganglionosis extending into the small bowel to 50cms from the ileocecal valve. Resection of the aganglionic small bowel and subtotal colectomy to the level of the distal sigmoid was performed and an end ileostomy was formed at 140cm from the Duodeno-Jejunal (D-J) flexure.The subsequent postoperative period was complicated by wound dehiscence, sepsis and stoma necrosis. On Day 47 of life, the patient underwent refashioning of her end ileostomy and wound closure. Recovery was further protracted as a result of high stoma losses. She was gradually weaned off TPN and eventually established on full enteral feeds by Day 70 of life. She is currently under regular surgical and medical review while being cared for at home and will be managed with an ileostomy for the foreseeable future.The present case was a challenging scenario for us where a diagnosis of malrotation had obscured the true causative pathology as HD. The obstructive symptoms at presentation were due to TCA and malrotation was probably an incidental finding. But in the setting of bilious vomiting with evidence of malrotation on UGI, laparotomy was mandatory. While many cases of bilious vomiting are ultimately not due to obstructive bowel pathology and a finding of malrotation may be incidental, the surgeon must be conscious of the association of malrotation with other congenital gastro-intestinal anomalies to avoid misdiagnosis. Confirmation of malrotation but exclusion of volvulus at laparotomy compels a thorough inspection of the entire bowel to detect atresia or transition zone in cases of HD to allow appropriate intervention at the same sitting [2]. In our case the extended small bowel aganglionosis, which is a rare phenomenon, was not apparent at laparotomy due to failure of the bowel to distend. The passage of meconium within 24 hours of birth and the intra-operative findings assuaged the suspicion of other intestinal anomalies and delayed the diagnosis of HD.Prolonged intestinal obstructive symptoms following Ladd procedure without volvulus and failure of spontaneous bowel movement, prompted us to investigate for alternative diagnoses and perform a rectal biopsy. At subsequent laparotomy this directed our management towards levelling biopsies where we may have otherwise just undertaken an adhesiolysis. Failure to identify the need for a rectal biopsy would have further protracted the diagnosis with increased morbidity and warranted further operative intervention. Importantly, of 15 cases of TCA with associated malrotation reported in the literature, patients had both asymptomatic malrotation incidentally diagnosed and malrotation with volvulus presenting as an emergency [4-8]. Even when evidence of other congenital bowel anomalies is present, it is necessary to remain vigilant to the evolving clinical picture, as malrotation remains a dangerous entity in the setting of HD.In conclusion, the following learning points are proposed: In neonatal bilious vomiting or bowel obstruction one must keep in mind the association of other anomalies such as HD with malrotation; In cases of HD and especially for TCA occurring in association with malrotation, the clinical examination as well as the radiological and surgical findings can be non-specific; A high index of suspicion is necessary for prompt diagnosis and early management of both malrotation and HD; Persistent intestinal obstructive symptoms following a Ladd procedure, particularly in the absence of volvulus, is a red flag and warrants an early rectal suction biopsy, prior to consideration of a subsequent laparotomy.Source of Support: NilConflict of Interest: Nil


Chondrosarcomas are a heterogeneous group of malignant neoplasms that arise from bones, cartilage or other soft tissues that produce cartilage and are commonly seen in the middle decades of life. Despite being the most common primary bone sarcoma in adults, chondrosacromas are rare in pediatric patients.We report the case of a six-year-old child with a painless enlarging sternal mass of which biopsy was consistent with low-grade surface chondrosarcoma. This is the first reported case of a chest wall chondrosarcoma in a young child. This unusual location in a young patient presented challenges to treatment. Resection of the manubrium was performed by a multidisciplinary team of orthopaedic oncology and pediatric general surgery. The patient underwent a wide resection of the sternal mass from an anterior approach performed by the orthopaedic oncology team using an oscillating saw under video-assisted thoracoscopic surgery to ensure adequate mass resection without injury to nearby structures. The patient was followed with quarterly physical exams and radiographs for 18 months postoperatively and did not have any pain or evidence of recurrence.Clinicians should consider utilizing multidisciplinary approaches to treat patients with chondrosarcomas of the chest wall. Chondrosarcomas are a heterogeneous group