Adenomyomatosis of the gallbladder (adenomyomas or adenomyomatous hyperplasia) is relatively uncommon tumor or tumor-like lesions that are found in 2% - 5% of all cholecystectomies. They may involve in the fundal localization as a second frequency and have a predilection for the middle and elderly-aged women. Adenomyomas are histologically characterized by epithelial proliferation and muscular hyperplasia of the gallbladder. The chronic irritation is mostly aetiological factor and rarely has malignant potential. In this paper, the authors present the clinicopathological features of three cases with adenomyomatosis of the gallbladder and discuss under the light of current literature because of the rarity of these conditions.Gallbladder; Adenomyomatosis; Fundal variant Adenomyomatosis of the gallbladder (adenomyoma or adenomyomatous hyperplasia) is a reactive, hamarthomatous malformation or non-neoplastic tumor-like lesion of gallbladder . It is mAdenomyomatosis has three morphological types according to the localization in the gallbladder wall: (a) segmental type is termed when the lesion is annular and separating the two compartments of the gallbladder; (b) fundal type is defined if the lesion is localized on the base of the gallbladder through the lumen in a hemispheric shape; (c) diffuse type is termed if it causes thickness in the gallbladder wall. Histologically, it is characterized by hyperplasia of the muscular layer and proliferation of the mucosal glandular structures , 13.Herein, we describe three cases with the macroscopic and microscopic characteristics in addition to the diagnostic and differential diagnostic criteria of fundal type gallbladders adenomyomatosis as it is a rarely encountered entity.In this study, the clinical and histopathological characteristics of two patients presenting to our university hospital, and of one patient presenting to a training and research hospital with the diagnosis of gallbladder adenomyomatosis were evaluated. The two cases were female, and were 54 and 73 years old, respectively. The lesion was located in the fundus of gallbladder in all cases. The reasons for admission were dyspepsia, abdominal pain, nausea and right flank pain that had been present for seven months to two years. The common finding in the physical examination was right upper abdominal pain. The examination findings of the other systems, hematological and biochemical parameters were unremarkable. Stones of variable numbers were detected in the gallbladder by abdominal ultrasonography. In one patient, a 1 x 1 cm mass with regular margins was detected, which protruded into the lumen in the fundus of the gallbladder, in addition to cholelithiasis. Two patients complaining of acute abdominal pain and nausea were clinically and radiologically diagnosed as cholelithiasis in emergency department of our university hospital. The other case was diagnosed as cholelithiasis and adenoma of the gallbladder via abdominal examination and ultrasound findings in the research and training hospital. Laparoscopic cholecystectomy was performed on all the patients.On macroscopic examination in all the cholecystectomy materials, gray-dull colored gallbladders were observed. When the gallbladders were dissected, a dark-green colored fluid and numerous yellow-brown gallstones of 0.3 - 1 cm diameter were demonstrated. The gallbladder lumens were smooth and the wall thickness was 0.2 - 0.3 cm. In two patients, a lesion of 0.7 cm diameter, and in one patient, a lesion of 1 cm diameter protruding into the lumen in the form of hemispherical bulging in the fundus of the gallbladder were detected. The sectional surfaces of these masses were gray-white colored and consisted of microcystic structures .On microscopic examination, within a background of connective tissue, bundle-arranged smooth muscle cells, and numerous glandular lobules floored with columnar epithelial cells with nuclei located at the base were examined. Some of these glandular structures were cystically dilated and irregularly shaped, and no mitotic figures, atypical or pleomorphic changes were demonstrated . ImmunohIn the series of Atsusi et al, an incidence of 34.5% was found for the fundal type of adenomyomatosis which was ranked second in the gallbladder, with the segmental type as 63.5%, and 2% for the diffuse type. The mean age of patients was 55.4 years and the range was 22 to 84 years . A few cThere are no symptoms in adenomyomatosis of gallbladder except for a vague abdominal pain. The co-existence with cholelithiasis is another reason for its clinically silent characteristic and incidental detection after cholecystectomy , 4, 13. Most cases with adenomyomatosis of the gallbladder are diagnosed preoperatively using radiological investigations. Ultrasonography is the method of choice as it is inexpensive and practical. Dilated intramural cystic glands which are seen as artifacts and echogenic focuses causing focal or complete thickening in the gallbladder on ultrasonography are important findings for the diagnosis and are accepted as satisfactory for the preoperative differential diagnosis , 13, 21.Fundal type adenomyomatosis is seen macroscopically as an intraluminal hemispheric mass in the fundus of the gallbladder. Section surfaces are hard and consist of gray-white tissue and between these, dilated cystic glands. Histological diagnosis is easy; it has distinguishing microscopic characteristics from other lesions. The cases with adenomyomatosis of the gallbladder have combinations of proliferated glandular lobules, cystically dilated structures, and smooth muscle cell bundles in the stroma which was composed of connective tissue. The proliferated glands are floored with cuboidal or cylindrical epithelial cells between dense interlacing smooth muscle cell bundles , 13, 15.Fundal type adenomyomatosis is usually medically treated if the diagnosis has been made radiologically. If there is no response to medical treatment, cholecystectomy is the treatment of choice , 4, 14. In conclusion, the macroscopic and microscopic assessments of cholecystectomies which were performed for cholelithiasis in routine clinical studies are usually predictable. However, incidental fundal type adenomatosis may also be seen. Recognition of this rare entity is important because the similar characteristics with the adenoma and carcinoma of the gallbladder may confound the surgeons. These lesions should be kept in mind due to their rare potential of developing into benign or malignant tumor.