of malignant neoplasms that arise from bones, cartilage or other soft tissues that produce cartilage and are commonly seen in the middle decades of life , 2.They most commonly involve flat bones of the pelvic and shoulder girdle, however approximately 15% present in the chest wall with annual incidence of 0.5 per million .They are rarely seen in young patients and to our knowledge no reports exist of a chondrosarcoma of the chest wall in a young child. A series of 89 patients with primary chest wall chondrosarcomas had a median age of 55 years and with the youngest being 15 years of age .Chondrosarcomas provide unique challenges to treatment. While intralesional curettage has been shown to be effective for low grade lesions; intermediate or high grade tumors typically require wide excision -7. This We present a unique case of a six-year-old with chondrosarcoma of the sternum successfully treated with wide resection with video-assisted thoracoscopic surgery (VATS).The patient and family provided informed consent for the publication of this case report.1). He was neurovascularly intact in bilateral upper extremities. The patient was otherwise healthy and the remainder of physical exam including cardiopulmonary auscultation was unremarkable.A six-year-old male was referred to the orthopaedic oncology clinic after initially presenting to a general surgeon regarding a mass on the left aspect of the manubrium. According to the patient’s parents, the mass was present since the child was two years old. It started as a pea-sized prominence and enlarged gradually. The patient denied any associated pain. There was no known history of trauma or injury. He denied any fevers, chills, sweats, or shortness of breath. At presentation, there was a three-centimeter palpable mass overlying the left side of the manubrium. This area was non-mobile and non-tender to palpation . Based on the patient’s history, physical exam, and imaging studies the differential diagnosis included osteochondroma, surface chondroma, or periosteal chondrosarcoma. On serial monitoring radiographs and a repeat CT scan eight months after presentation, the lesion was found to be gradually enlarging. At that time, after a discussion with the patient’s family a decision was made to perform an open biopsy of the lesion through a direct anterior approach.Plain radiographs demonstrated a mass that extended into the soft tissues anterior to the manubrium with partially ossified edges. A computed tomography (CT) scan showed a bony exostosis arising from the left anterior aspect of the manubrium with a cartilage cap and internal mineralization. No underlying marrow changes were noted  demonstrated a lesion with heterogeneous internal enhancement without an associated vascular component. The mass was seen extending posteriorly with abutment of the left thymus and great vessels . Given the histology as well as the aggressive features on MRI there was concern for an intermediate grade lesion, therefore a complete resection was recommended. Pediatric general surgery was consulted given the location of the lesion to ensure the deep structures posterior to the sternum were not compromised during resection.The patient underwent open biopsy of the sternal mass under general anesthesia. It was performed 4) shows the chest wall after resection and Fig. (5) shows the resected specimen. The location and size of the defect did not require reconstruction. A chest tube was placed by the pediatric surgery team and patient was admitted for monitoring. His postoperative course was uncomplicated. The chest tube was removed on post-operative day two and he was discharged home in stable condition on post-operative day three.Imaging demonstrated the mass was adjacent to the great vessels and internal mammary artery therefore safe resection required dissection of soft tissues prior to osteotomy. Wide resection of the lesion was performed under general anesthesia. Pediatric general surgery performed a left thoracoscopic dissection and mobilized the posterior portion of the mass away from the internal mammary artery and thymus. Next, the patient underwent a wide resection of the sternal mass from an anterior approach performed by the orthopaedic oncology team using an oscillating saw under direct thoracoscopic visualization; this ensured adequate mass resection without injury to nearby structures. Fig. (6). Given the complete excision, no adjuvant treatment was recommended. The patient was followed with quarterly physical exams and radiographs and as of 18 months postoperatively has no pain or recurrence noted on a most recent chest CT scan . Per National Comprehensive Cancer Network guidelines, the frequency of surveillance will decrease with time, but should be 10 years at minimum [Final pathology demonstrated neoplastic cartilage surrounding pre-existing trabecular bone with associated osteonecrosis consistent with low grade chondrosarcoma with microscopically negative margins and closest being at 2 millimeters from the superficial skin (Fig. This case report presents a six-year-old patient with a painless low grade chondrosarcoma of the sternum that progressively enlarged over four years. Chondrosarcomas are typically seen in adults. Although previous studies have evaluated primary chondrosarcomas of the chest wall, none have included patients under the age of 15 , 11. FurThe pediatric general surgery team performed a thoracoscopic mobilization of the posterior aspect of the tumor combined with resection of the manubrium. This unique approach allowed excellent visualization of both the anterior and posterior portions of the tumor ensuring adequate resection without injury to surrounding structures. Given that only four centimeters of the manubrium was removed and the sternum remained intact, a reconstruction was not necessary. The patient has done well post-operatively with no functional limitations or pain. Previous published reports have used five centimeters as the maximum amount of chest wall that can be resected without reconstruction , 9, 12.Chondrosarcomas of the chest wall are rare and few studies have been performed evaluating optimal treatment. These lesions are often treated by general or thoracic surgeons who are not in larger sarcoma centers, which may lead to variability in management. Due to the challenges of establishing the correct diagnosis, it is important to utilize a multidisciplinary approach for treatment of each patient with a team of experienced radiologists, pathologists, medical oncologists and orthopaedic oncologists. A Scandinavian study evaluated 106 chest wall chondrosarcomas and found that approximately 60% were treated at general hospitals as opposed to sarcoma centers. Patients treated at non-sarcoma centers had higher rates of recurrence and lower survival .This case report presents a painless chondrosarcoma in a young patient. The VATS allowed lesion resection with minimal morbidity and with adequate margins. Clinicians should consider utilizing multidisciplinary approaches to treating patients with chondrosarcomas of the chest wall.


Undifferentiated pleomorphic sarcoma is extremely rare in the head and neck area, and the aggressive nature of this tumor has not been previously described.Therefore, we report a unique case of a very rapidly growing primary undifferentiated pleomorphic sarcoma in the eyelid. A 67-year-old woman presented with a left lower lid mass. She had no previous history of surgery, skin malignancy, or radiation to the eyelid. On initial presentation, the patient showed a 2 × 1 × 1 cm firm, yellow nodule in the left lower eyelid.We planned complete surgical removal of the mass; however, the patient was lost to follow-up. One month after the initial visit, the mass had markedly enlarged to 3 × 2.2 × 2.3 cm and a new 2 × 2 × 2 cm mass was observed in the left lower eyelid.Complete surgical resection of the tumor was performed with a myocutaneous free flap. At that time, two masses were emerged, which had grown to 8 × 8 × 5 cm.The patient died from sepsis caused by pneumonia 2 months after surgery without evidence of local recurrence.Primary undifferentiated pleomorphic sarcoma in the eyelid is extremely rare. Surgeons should be aware of this abruptly presenting, rapidly growing primary eyelid tumor and it is essential to excise the tumor completely as soon as possible. Furthermore, after the term MFH was completely replaced by undifferentiated pleomorphic sarcoma in the 2013 WHO soft tissue sarcoma classification, there has been no report about primary eyelid undifferentiated pleomorphic sarcoma. Here, we report a case of extremely rare and very rapidly growing primary undifferentiated pleomorphic sarcoma originating from the lower eyelid over a period of 6 weeks.Sarcomas are malignancies that arise from transformed cells of mesenchymal origin. High grade pleomorphic malignant tumors of the soft tissue that do not differentiate into specific histologic features are classified as undifferentiated pleomorphic sarcoma, previously known as malignant fibrous histiocytoma (MFH). After the World Health Organization (WHO) classification of soft tissue tumors was published in 2002, an extensive literature review identified only few articles describing cases of primary undifferentiated pleomorphic sarcoma of the eyelid.2A 67-year-old woman presented to the out-patient clinic with a 3-month history of a left lower lid mass. The patient had a 20-year history of type 2 diabetes mellitus that required insulin, with micro- and macro-vascular complications such as chronic kidney failure and diabetic retinopathy. She was on hemodialysis and also suffered from arterial hypertension and carotid artery disease. She was being treated with amlodipine, valsartan, ferrous sulfate, bisoprolol, clopidogrel, and acetylsalicylic acid. She reported no previous history of trauma, surgery, skin