Clinically curable adrenal metastasis is rare. We report a case of synchronous solitary adrenal metastasis from rectal cancer in a 51-year-old man who underwent curative resection. A right adrenal mass was found by ultrasonography during his routine physical examination and this was confirmed by computed tomography (CT). His serum carcinoembryonic antigen (CEA) level was found elevated, and colonoscopy revealed a rectal tumor located 10cm from anal verge. A simultaneous laparoscopic right adrenalectomy and anterior resection for rectal carcinoma was performed. Histopathological examination revealed well-differentiated rectal adenocarcinoma with adrenal metastasis. The patient is still alive and free from disease 6 years after the surgery. A review in the literature showed that synchronous solitary adrenal metastasis from colorectal carcinoma is very rare. Surgical resection and for selected patients, laparoscopic procedure may provide survival benefit and potential surgical cure for a solitary metastasis. Solitary and clinically curable adrenal metastasis from colorectal carcinoma is very rare.3 Here, we present a long-term survival case with rectal cancer and a synchronous solitary adrenal metastasis who underwent radical surgical resection.Distant metastases to organs such as liver and lung are common in colorectal cancer patients. Approximately 20 percent of patients have liver metastatic disease at the time of presentation.The patient was a 51-year-old man. A right adrenal mass was found by ultrasonography during his routine physical examination in December 2006. Computed tomography (CT) scan confirmed a moderately enhanced mass in the region of the right adrenal gland, which was 3.3×2.0 cm in size . FurtherPostoperative pathological examinations showed that the rectal tumor, which measured 4.0×3.0 cm, was a well-differentiated adenocarcinoma that infiltrating to the adventitia layer, with metastasis to two of three dissected lymph nodes. The pathological findings of the right adrenal tumor revealed infiltrating adenocarcinoma within the normal adrenal gland, compatible with metastasis from the rectal carcinoma .The patient’s postoperative course was good; he did not develop adrenal insufficiency and was discharged 14 days after surgery. His serum CEA level normalized to 4.5ng/ml one month postoperatively. Totally, he received 12 cycles of adjuvant chemotherapy with FOLFOX4 schedule. At last follow-up, in January 2013, he showed no evidence of recurrence or metastasis with a normal CT scan and serum CEA, 6 years after resection of his rectal cancer and synchronous solitary adrenal metastasis.4 Metastatic adrenal disease is usually part of a disease disseminated to several organs and has limited survival. Solitary adrenal disease is infrequent and always in a metachronous setting. Some of the reported cases include patients who had other distant metastases before the adrenal metastasis; strictly speaking, such patients might not be considered to have a solitary metastasis. By performing a Medline literature search, we were able to identify 16 previous reported cases of resection of solitary adrenal metastasis from colorectal carcinoma . The primary carcinomas were mostly in rectum (10 cases), others were in sigmoid colon (3 cases), ascending colon (2 cases), descending colon (1 case) and cecum (1 case).5,6 However, it does not provide any distinction between primary lesions and secondary lesions. Magnetic resonance imaging (MRI) is reported effective for distinguishing adenomas, carcinomas, and metastases in case of adrenal incidentaloma.6 Recently, A few reports and studies in the literature have suggested that fluorine 18-fluoro-deoxy-glucose positron emission tomography (18F-FDG PET-CT) is useful to differentiate benign from malignant adrenal lesions, with sensitivity ranging from 93 to 100%, specificity ranging from 80 to 100% and accuracy ranging from 92 to 100%.7 Although PET-CT was not performed preoperatively in our patient, we think it is useful to exclude metastatic disease elsewhere prior to a potentially curative adrenal resection. Candel et al.8 emphasized that fine-needle aspiration biopsy of adrenal masses was helpful for making an accurate diagnosis. However, in our opinion, this risky technique in characterizing adrenal masses, may eventually be assumed by the less invasive and more accurate PET-CT.In all cases, patients with metastatic adrenal tumor were asymptomatic, without pain or adrenal insufficiency. All metastases were diagnosed by CT scan or elevation in CEA levels synchronously or in follow-up surveillance. Serum CEA level is a useful indicator for the presence of adrenal metastases. In most of the cases, including ours, CEA levels were elevated at the time of adrenal metastasis and decreased considerably after adrenalectomy. Only in one case (case 16), CEA level remained within the normal range. CT scan is a cost-effective means of identifying adrenal metastases. The findings of CT in our patient disclosed the low density of the adrenal gland, in accordance with reports by other authors.It is thought that there are several routes by which colorectal cancer can metastasize to the adrenal glands, including systemic venous, portal venous, arterial and lymphatic routes. The 4 latent liver metastases  and 2 latent lung metastases  4-12 months after adrenalectomy might suggest that the route from primary lesion via liver or lungs to systemic venous circulation and silent liver or lung metastases had already occurred at the time of detection of the adrenal metastases. In contrast, there were 3 patients, including ours  had no other metastases detected during the long-term (>5 years) follow-up.The follow-up period of the 17 patients after resection of adrenal metastases ranged from 8 months to 9 years. Five patients died of recurrence with extensive metastases to multiple organs 24-48 months (median 33 months) after adrenalectomy. One patient showed liver recurrence 8 months after adrenalectomy, but was still alive at the time of report. Two patients presented metachronous bilateral adrenal metastases, after operations, they were alive without metastatic and recurrent disease at the time of report. Nine patients were alive without signs of recurrence 8 months-9 years after adrenalectomy (median follow-up period of 14 months). In the 6 synchronous adrenal metastases patients, 4, including ours, were alive without signs of recurrence at the time of report. These data suggest that resection of solitary adrenal metastasis from colorectal carcinoma may improve patients’ chances of survival. Our long-term survival case also suggests that synchronous solitary adrenal metastasis may respond well to radical surgical resection, chemotherapy and vigilant follow-up.9 Recently, several reports have described long-term survival after laparoscopic adrenalectomy for solitary adrenal metastasis.10,11 However, no prospective randomized studies have compared open versus laparoscopic resection of solitary adrenal metastasis, because it is difficult to recruit patients. In our case, since the adrenal mass was relatively small (3.3×2.0 cm) and no signs of invasion of the surrounding organs or lymph nodes, we performed laparoscopic approach. It suggests that laparoscopic adrenalectomy for small solitary metastasis is a feasible procedure.With technological advances, laparoscopic adrenalectomy is now considered as the gold-standard approach for small benign secreting tumors, but it remains a matter of debate for large and potentially malignant adrenal tumors and adrenal metastases.In conclusion, synchronous solitary adrenal metastasis from colorectal carcinoma is very rare. On the basis of our case and international literature, we believe that surgical resection and for selected patients, laparoscopic procedure may provide survival benefit and potential surgical cure for a solitary metastasis.


The present study describes the findings from three cases of peripheral primitive neuroectodermal tumors (PNETs) diagnosed using computed tomography (CT) or magnetic resonance imaging (MRI). The patients were all diagnosed with PNETs of the peripheral central nervous system. All the lesions were soft-tissue masses with cystic degeneration. The CT images revealed that the lesions were large and inhomogeneous, with unclear borders and cystic degeneration. The surrounding tissues and structures were compressed against each other. Following the enhancement of the CT, the solid components of the tumors were enhanced, whereas the cystic components did not show enhancement. Following the enhanced MRI, irregular enhancement was noted in the solid components and the cystic and solid masses showed unclear borders. The surrounding tissues were compressed. The solid components of the tumors were enhanced, whereas the cystic components showed no enhancement. Based on these observations, PNETs were diagnosed. Thus, a CT or MRI is mandatory for the precise diagnosis of a peripheral PNET. A peripheral primitive neuroectodermal tumor (PNET) is a rare disease that was first recognized by Arthur Purdy Stout in 1918 and is classified within the family of small round cell tumors . SeveralA 51-year-old male with a mass in the abdominal cavity was referred to the Yantai Yuhuangding Hospital . Following a CT examination, a plain scan revealed cystic and solid masses in the abdominal cavity with large low-density areas. The largest mass measured ~19×9×13 cm. The masses involved the mesentery, omental bursa and retroperitoneum, with uneven density and a CT value of 19–46 HU . SubsequA 53-year-old female with precordialgia and chest tightness was referred to the Yantai Yuhuangding Hospital. No obvious abnormality was identified on the clinical laboratory tests. Using a CT examination that was performed following hospitalization, a plain scan was performed on the right side of the pericardium to obtain an image of the cystic hypodense shadow, in which a homogenous density, a thicker cyst wall and calcification were observed . SubsequA 26-year-old male with exophthalmos was referred to the Yantai Yuhuangding Hospital. The MRI examination revealed a mass at the base of the anterior cranial fossa involving the right orbital, sphenoid and pituitary glands. The mass invaded the intracalvarium, in which the four ventricles were deformed by compression. The mass displayed a T1-weighted image (T1WI) signal, compared with the muscle, which represented an area of bone destruction or bone marrow replacement . At a hiet al(A PNET has a high degree of malignancy and recurrence and a poor prognosis. In clinical practice, this tumor is a rare malignant disease that rapidly enlarges in a short period of time, compressing the surrounding structures and invading the adjacent tissues to cause local symptoms. Light microscopy reveals that the histopathological features of a PNET are cells that are arranged like a nest and tumor cells that are round or oval with a deep-staining nucleus. Less cytoplasm and an unclear border are also observed. Homer-Wright rosettes are usually visualized. Necrosis and mitotic activity are easy to identify . Weissfeet al reportedAnother previous study reported that peripheral imaging shows PNET to have a lack of characteristics . The majThe three cases in the present study shared certain similar imaging observations. The cystic and solid masses were large and certain masses were accompanied by calcification. No clear border was evident between the masses and the surrounding tissues. The surrounding organs were compressed and shifted from the usual position. Subsequent to enhancement of the CT or MRI, the solid components of the mass showed significant inhomogeneous enhancement. These observations were similar to those of previous studies . HoweverIn conclusion, CT and MRI examinations clearly show the predilection site of a PNET, the internal structure of the tumor, anatomical associations between the tumor and adjacent nerve and vascular structures, tumor recurrence and distant metastasis. In the present case reports, the predilection sites of the cases were unique and their imaging observations shared certain similarities that may provide reference for similar clinical diagnoses in the future.