malignancy, or radiation to the eyelid.The clinical examination showed a firm and yellow nodule adherent to the adjacent tissue, resembling a sebaceous cell carcinoma in the medial part of the left lower lid Fig. A. We plaFour weeks after the initial visit, she visited our hospital again complaining of markedly enlarged lower eyelid mass Fig. B. A magnHistological findings with immunomarkers led to a definitive diagnosis of undifferentiated pleomorphic sarcoma of the lower eyelid. The tumor was composed of fascicular and haphazard arrangement of spindle and pleomorphic cells with eosinophilic cytoplasm and many typical and atypical mitoses. Frequent multinucleated giant cells were present Fig. A. The tuNo metastatic lesions were detected on a positron emission tomography/CT scan. The patient and her son refused further radiation therapy. Two months after the operation, the lesion was stable without evidence of a local recurrence; however, she died from sepsis caused by pneumonia.This study was approved by the Institutional Review Board of Chonnam National University Hospital. It was in accordance with the principle of the Helsinki Declaration II. Because she died, the written informed consent was obtained from legal guardian.3 Furthermore, the category of MFH was completely deleted from the 2013 WHO classification of soft tissue sarcoma. In the head and neck, undifferentiated pleomorphic sarcoma is extremely rare and accounts for only about 3% of all undifferentiated pleomorphic sarcomas. After the 2002 WHO classification, there has been few articles published about a primary undifferentiated pleomorphic sarcoma of the eyelid.–3 Hence, our report represents an extremely rare case of a rapidly progressing primary undifferentiated pleomorphic sarcoma of the eyelid after 2002 and 2013 WHO classification.The 2002 WHO soft tissue sarcoma classification regarded the alternative name of old nomenclature MFH to undifferentiated pleomorphic sarcoma because it provided a more accurate description of the origin of the tumor cells. According to up to date available immunohistochemical techniques, many previously diagnosed MFH tumors have been reanalyzed and only one-tenth of the cases have been rediagnosed as MFH.3 volumed yellow nodule on the lower eyelid. Four weeks after initial visit, she visited again complaining of a marked increase in the size of the previous mass and the appearance of a new round lower eyelid mass. The tumor's volume increased approximately 12 times over just 4 weeks. Furthermore, 6 weeks after the initial visit, 2 masses had emerged and grown to 100 times larger than at the initial visit. Therefore, this is the first report specifically describing the very aggressive nature of undifferentiated pleomorphic sarcoma of the eyelid according to the time passes. The patient underwent complete surgical excision and reconstruction with a free flap of latissimus dorsi myocutaneous graft; however, she died from sepsis caused by pneumonia even though the lesion was stable without evidence of local recurrence.Our case involved a 67-year-old woman who initially presented to our hospital with a 2 cm,9 Neoadjuvant chemoradiation treatment have been investigated at the extremity and retroperitoneal tissue sarcomas during the past 2 decades, however, similar approaches for head and neck sarcomas have been investigated less. Adjuvant radiotherapy is advised for undifferentiated pleomorphic sarcoma. This is based on the findings of previous randomized trials that surgery plus adjuvant radiotherapy reduces the local recurrence rate compared with surgery-only group.,11 However, previous studies have shown that adjuvant radiation therapy does not increase overall survival. Chemotherapy is usually reserved for metastatic cases. The most commonly used anticancer drugs are cyclophosphamide, vincristine, and adriamycin. Trials evaluating the advantages of chemotherapy did not help to detect differences in overall survival. In addition, trials often enrolled patients with tumors of various sizes, grades, histologic subtypes, and location, and used different chemotherapy regimens. Therefore, the role of chemotherapy in undifferentiated pleomorphic sarcoma is very limited. In our case, negative intraoperative margins were obtained; therefore, the lesion was stable until she died 2 months after the operation.The primary management of undifferentiated pleomorphic sarcomas in the head and neck is complete surgical resection. The type of surgical resection can be decided by various aspects, which consist of tumor location, size, depth, invasion of adjacent structures, requirement for reconstruction, and the patient's general condition. The excision contains the skin, subcutaneous tissue, and soft tissue or bone adhere to the mass. Positive resection margin is an important predictor of local recurrence and disease-related mortality. Positive results for S-100 and Melan-A stainings can