Verruciform xanthoma is a benign mucocutaneous, uncommon, nonsymptomatic lesion of uncertain etiopathology, which occurs mostly on the oral mucosa of middle-aged individuals. Histopathologically, VX is diagnosed by presence of lipid-laden foam cells in papillary region of connective tissue. A 60-year-old male patient presented with a painless growth on the left buccal mucosa. On clinical examination a yellowish white exophytic lesion, measuring 11 × 7 mm in size, was found, which was cauliflower-shaped on inspection and painless on palpation. Histopathological examination revealed varying degrees of surface parakeratosis and the accumulation of numerous foam cells in the connective tissue papillae among the uniformly elongated epithelial ridges. On immunohistochemical staining, there was a neutrophilic infiltrate of the epidermis with CD68 positive xanthoma cells restricted to the papillary dermis, mixed with other chronic inflammatory cells. Verruciform xanthoma (VX) is an uncommon benign mucocutaneous lesion that resembles virus-induced papilloma but has an unknown etiology and uncertain nature. It was first reported by Shafer in 1971. VX usually occurs in the oral mucosa of the middle-aged individuals. It most commonly presents with a verrucous appearance. However in some instances it may appear polypoid, papillomatous, or sessile. It occurs as a small 0.2–2 cm), solitary, asymptomatic, slow growing, white or yellowish red lesion with no sex predilection [–2 cm, soHistologically VX is distinguished from other lesions by the presence of large numbers of foam cells in, and essentially limited to, the connective tissue papillae. The foam cells on ultrastructural studies have been concluded to be fat-laden macrophages , 4. OtheDifferential diagnosis includes erythroplasia of Queyrat (Bowen disease of the glans penis), seborrheic keratosis, verrucous carcinoma, verruca simplex, and condyloma acuminatum , 7. The A-60-year old male patient presented with the chief complaint of a painless growth on the left buccal mucosa . He had On histopathological examination, the hematoxylin and eosin (H&E) stained sections showed parakeratotic epithelium with columns of parakeratin plugging into it. Owing to the uniformly elongated rete pegs, deep connective tissue papillae were seen, some of which extended into overlying surface epithelium Figures  and 3. FThe immunohistochemical staining for CD68 was positive for the foamy macrophages. All the foam cells were strongly stained with anti-macrophage antibodies Figures  and 5.The treatment was performed under local anesthesia. The whole lesion was excised in one piece during excisional biopsy . PostopeVerruciform xanthoma is a rare lesion, accounting for 0.025–0.095% of all cases  with an Histologically, VX shows three patterns: verrucous (most common), flat, and papillary (least common) , 17. TheThe treatment of choice is complete surgical excision which is very effective with no recurrence. However, a recurrent VX of the vulva has been reported in a 30-year-old woman, 8 years after the initial treatment . Histopa


Fibrovascular polyps (FVPs) of the esophagus are rare, and their course is usually indolent until reaching enormous proportions. It is a dramatic entity owing to its tendency to cause bizarre complications. We describe a 49-year-old female patient with sudden dyspnoea that required digital maneuvers to clear the airway. After diagnosing, an FVP of the esophagus, a video-assisted endocavitary surgery was made. Histopathological examination revealed a fibrovascular polyp. Endoscopic controls after excision show no mass or symptoms recurrence. Fibrovascular polyps (FVPs) of the esophagus are benign, rare, intraluminal tumors –8 and reA 49-year-old female patient described an episode of sudden dyspnoea that required digital maneuvers to clear the airway. She described that during the maneuver a smooth mass was detached from the pharynx and swallowed. Similar episodes were repeated in two occasions. During examination a progressive dysphagia to solids was noted. No history of loss weight, cough, or hematemesis was noted. She had no comorbidity of interest.A CT scan was performed revealing a soft tissue mass in the esophagus, extending from the level of the cervical esophagus to the lower esophagus with no clear relation to the esophagus wall. The upper endoscopy showed a sausage-shaped mass obstructing the esophageal lumen, arising from the upper esophageal sphincter and ending 13 cm below . Tracheal intubation previous to upper endoscopy was made; the mass measured 13 × 1,5 cm and was sneared from his distal portion  and pullTwenty-four hours later the patient was able to eat soft meals and 48th  hours after the procedure she was discharged.Histopathological examination revealed a polypoid lesioncoveredbysquamous epithelium withacanthosis liningedematousstromawith areas offibrosis.Blood vessels andabundantlymphoplasmacytic infiltrate , concludEndoscopic controls were made 4 months and 24 months after excision with no mass or symptoms recurrence.2 laser under laryngoscope [Despite its benign condition, it has a life threatening feared complication. This is an unusual tumor and different approaches have being proposed, including transverse cervical incision, transoral resection under direct visualization, endoscopic ligation and electrocoagulation of the pedicle , biapproach surgical technique (eoesophagostomy plus gastrostomy) and COngoscope –9.The novel technical approach that we propose includes the safety and accuracy of transverse cervical incision, the simplicity of an endoscopic procedure, scarless, with early discharge and recovery.It is important to consider that if FVP has become giant, it means we have got late to the patient, and this may be either because the patient shows no complaints, or because its symptoms have not been studied enough due to their nonspecificity.


Langerhans cell histiocytosis is a rare disease within the adult population, with very few cases reported as solitary hypophyseal lesions in adults. Of the reported cases, most have been treated successfully with surgery, radiotherapy, and/or chemotherapy. Radiotherapy has been thought to be curative at the relatively low dose of 20Gy. Here we report a case of recurrent hypophyseal Langerhans cell histiocytosis 9 months after radiotherapy with an interval period of symptomatic and radiographic response to therapy.A 50-year-old Caucasian woman who had headaches, memory difficulties, and diabetes insipidus was found to have a 2.5cm suprasellar mass. Langerhans cell histiocytosis was diagnosed following stereotactic brain biopsy. Further workup revealed no other lesions. Initial radiation treatment succeeded in shrinking the tumor and relieving clinical symptoms temporarily; however, growth and recurrence of clinical symptoms was noted at 9 months. Re-irradiation was well tolerated and the patient had no acute side effects.Isolated hypophyseal involvement by Langerhans cell histiocytosis in adults is a unique presentation of a rare disease. Although radiotherapy doses as low as 20Gy have been reported to offer control, this case demonstrates that higher doses may be warranted to ensure tumor control. With modern imaging and radiotherapy techniques higher doses should offer little increased more durable risk to surrounding critical structures. Langerhans cell histiocytosis (LCH) is a rare disorder of antigen-presenting cells derived from the bone marrow that are termed ‘Langerhans cells’ in honor of their discovery by Paul Langerhans. The disease is believed to have first been reported in 1865 and has been known by many different names . In 1953Although LCH is more common and best characterized in children, the disorder also presents in adults. It has an estimated incidence of 1 to 2 cases per million per year in adults with peak incidence at 33 years of age .Endocrine abnormalities are relatively common in LCH multisystem disease. In one study, 81 of 188 43.1%) adult patients with multisystem disease developed DI [.1% adultThe ‘gold standard’ for histopathologic diagnosis is the presence of characteristic Birbeck granules on electron microscopy . Other hWe present a case of isolated hypophyseal histiocytosis in a 50-year-old woman complicated by recurrence 9 months after initial radiation therapy. It rarely presents as an isolated lesion of the hypophysis. Of the previously reported cases we found in the literature, six were successfully treated with radiation therapy. This case was complicated by an early recurrence, suggesting that these tumors may require higher initial radiation doses, at least in adult presentations, to ensure good local control.A 50-year-old right-handed previously healthy Caucasian woman presented with a several month history of daily headaches, blurry vision, worsening daytime somnolence, and progressive memory difficulties. These symptoms had a significant impact on her activities of daily living and finally resulted in loss of employment. During this time she reported sleeping between 12 and 14 hours per day without improvement in energy level and had gained 18kg (40 pounds) in weight. She also reported a constant feeling of thirst, polydipsia (drinking 10 liters of fluid daily), polyuria, and nocturia. She had been amenorrheic for 5 years and reported no recent vaginal bleeding. She denied nausea, vomiting, heat or cold intolerance, and galactorrhea. Her past medical history was significant only for medication-controlled hypertension and she had no family history of endocrine abnormalities. Her physical examination was significant for bilateral peripheral visual field loss. The remainder of the examination, including a thorough neurological examination, was unremarkable. Laboratory studies demonstrated panhypopituitarism as follows : thyroid-stimulating hormone 1.8uIU/mL (0.35–5.50), free thyroxine 0.52ng/dL (0.90–1.80), prolactin 42ng/mL (2.8–29.0), luteinizing hormone <1IU/L , follicle-stimulating hormone 2.1IU/L (23.0–116.3), adrenocorticotropic hormone 19pg/mL (0–49), insulin-like growth factor 38ng/mL (100–250), and cortisol at 4:00 p.m. <1.5mcg/dL (4.3–22.4).A magnetic resonance image (MRI) of the patient’s brain with contrast showed a 25 × 22 × 20mm intensely enhancing suprasellar mass involving the hypothalamus and optic chiasm. The mass appeared to be separate from the pituitary on both sagittal and coronal imaging Figure . DiffereA stereotactic needle biopsy of the mass was performed. A histological examination showed mildly gliotic brain parenchyma with a weakly angiocentric lymphoplasmacytic infiltrate that ranged from modest to intense. Also present were a few aggregates of histiocytes, one resembling a well-formed non-necrotizing granuloma Figure A. Close Prednisone and replacement hormone therapy, initiated immediately after her initial diagnosis, significantly improved the patient’s functional status; however, she remained fatigued and did not remember her initial hospital visits. Her case was presented at our multidisciplinary Tumor board. The consensus was that radiation therapy was a reasonable treatment approach for control of her tumor. She underwent CT simulation for radiation therapy planning. A 4-field 3D-conformal plan was generated with a prescription dose of 20Gy in 10 once-daily fractions. She completed radiation therapy with no acute complications.At 3 months post-radiotherapy, she reported her energy level and cognition had significantly improved on endocrine therapy and at 5 months post-radiotherapy, an MRI demonstrated a marked decrease in the lesion size (8mm greatest dimension) with decreased edema in surrounding tissues as seen on fluid attenuated inversion recovery (FLAIR) imaging. At 9 months post-radiotherapy, she presented with a 2-week history of mild confusion, forgetfulness, increased fatigue, and an increase in weight. She has had routine follow-up visits with her endocrinologist with no recent changes in her endocrine replacement regimen. MRI at this time demonstrated an interval increase in lesion size from 8mm to 14mm and increased edema in the surrounding tissues.Her case was presented at our multidisciplinary Neuro-Oncology Tumor board. Because she initially responded well to radiation therapy, a second course of radiation was recommended. She was re-simulated and treated with an additional 30Gy in 15 once-daily fractions. She completed radiotherapy with only mild fatigue. At the completion of treatment, she reported improvement in her vision and was able to reduce her steroid dose. Three months after completion of her second course of radiotherapy, an MRI demonstrated a decrease in size of the mass to 10mm with resolution of surrounding FLAIR signal, representing a favorable response to her second course of radiotherapy.Overall, LCH is a rare disease in adults. As such, there is a paucity of data available to guide treatment of adult LCH. Depending on disease site and symptoms, therapy may include chemotherapy, immune modulation, radiation therapy or observation. An ongoing trial evaluating chemotherapy is being conducted by the Histiocyte Society , but results have yet to be published. In 2003 the Histiocyte Society published a retrospective analysis of 274 adults in 13 countries with LCH . All patTo date, only six cases of isolated pituitary lesions in adults treated with radiotherapy have been described in the literature -19. The There are reports suggesting the use of chemotherapy as a first-line treatment for these patients ,19. ThisThis case demonstrates a rare presentation of a rare disease. Although a handful of cases involving LCH limited to the hypothalamic region have been reported, this one represents only the second example of recurrence after radiation therapy. Given the location of this disease, a higher initial radiotherapy dose may be justified because this will probably reduce the chance of failure in a region of the brain surrounded by multiple critical structures. Higher radiotherapy doses are easily achievable in this region using modern radiotherapy techniques that maintain minimal risk of radiation-related toxicities.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal..The authors declare that they have no competing interestsThe patient was under the care of MC and JRS. Pathology was reviewed by RP and KH. Imaging was reviewed by JJS. DF, RF, DM and JJ contacted the patient for consent and drafted the manuscript. RF and DF contributed equally to this manuscript. All authors reviewed and approved the manuscript prior to publication.