be valuable clues with regard to the differential diagnosis of this tumor from melanoma. In addition, CD30 or CD45 staining by immunohistochemistry provides helpful clues to differentiate undifferentiated pleomorphic sarcoma from lymphoma. Judicious use of additional immunohistochemistry can facilitate the identification of the origin of tumors to rule out pleomorphic leiomyosarcoma , pleomorphic rhabdomyosarcoma (desmin), pleomorphic liposarcoma .,12,15 In our case, some tumor cells were weakly positive for EMA, however, they were negative for cytokeratin, smooth muscle actin, desmin, S-100, melan-A, CD34, etc. Therefore, we could exclude such entities and finally diagnosed as undifferentiated pleomorphic sarcoma. These are summarized in Table The differential diagnosis of undifferentiated pleomorphic sarcoma includes sarcomatoid carcinoma, melanoma, anaplastic large cell lymphoma, and other high grade pleomorphic sarcomas such as leiomyosarcoma, liposarcoma, and rhabdomyosarcoma. The appropriate immunohistochemical cocktail usually resolves any diagnostic dilemmas in the distinction from carcinoma, melanoma, and lymphoma. Sarcomatoid carcinoma can be distinguished from undifferentiated pleomorphic sarcoma by the positive staining result for cytokeratin. On the other hand, trunk and extremity tumors were 73%, which indicated better prognosis than head and neck undifferentiated pleomorphic sarcoma. This result could be explained by higher grade tumors and difficulties to obtain negative tumor margins due to adjacent important structures in the cases of head and neck cancer. At initial presentation, size larger than 5 cm, high histologic grade, presence of metastases were related to a poor prognosis.–21 In our patient, the tumor was greater than 5 cm and high histologic grade, however, since she died at 2 months after operation due to sepsis originated from pneumonia, there are limitations for analyzing prognostic factors.According to the literature, the 5-year overall survival rate for head and neck undifferentiated pleomorphic sarcoma was known as 50% to 60%.,3 In the previous case report series, all cases underwent local excision as initial treatment and additional wide resection with adjuvant radiotherapy were used for subsequent local recurrence. While all of these cases were high grade and had local recurrence, it is notable that no patient has died as a result of the tumor until several years after operation. This could also be a result of the anatomic location of periocular undifferentiated pleomorphic sarcoma, which leads to earlier presentation, as compared with undifferentiated pleomorphic sarcoma arising in deeper soft tissue.In our case, periocular undifferentiated pleomorphic sarcoma was small, nodular, and located superficially at initial presentation similar to the previous reports. The tumor was located at the medial aspect of lower eyelid. Previously reported eyelid MFH did not show a tendency of location at the eyelid .Our case is important for several reasons. First, this is an extremely rare case of primary undifferentiated pleomorphic sarcoma of the eyelid that was progressing very rapidly as we presented with time passes. Second, this case highlights the necessity of a prompt diagnosis and immediate surgical intervention if clinically suspected. Therefore, it is necessary to keep the possibility of rapid progression in mind for plastic surgeon and ophthalmologists in the cases of clinically suspected as undifferentiated pleomorphic sarcoma.


In type 1 multiple endocrine neoplasia (MEN1), esophageal diseases association with excessive gastrin secretion in Zollinger-Ellison syndrome (ZES) sometimes develop. Here, we reported a case of MEN1/ZES, who developed dysphagia due to reflux esophagitis with severe esophageal stricture. Treatment for his esophageal stricture and ZES was discussed.MEN1 mutation. Repetitive endoscopic esophageal dilatation with intralesional corticosteroid injection, coupled with pancreatoduodenectomy were performed to improve the patient’s symptoms and to treat pancreatic tumors. The histology of multiple tumors in the duodenum and pancreas were all consistent with neuroendocrine tumors. His hypergastrinemia subsided and he remained asymptomatic in his gastrointestinal tract after these treatments.A 43-year-old man with progressive dysphagia and diarrhea was referred to the teaching hospital. He had a history of recurrent duodenojejunal perforations despite the anti-secretory medication. Blood examinations revealed elevated serum gastrin, calcium, and parathyroid hormone. Upper gastrointestinal endoscopy demonstrated a severe esophageal stricture, multiple gastroduodenal ulcer scars, and a duodenal submucosal tumor. Enhanced computed tomography showed multiple hypervascular tumors within the pancreas and duodenum, suggestive of MEN1. Genetic examination demonstrated a pathogenic For esophageal stenosis in case of MEN1/ZES, anti-secretory