Paragangliomas are rare tumors that originate from the autonomic nervous system-associated paraganglia. They metastasize infrequently. Malignancy can only be demonstrated by the presence of chromaffin tissue at sites where it usually is not present, such as bone, lung or liver, or local recurrence after total resection of a primary mass. Paragangliomas within the central nervous system are usually intradural near the conus medullaris. The metastatic spread of a retroperitoneal paraganglioma to a vertebral body is extremely rare, and there are only a few cases reported in the literature.We report the case of a 16-year-old Caucasian girl who had undergone resection of a retroperitoneal paraganglioma that measured 15 × 11.5 × 9.5 cm. After further staging, a solitary metastatic paraganglioma was detected in the first lumbar vertebral body. After initial chemotherapy, marginal en bloc resection and reconstruction were performed followed by radiotherapy. Histologic examination of the specimen revealed that the tumor cells did not show any response to preoperative chemotherapy, which is in line with a few other reports in the literature. Ten years after operative treatment, the patient is free of complaints, very satisfied with the result and without signs of local recurrence or distant metastases.We recommend en bloc spondylectomy and local radiotherapy in the treatment of solitary spinal metastatic paragangliomas. Paraganglioma is a rare tumor that originates from the autonomic nervous system-associated paraganglia. Approximately 90% of paragangliomas arise from the adrenal medulla, carotid body and glomus jugulare -3. TheseWe present one rare case of a solitary L1 metastatic paraganglioma, which was detected after removal of an intraabdominal paraganglioma. Preoperative chemotherapy, en bloc spondylectomy and postoperative radiotherapy were performed.An otherwise healthy 16-year-old Caucasian girl presented with a sudden onset of cramp like pain in the right lower abdomen. After examination, her gynecologist performed laparoscopy and found extreme varicosis of the internal genital tract but without further pathologic findings. To exclude thrombosis and consecutive collateral circulation, postoperative phlebography was done and showed excessive displacement of the inferior vena cava. A retroperitoneal tumor was suspected, and magnetic resonance imaging (MRI) revealed a tumor measuring 14 × 10 × 14 cm in the right abdomen with a craniodorsal shift of the kidney. Laboratory parameters, including tumor markers , β-human chorionic gonadotropin) were within normal ranges.Explorative laparotomy was performed, and the retroperitoneal tumor was resected. The tumor weighed 817 g, and macroscopic examination demonstrated a thinly encapsulated neoplasm. The diagnosis of a paraganglioma was confirmed by histologic and immunohistologic examinations. Because vascular invasion and focal infiltration of the fibrous capsule could be shown, it was an R1 marginal resection.The postoperative course was uneventful, but because of the potential malignant behavior of extra-adrenal paragangliomas, Tc-99-MDP (Tc-99m-methylene diphosphonate) and I-123-MIBG (123 I-metaiodobenzylguanidine) scintigraphy was performed 10 and 21 days postoperatively. An increased uptake in the first lumbar vertebra was noted and MRI showed a lesion in the left dorsal third of the L1 vertebral body  are extra-adrenal rests of neural crest-derived cells that are closely associated with the autonomic nervous system. They are found in disparate areas of the body, including the head, neck, thorax, abdomen and retroperitoneal space. Paragangliomas arising from carotid bodies appear to have the highest propensity for metastatic spread to the spine . The retRazakaboay et al  reportedA third case was published by Hamilton and Tait , who desThe latest report was published by Lehmen et al , who desIn our case, the superficial intraosseous extension of the tumor within the vertebral body, which occupied zones 4 to 9, according to the staging system of Boriani et al , (WeinstChemotherapy was applied before en bloc resection of L1, according to the recommendation of our pediatric oncologists, but histologic microscopy examination of the specimen did not show any response of the tumor cells to the preoperative chemotherapy. This finding is in line with a number of disappointing reports on chemotherapy for this type of tumor ,24,25, aIn a 1992 review, Schild et al  showed tTherefore, we decided to apply radiotherapy with 50 Gy postoperatively. Ten years after surgery, the patient is without signs of local recurrence or distant metastasis, completely asymptomatic and very satisfied with the result of the operation.En bloc resection of a solitary metastatic paraganglioma combined with postoperative radiotherapy seems to be the ideal and only curative therapeutic modality, which is in line with other report on the treatment of specific solitary metastasis as well as primary tumors of the spine ,23,26-30The authors declare that they have no competing interests.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.AR and HFH contributed to this case report's conception and design. They also performed the literature research, prepared the manuscript and reviewed it for publication. URL, TL and MQ were involved in the literature review and helped draft parts of the manuscript. MQ supervised the writing of the manuscript. URL and HFH performed the operation. HFH, URL and TL supervised the general management and follow-up of the patient. All authors have read and approved the final manuscript.


Ethmoid adenocarcinoma is the most frequent ethmoid tumor. To date, only a single case of spinal cord compression resulting from ethmoid adenocarcinoma has been reported. The current case study presents a recent case of vertebroepidural metastasis of an ethmoid adenocarcinoma leading to spinal cord compression. Modern imaging studies, including magnetic resonance imaging (MRI) and 18 fludeoxyglucose positron emission tomography (FDG PET), as well as histological and immunohistochemical analyses, have led to diagnoses of a metastasis of an ethmoid adenocarcinoma, which is a mucinous variant, dedifferentiated when compared to the primary tumor. The present case discusses current diagnostic and treatment protocols of this condition. Since survival rates associated with the primary tumor are improving, the incidence of spinal metastasis of ethmoid carcinomas is likely to increase in the future, therefore requiring appropriate diagnostic and therapeutic management. Although rare, ethmoid adenocarcinoma is the most frequent ethmoid tumor. Chronic exposure to wood dust is a risk factor for its development ,2. ProgrA 70-year-old male, who had previously been a carpenter for 43 years, presented with a 4-month history of headaches with epistaxis. Enhanced head CT and MRI scans revealed a right ethmoid tumor with dural invasion  revealed diffuse metastatic dissemination to the spine, the hilar, mediastinal and peritoneal lymph nodes, the right parotid gland, the right gluteus, the lungs and each adrenal gland (The patient underwent post-operative intensity-modulated radiation therapy (60 Gy to the target volume). Two months later, the patient was admitted to the emergency department with an acute onset of the inability to empty the bladder. In addition, the patient reported severe dorsal pain and progressive lower limb weakness and numbness. A physical examination revealed unexplained weight loss and lower extremity weakness (3/5) to the point where the individual was no longer mobile. Proprioception and sensation to pain and temperature were diminished from the xiphoid process downward. Gadolinium-enhanced T1- and T2-weighted MRI scans revealed abnormal bone marrow signal enhancement at T5 and T6  that wasal gland . By contal gland . Compareal gland .Although rare, ethmoid adenocarcinomas account for >50% of malignancies involving the ethmoid sinuses and ~15% of all sinonasal malignancies ,7. Their18FDG-PET, instead of myelography. In addition, histological and immunohistochemical analyses were performed, which are key tools for diagnosis. The histology of the primary lesion revealed an ethmoid adenocarcinoma, mucinous variant, that was expressing CK20 and partially expressing CK7. Compared with the primary tumor, the metastatic lesion was dedifferentiated, revealing a partial expression of CK20 and no CK7, which is not a rare observation  flow or occur through perivascular and perineural lymphatics ,11,15. Set al recommended a systemic search for tumoral cells within the CSF prior to radical surgery to exclude iatrogenic dissemination (These observations indicate the current issues associated with the initial management of patients affected by T4b tumors, and the rarity of cases explains the lack of available guidelines. Wen mination . Howevermination . Gadolinmination . Despitemination ,4,7,16.In conclusion, vertebroepidural metastasis of ethmoid carcinoma, although rare, must not be disregarded. Initial intradural extension may favor the onset of metastasis and justify a systematic search for neoplastic cells in the CSF and the performance of pre-operative and post-operative gadolinium-enhanced spinal MRI. Histological and immunohistochemical analyses are important for the differential diagnosis of more frequently occurring tumors and allow for establishment of a firm diagnosis. The prognosis of this type of metastasis is poor and depends more on the diffusion of the disease than on local recurrence. Due to current improvements in survival associated with the primary tumor, the incidence of spinal metastasis of ethmoid adenocarcinomas is likely to increase in frequency in the near future, therefore requiring appropriate diagnostic and therapeutic management.


With the help of this case we summarize some crucial features to be picked up from history and examination before labelinga case as Bartholin's abscess or cyst. A 20-year old unmarried girl, deaf and mute since birth, was initially diagnosed to have Bartholin'sabscess. On careful reexamination after inflammation subsided, a decision of excision of this tumor was taken. Intraoperatively it was found to bewell encapsulated. Histopathology ascertained the diagnosis of vulval leiomyoma. Unilateral inflamed vulval swelling in a woman of reproductive age is commonly Bartholin's abscess. We report here an interesting case of vulval leiomyoma which was initially misdiagnosed as Bartholin's abscess. We summarize some crucial features to be picked up from history and examination before labeling a case as Bartholin's abscess or cyst.A 20-year old unmarried girl, deaf and mute since birth, presented with swelling in vulva for 6 months. The swelling which was slowly increasing in size had become extremely painful for the last one week. There was no history of fever and vaginal discharge. On examination her general condition was fair and vital signs were stable. She was afebrile and there was no inguinal lymphadenopathy. Local examination revealed a labial swelling on the left side with signs of inflammation. Diagnosis of Bartholin's abscess was made and she was started on parenteral antibiotics. Within 24 hours her pain decreased and inflammation subsided. As the girl and her parents insisted that she was sexually naïve, she was reexamined. On reexamination 5 × 5 cm swelling on the left labia majora was noted. There were no signs of inflammation now. The swelling felt firm in consistency (not cystic). Labia minora was not everted and hymen was intact . KeepingOn histopathological examination grossly the specimen consisted of single, nodular, grey-white tissue mass weighing 56 g that measured 6 × 4 × 3 cm . On cut This case emphasizes the importance of detailed history and meticulous examination. Bartholin's cyst or abscess is commonly seen in sexually active women. Any case diagnosed initially as Bartholin' abscess needs to be reexamined once the inflammation subsides. Inverted labia minora, firm consistency, and intact hymen point towards reconsidering the diagnosis . Smooth Leiomyoma or fibroid though very commonly seen in the uterus is a rare entity in vulva, ovaries, urethra, and urinary bladder. It is interesting to note that it may arise in nearly any anatomic site .Recently Youssef et al. reported a case of 39-year-old, P2L2 lady who had 2 to 3 cm solid mass on her external genitalia for 4 years enlarged to 15 cm in 6 months. This lesion was excised. On histopathological examination it was diagnosed as leiomyoma of vulva [Nielsen et al. studied 25 cases of leiomyoma of vulva. Most patients in their series presented with a painless mass, other symptoms being pain, itching, and erythema. Most common preoperative diagnosis was Bartholin's gland cyst. There were 20 leiomyoma 4 atypical) and 5 leiomyosarcoma. On followup 4 leiomyosarcomas recurred and one of these women died. Only 1 patient with leiomyoma had recurrence after 10 years. According to them major diagnostic problem with smooth-muscle tumors of the vulva is the distinction between benign and malignant forms  atypical–9. Very Transperineal ultrasonography is of help in establishing the diagnosis of vulval leiomyoma while MRI is of help in differentiating benign and malignant forms in doubtful cases. A characteristic finding of low signal intensity mimicking that of smooth muscle on T2-weighted images is the key to diagnosis. The MR signal in the tumors is isointense to that in muscle on T1-weighted images, and the tumors enhance homogeneously after the administration of contrast material [Surgical excision is the treatment of choice in all smooth muscle tumors of vulva. Histopathological confirmation of its benign or malignant nature is mandatory. This differentiation is a great challenge to the pathologist. Long term followup of all cases is advisable.Extrauterine leiomyomas are rare, and they present a greater diagnostic challenge. Unusual sites of origin include the vulva, ovaries, urinary bladder, and urethra. Other rare locations are sinonasal cavities, orbits, kidneys, and skin. Differential diagnosis includes Bartholin's cysts, fibromas, lymphangiomas, soft-tissue sarcomas, and neurogenic tumors. Ultrasonography/MRI might be of help in establishing the diagnosis. Labial leiomyomas are treated with conservative surgery. After the surgery long-term follow up is advised.