therapy and endoscopic dilatation with corticosteroid injection could be recommended. However, in refractory cases with repetitive and/or severe complications due to high acid secretion, surgical treatment could be considered as an option. MEN1 gene is recognized in 80–90% of familial cases and in about 65% of sporadic cases [Type 1 multiple endocrine neoplasia (MEN1) is an autonomic dominant hereditary syndrome that carries a lifetime risk of developing an endocrine neoplasm in ≥95% of cases. Clinical diagnostic criteria require two or more of the following three items: 1) hyperparathyroidism; 2) pancreatic endocrine neoplasm; and 3) pituitary neoplasm. Germline mutation of the A 43-year-old man visited the nearest hospital for examination of his increasing complaints of nausea and diarrhea for 4 years and recent development of dysphagia. He had a history of duodenal ulcer perforation, and underwent omental patching surgery 4 years earlier, followed by oral treatment with a PPI (30 mg/day of lansoprazole). Octreotide analog was not used. Upper gastrointestinal endoscopy (UGE) demonstrated reflux erosive esophagitis with severe esophageal stricture. Plain computed tomography (CT) showed a urinary tract stone and a pancreatic mass. Despite continuous anti-acid therapy, perforation of the small intestine developed, and he underwent closure surgery. Using the selective arterial secretagogue injection (SASI) test, a response by calcium injection was obtained when examined from the superior mesenteric artery, which connects to the feeding arteries to either the pancreatic head, body, or tail. However, it was not obtained by the examinations from the gastroduodenal artery and splenic artery.The patient was referred to the study hospital to further investigate the suspected diagnosis of MEN1. Blood examination revealed an elevated level of serum gastrin , glucagon , calcium  and intact-parathyroid hormone (PTH) . Enhanced CT demonstrated multiple highly vascular lesions within the pancreas (head to tail) and duodenum, with up to 20 mm in the pancreas   of triamcinolone acetonide  was injected to the stenotic portion at the 6th-8th procedure. In detail, 0.5–1.0 ml of triamcinolone acetonide solution was injected into 5–8 sites within the stenosis using an injection needle  each after the balloon dilation. The patient’s symptoms and stricture had disappeared by the 15th procedure, and the 16th dilation was performed at the patient’s request  Fig.  was repend and 3rd esophageal dilation procedures. The histology of the multiple tumors in the pancreas (4) and duodenum (1) was entirely neuroendocrine tumor (NET), with histological grade 2 (G2). PNETs were recognized in all parts of pancreas . Immunostaining of gastrin was strongly positive within the duodenal tumor, but only faintly recognized in the pancreatic tumors , which was employed in the on-going institutional project [Genetic counseling  was comp project , and wasThis case of MEN1 with pancreaticoduodenal gastrinomas, accompanied with a high level of serum gastrin , developed severe esophageal stenosis and complained of dysphagia, despite administration of full-dose PPI. He also had repeated histories of duodenojejunal perforations. To treat this condition, repetitive esophageal endoscopic dilations were performed for local therapy and resection of the pancreaticoduodenal NETs for removing the causative lesion of acid hypersecretion.TM) is the first step to treat strictures. Cases with mild to moderate stricture usually need only one to three repetitions of dilation [Benign esophageal stricture can occur in various disorders in adults. For mild to moderate levels of strictures, peptic injury and Schatzki’s ring or web  can be cdilation , 9 to redilation , 9. In sdilation , lesionadilation , 8, 11 idilation , 9, 10. dilation , 13), pedilation , 12, 14)dilation , can occIn the recently published guideline for the management of PNET , surgicaMEN1 mutation confirmed this genetic syndrome. This information was beneficial not only for the patient but also for his family [The patient in this case was first suspected of having MEN1 based on the clinical findings of multiple peptic lesions accompanied by high levels of serum gastrin, calcium, and intact PTH, pancreatic and duodenal tumors suspected for NETs, and a urinary tract stone. However, it could not be confirmed until surgery and genetic testing. Confirmation of a germline s family , especiaIn conclusion, MEN1-ZES can develop severe esophageal strictures, which can be treated by anti-acid therapy and repetitive endoscopic balloon dilation. Cautious stepwise procedures with intralesional corticosteroid injection facilitated safer esophageal dilation. Additional surgical resection of NET needs to be considered in severe refractory cases. The desirable extent of surgical resection is arguable in cases with multiple pancreaticoduodenal NETs, including functional ones.