Pheochromocytomas are rare, potentially fatal, neuroendocrine tumors of the adrenal medulla or extra-adrenal paraganglia. Their clinical presentation varies greatly from the classic triad of episodic headache, diaphoresis and tachycardia to include a spectrum of non-specific symptomatology.A 43-year-old Caucasian woman was referred to us from primary care services with a three-month history of recurrent urinary tract infections on a background of hypertension, latent autoimmune diabetes of adulthood and autoimmune hypothyroidism. At 38 years she required insulin therapy. Despite medication compliance and dietary control, she reported a recent history of increased insulin requirements and uncontrolled hypertension with concomitant recurrent urinary tract infections. A renal ultrasound examination, to rule out underlying renal pathology, revealed an incidental 8cm right adrenal mass of both solid and cystic components. A subsequent computed tomography of her abdomen and pelvis confirmed a solid heterogeneous mass consistent with a pheochromocytoma. There were no other features suggestive of multiple endocrine neoplasia. Urinary collection over 24 hours revealed grossly elevated levels of catecholamines and metabolites. Following an open right adrenalectomy, our patient's insulin requirements were significantly reduced and her symptoms resolved. Two weeks post-operatively, an iodine-131-metaiodobenzylguanidine scintigraphy was negative for residual tumor and metastatic disease. Urinary catecholamine and metabolite concentrations were within the normal range at a follow-up six months later.Pheochromocytoma is a rare catecholamine-producing tumor requiring a high index of suspicion for early diagnosis. Our case report serves to highlight the importance of considering pheochromocytoma as a differential diagnosis in the atypical setting of recurrent urinary tract infections and concomitant autoimmune disease. Pheochromocytoma is a rare, insidious adrenal medullary neuroendocrine tumor representing approximately 5% of adrenal incidentalomas . It is aClinical awareness of the variable manifestations of this insidious tumor is key for early diagnosis and optimal patient management. Clinical presentation varies from the classic triad of episodic headache, diaphoresis and tachycardia to include a spectrum of non-specific symptomatology. Despite improved diagnostic techniques, there remains an approximate delay of three years between initial symptoms and a final diagnosis.We discuss an atypical case of a 43-year-old woman presenting with recurrent urinary tract infections in the setting of an undiagnosed pheochromocytoma. We review the contribution of catecholamine hypersecretion to patient symptomatology and the unique association of pheochromocytoma with other autoimmune endocrinopathies.Escherichia coli urinary tract infections. She was diagnosed with gestational diabetes at age 33 years, and in the postpartum period, diet controlled type 2 diabetes mellitus and concomitant hypertension were confirmed. She required insulin therapy some five years later, and, following seropositivity to anti-glutamic acid decarboxylase antibodies, was diagnosed with latent autoimmune diabetes of adulthood (LADA). In addition, she developed autoimmune hypothyroidism at age 38 years. Despite medication compliance and dietary control, she reported a recent history of increased insulin requirements and uncontrolled hypertension with concomitant recurrent urinary tract infections. Our patient is not sexually active.A 43-year-old Caucasian woman presented to our primary care services with a three-month history of recurrent, laboratory confirmed Renal ultrasound examination, to rule out underlying renal pathology, revealed an incidental 8cm right adrenal mass of both solid and cystic components Figure . A subseFollowing a 10-day course of phenoxybenzamine α-adrenergic blockade, the patient underwent an open right adrenalectomy. The immediate post-operative period was complicated by profound episodes of hypotension, requiring three days of inotropic support in high dependency care. Her recovery after this period was uncomplicated with an observed reduction in insulin requirements and anti-hypertensive therapy Figure .Histopathological examination of the excised tissue confirmed a 9.5x9.6cm solid heterogeneous tumor arising from her right adrenal medulla Figure . Two yeaPheochromocytomas are rare, adrenal medullary neuroendocrine tumors with a worldwide incidence of two to eight per million which peaks in the fourth and fifth decades of life ,5. FortyThe lethal nature of this neoplasm is dependent on two major characteristics. The first of these is its ability to secrete excess catecholamines, resulting in potentially catastrophic consequences. The second is its malignant potential. In this context, a multidisciplinary team approach involving anesthesia, surgical and endocrinological expertise is necessary to maximize optimal patient outcome ,12. SurgThe association of pheochromocytoma with other endocrinopathies is a rare yet recognized phenomenon. Previous studies have concluded that diabetes mellitus is present in up to a third of patients presenting with pheochromocyotma ,8,14. ThTo the best of our knowledge, there have been no previous cases reported in the current literature in which a patient presents with recurrent urinary tract infections in association with pheochromocytoma. We postulate that catecholamine overproduction in pheochromocytoma causes decreased insulin secretion and increased end-organ resistance, resulting in hyperglycaemia. Studies have demonstrated impairment of host defenses, including decreased polymorphonuclear leukocyte mobilization, chemotaxis, and phagocytic activity related to hyperglycemia . Recent Pheochromocytoma is a rare catecholamine-producing tumor with potential life-threatening consequences. Clinical manifestations unique to this tumor are occasional atypical and non-specific symptomatology and its association with autoimmune disorders. A multidisciplinary approach involving anesthesia, endocrinology and surgical expertise is the gold standard in maximizing patient care. This case exhibits a rare combination of pheochromocytoma with LADA and autoimmune hypothyroidism. Here we highlight the importance of considering pheochromocytoma as a differential diagnosis in the setting of recurrent urinary tract infections and concomitant autoimmune endocrinopathies.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.RD conceived the study, acquired patient data and drafted the manuscript. JB critically reviewed the manuscript. All authors contributed intellectual content and have read and approved the final manuscript.


In this report, we present the case of a patient affected by appendiceal cystadenoma, a colorectal adenocarcinoma, and a concomitant bladder carcinoma, as well as the results of the molecular study of the most relevant mutational pathways involved in these tumors.PIK3CA mutations, screening revealed that all three samples analyzed carried mutations in exon 9.A 68-year-old Italian man was admitted to our unit complaining of macrohematuria, rectorrhagia, and rectal tenesmus for about 2 months. A colonoscopy showed the presence of a rectal lesion at 11cm from the anal margin; multiple biopsies were performed and a diagnosis of moderately differentiated adenocarcinoma was made. Abdominal ultrasonography and total body computed tomography performed subsequently to stage the rectal cancer showed the presence of two round nodules, interpreted as swollen lymph nodes of neoplastic origin, at the anterior aspect of the iliopsoas muscle and a budding lesion affecting the bladder. The patient underwent transurethral biopsy of the lesion in the right retrotrigonal region; the diagnosis was grade II urothelial carcinoma. The patient underwent an open anterior rectal resection with loco-regional lymphadenectomy. An enlarged appendix and a voluminous whitish soft-tissue lesion requiring an appendicectomy were detected perioperatively. Transurethral resection of the bladder lesion was also performed. The histological examination revealed that the nodular lesions in the appendix were due to a cystadenoma. For mutation analysis, genomic deoxyribonucleic acid was isolated from tumor tissue samples; for PIK3CA-depending pathway may contribute substantially to the pathogenesis of the different solid tumors in the same patient.Appendiceal mucoceles are rare but require adequate surgical treatment, given their malignant potential and the possibility of causing peritoneal pseudomyxoma. It is essential to make a correct preoperative evaluation based on a colonoscopy rather than ultrasound and computed tomography to exclude synchronous neoplasias often associated with mucoceles and to plan the optimum surgical strategy. The association between appendiceal mucoceles