To our knowledge, highly detailed findings of flexible hysterofiberscopy in patients with adenofibroma have not been described. A 75-year-old nulliparous asymptomatic woman presented with a uterine polyp, which exhibited punctate heterogeneous hyperintensity or islands of isointense-to-hypointense signals on T2-weighted magnetic resonance imaging (MRI), hypointense signals on T1-weighted images (T1WI), and a little enhancement on contrast-enhanced T1WI. Flexible hysterofiberscopy revealed a red-pink polyp with a white-yellow, cobblestone-like surface easily deformed by perfusion fluid. The tumor was diagnosed histologically as an adenofibroma. Total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed. The tumor in the uterus was necrotic macroscopically and histologically, and a residual adenofibroma could not be confirmed. At present, two years after surgery, the patient remains healthy. MRI and hysterofiberscopy can reveal the histological features of uterine adenofibromas and be useful for their diagnosis. Adenofibroma is an extremely rare benign biphasic neoplasm classified as an epithelial and mesenchymal tumor. Only approximately 30 cases have been reported to date  1–18]. –18. 1–18A 75-year-old nulliparous woman was referred to our hospital by a private internal medicine clinic for an asymptomatic intrauterine mass. She had been treated for type 2 diabetes mellitus and cholelithiasis for 20 years. The mass was detected on abdominal ultrasonography at the clinic. Transvaginal color Doppler ultrasonography at our hospital revealed a polypoid mass, measuring 3 × 2 cm, in the uterine cavity, and consisting of multiple low echogenic cysts that differed in size without pulsatile blood flow . The patEndometrial adenofibroma was first described in 1959 as a benign form of mixed mesodermal tumor . MoreoveTo date, approximately 30 cases of adenofibroma have been reported in the literature (considering only English publications)  1–18]. –18. 1–18Although attempts have been made to diagnose adenofibroma using ultrasonography, CT, MRI, hysteroscopy, and/or biopsy, these methods were successful in only three patients , 16, 17.Although endometrial adenofibromas are benign lesions, total hysterectomy is recommended, because these neoplasms may recur if incompletely curetted or locally excised , 5. HystYoung women with adenofibroma may be given the option of lesion removal under hysteroscopic visualization, allowing retention of the uterus and reproductive potential. Surgical excision of adenofibromas in two patients, including one who underwent operative hysteroscopy with wide local excision, was successful, with no evidence of recurrence . RecurreThe tumor in our patient was well demarcated, with no evidence of invasion into the myometrium on MRI. Necrotic tissue, likely the adenofibroma, was present in the uterine cavity after hysterectomy. Transcervical exeresis may have caused twisting of the tumor, resulting in necrosis. The tumor could be completely removed after hysterofiberscopy. The uterus did not contain any residual adenofibroma tissue, but the endometrium contained an endometrial polyp. Because the pathological morphology of these tumors differed completely, their causal relationship could not be determined.Flexible hysterofiberscopy and MRI are also useful for the diagnosis of adenofibroma, which formed a polypoid mass in the uterine cavity.


Pachydermodactyly is characterized by asymptomatic, progressive swelling of the lateral aspects of the 2nd to 4th finger along the proximal interphalangeal (PIP) joint without involving the joint itself. We present 2 interesting cases of patients with periarticular swelling who were initially diagnosed and treated as juvenile idiopathic arthritis (JIA) with subsequent clinical and pathology confirmation of pachydermodactyly. These cases emphasize the importance of considering pachydermodactyly in young patients with development of periarticular swelling and no joint involvement. Pachydermodactyly is a rare, benign, acquired digital fibromatosis characterized by asymptomatic, progressive swelling of periarticular soft tissues of the fingers without joint involvement. The diagnosis is usually made clinically; however, histopathology will demonstrate coarse collagen bundles and dermal mucin deposition. The etiology is believed to be mechanical trauma in most cases. Treatment is often not indicated given its benign prognosis.A 17-year-old young man was seen in the pediatric rheumatology clinic for investigation of presumed JIA polyarthritis affecting his hands. The patient described the gradual appearance of discrete “swellings” of his fingers beginning 5 years earlier, without pain, or significant physical limitations. He reported mild morning stiffness of short duration and fatigue in the hands after prolonged writing. He was otherwise well, and a review of systems was unremarkable. Prior to the rheumatology assessment, he was seen by his pediatrician who diagnosed JIA and initiated NSAID treatment. On physical exam, a nodular swelling was noted adjacent to the PIP and to a lesser extent the metacarpophalangeal (MCP) joints of both hands . There wThe radiograph of the hands revealed soft tissue swelling concordant with the clinical presentation, but no joint space narrowing or erosions. Magnetic Resonance Imaging (MRI) with gadolinium of the hands did not reveal signs of synovitis. Complete blood count (CBC), antinuclear antibody (ANA), rheumatoid factor (RF), and inflammatory markers were all normal.As the presentation was not typical for JIA, a biopsy of a nodule was performed to clarify the diagnosis. Histological examination revealed coarse dermal collagen bundles with perieccrine collagen deposition, increased fibroblasts, reduction in elastic fibers, and scant perivascular lymphocytic inflammation . FurtherA diagnosis of pachydermodactyly was provided and dermatology was consulted for further management. During the subsequent 4 years, the patient remained well with no change in the condition. As the patient was concerned about the appearance of his hands, a trial of hydroxychloroquine was then initiated, but discontinued after 1 month due to a side effect of vertigo. One year later, he remained well with no change in the condition.A 14-year-old girl was referred to pediatric rheumatology for investigation of a one-year history of “joint swelling” of the fingers in her right hand. She described some mild discomfort when opening and closing her hand, but no morning stiffness or other physical limitations. She had occasional arthralgia of her knees with activity, but was otherwise well, and a review of systems was unremarkable. There was a significant family history of rheumatoid arthritis. Physical exam revealed a nodular prominence, with mild tenderness, in the area of her PIP and DIP joints of the right hand . She hadInvestigations including CBC, ANA, RF, thyroid testing, and inflammatory markers were all normal. The radiograph of the right hand revealed soft tissue swelling, but no joint space narrowing or erosions. A subsequent MRI with gadolinium of the hands was of poor quality due to motion artifact, but was reported as showing possible synovitis, resulting in a preliminary diagnosis of polyarticular JIA. The patient was then started on a NSAID and methotrexate therapy.Despite six months of treatment, she reported no change in her symptoms or the appearance of her hand, prompting reconsideration of the arthritis diagnosis. Subsequently, a biopsy of one of the nodules was performed. Histology showed increased mucin deposition in the full thickness of the dermis, strongly positive for alcian blue and colloidal iron staining Figures  and 6. TPachydermodactyly is an uncommon type of digital fibromatosis. Typically, it presents in adolescent boys as swelling of the soft tissue involving the lateral aspects of the PIP joints particularly of the second to the fourth fingers. Involvement of other joints including the DIP and the MCP, consistent with our patients' clinical appearance, has also been described . In certThe treatment of this condition is not well-defined and few studies examining treatment options have been published. Elimination of mechanical stimulation with occupational therapy has been shown to be helpful . Oral trHere, we have described 2 additional cases, who were initially referred for possible JIA prior to clinical and histopathological confirmation of pachydermodactyly. JIA is also a relatively rare condition in which rheumatoid nodules may develop over MCP and PIP joints in polyarticular disease. A key distinguishing feature is joint involvement in JIA. Other differential diagnoses include self-healing juvenile cutaneous mucinosis and acral persistent papular mucinosis. Self-healing juvenile cutaneous mucinosis is less likely given the lesions persisted in our patients. While acral persistent papular mucinosis has similar histopathologic findings, the primary skin lesions are papules rather than the nodules seen in our patients. While milder cases of pachydermodactyly can be observed and patients can be counselled to avoid any possible aggravating factors, both patients were concerned about the appearance of their hands. Consequently, we proposed treatment with hydroxychloroquine given its anti-inflammatory and antiproliferative actions. As the pathogenesis of pachydermodactyly is driven by fibroblastic proliferation and resultant collagen deposition, we feel there is some rationale to this approach . One of Pachydermodactyly is a rare condition which can mimic the appearance of arthritis. Careful attention to the physical exam, particularly to distinguish between an intra-articular and a cutaneous process, is important to facilitate arrival at the correct diagnosis and avoid potentially unnecessary investigations. More research is needed to determine the optimal treatment choice for this rare condition.