and other neoplasias is relatively frequent, especially with colorectal cancer. Oncogenic activation in the  The first description of mucoceles of the appendix was made by Rokitansky in 1842, who described a dilatation of the appendiceal lumen due to mucus accumulation . MucocelMucoceles are distinguished histopathologically as retention cysts (18%), focal or diffuse mucosal hyperplasia (20%), mucinous cystadenomas 52%), or mucinous cystadenocarcinomas (10%) [2%, or muSeveral neoplasias are associated with appendiceal mucoceles. The most common is adenocarcinoma of the colon and rectum, which is detected in approximately 20% of cases -8.PIK3CA gene may be somatically mutated in several types of human cancer [PIK3CA is an effector of the phosphatase and tensin homolog (PTEN)–AKT pathway, which is involved in the inhibition of focal adhesion formation, cell spreading and migration as well as in the inhibition of growth factor-stimulated MAPK signaling  [The mitogen-activated protein kinase (MAPK) pathway  has emerged as a major signaling cascade involved in the control of cell growth, proliferation, and migration in the majority of cancers. A high amount of reports has been published revealing that the n cancer . In partirments) .In this report, we present the case of a patient affected by adenocarcinoma of the colon and rectum, a bladder carcinoma, and a concomitant appendiceal cystadenoma and the results of the molecular study of the most relevant mutational pathways involved in these tumors.A 68-year-old Italian man was admitted to our unit complaining of macrohematuria, rectorrhagia, and rectal tenesmus for about 2 months. No personal or family history of colorectal or urothelial cancer was evidenced. A physical examination revealed pain in the hypogastrium and the right iliac fossa. Laboratory tests showed an increased carcinoembryonic antigen level of 6.46ng/mL  and increased tissue polypeptide antigen .A colonoscopy showed the presence of a rectal lesion at 11cm from the anal margin, which was ulcerated, easily bled, and narrowed the colic lumen. Multiple biopsies were performed and a diagnosis of moderately differentiated adenocarcinoma was made.Abdominal ultrasonography (US) performed subsequently to stage the rectal cancer showed the presence of two round nodules, measuring 33mm and 30mm, at the anterior aspect of the iliopsoas muscle. Moreover, US revealed a budding lesion  affecting the bladder. The patient underwent transurethral cystoscopy and a biopsy of the lesion in the right retrotrigonal region; the diagnosis was grade II urothelial carcinoma.Furthermore, the patient underwent a total body computed tomography (CT) scan, which showed the rectal tumor and the two abdominal nodules detected previously by US. These nodules were interpreted as swollen lymph nodes, probably of neoplastic origin.The patient underwent an open anterior rectal resection with a termino-terminal anastomosis and a loco-regional lymphadenectomy. An enlarged appendix and a voluminous whitish soft-tissue lesion requiring an appendicectomy were detected perioperatively. The patient also underwent transurethral resection of the bladder lesion. The postoperative course was uneventful, and the patient was discharged 11 days after surgery.The histological examination of the specimens confirmed the presence of an ulcerated rectal adenocarcinoma, moderately differentiated and completely infiltrating the rectal wall, but not the perirectal fatty tissue Figure . No neopKRAS exons 2 to 3, BRAF exon 15, and the PIK3CA exons 9 to 20 were screened for mutations by direct sequencing, using an automated fluorescence-based cycle sequencer . Primer sequences were as reported in the Genome DataBase.For mutation analysis, genomic deoxyribonucleic acid (DNA) was isolated from tumor tissue samples, using standard methods. Tumor tissues were estimated to contain at least 70% neoplastic cells by light microscopy. The complete coding sequences and intron–exon boundaries of the KRAS and BRAF genes. For PIK3CA mutations, screening revealed that all three samples analyzed carried a variant (p.S553fs*7 in exon 9) which has been firstly reported as associated with the CRC (catabolite repression control) gene, although it has been previously described in association with an hematopoietic neoplastic disease [PIK3CA gene in our patient. The PIK3CA sequence variation identified was not present in normal adjacent tissues, indicating that these variants are tumor specific and somatically acquired mutations.No mutation was detected in both the  disease . Figure Mucoceles of the appendix are rare tumors, detected in 0.2% to 0.4% of all appendicectomy specimens . They arMucoceles can be symptomatic or totally asymptomatic and diagnosed accidentally. Symptomatic lesions tend to be associated with malignant forms. The most common signs and symptoms are right-sided acute or chronic abdominal pain (65%) and a palpable abdominal mass (15%). Enterorrhagia due to cecal invagination or ileal volvulus, peritonitis by perforation, sepsis, and urological complications are rarer. In our case, the symptoms were pain at the right iliac fossa, rectorrhagia, and macrohematuria .Abdominal US and CT scan are commonly used to diagnose these neoplasms. A mucocele is usually encapsulated on US, with variable echogenicity in relation to the quantity and fluidity of the mucous contained. The inner wall can appear irregular due to the presence of debris or epithelial hyperplasia. US may be decisive for the differential diagnosis of appendiceal mucocele and acute appendicitis; appendicular diameters of ≥15mm are the threshold for diagnosing appendiceal mucocele, with a sensitivity of 83% and specificity of 92% .et al. speculated that nodular lesions in a cystic mass with the above-mentioned features suggest a diagnosis of cystadenocarcinoma [The typical CT scan appearance of an appendiceal mucocele is that of a cystic mass with regular walls that demonstrate contrast enhancement and low attenuation content. Parietal calcifications may occasionally be present. Moreover, Kim arcinoma . US and arcinoma .Endoscopic US seems to result in better evaluation of features suspicious for malignancy, even if it is not commonly used . ColonosMucoceles are a heterogeneous group comprising various histopathological lesions, including retention cysts, mucosal hyperplasia, cystadenomas, and cystadenocarcinomas . AppendiThe treatment of appendiceal mucoceles is essentially surgical, and retention cysts, mucosal hyperplasia, and cystadenomas must be treated with a simple appendicectomy. Larger ileocolic resections, such as a right hemicolectomy, are recommended for cystadenocarcinomas or in the presence of peritoneal pseudomyxoma. This last condition, defined as gelatinous ascites with peritoneal dissemination of neoplastic epithelial cells secreting mucus, is frequently associated with cystadenocarcinoma of the appendix or ovarian cystic tumors and heavily influences prognosis . Aggressive surgical management and intraperitoneal and systemic chemotherapy is recommended for these cases ,19. In oet al. suggested conversion from laparoscopy to open surgery when the presence of a mucocele is confirmed [Tissues must be handled with care during surgery to reduce the risk of rupture or peritoneal dissemination . Thus, confirmed . NeverthPIK3CA gene participated in tumorigenesis of the three different somatic lesions in our patient. Conversely, no pathogenetic implication was inferred for the BRAF and KRAS genes, which are members of the MAP kinase (MAPK: RAS-BRAF-MEK-ERK) pathway and mediate cellular response to growth signals. In general, the MAPK and the PIK3CA signaling pathways both play a key role in cell proliferation and survival; in our case, an oncogenic activation occurred in the PIK3CA-dependent pathway, contributing to pathogenesis of the different solid tumors in the same patient.The association between appendiceal mucoceles and other neoplasias such as gastrointestinal, ovarian, mammary, and renal tumors has been reported. Appendiceal mucoceles and colorectal cancer are associated in approximately 20% of cases -8. Our sThe prognosis for appendiceal mucoceles depends on the histology and the eventual spillage of mucus into the peritoneum. The 5-year survival rate is 100% for low-grade mucinous neoplasias confined to the appendix; however, it decreases to 45% when peritoneal dissemination occurs .Appendiceal mucoceles are rare but require adequate surgical treatment, given their malignant potential and the possibility of causing peritoneal pseudomyxoma. It is essential to make a correct preoperative evaluation based on a colonoscopy rather than US and CT to exclude synchronous neoplasias often associated with mucoceles and to plan the optimum surgical strategy. The association between appendiceal mucoceles and other neoplasias is relatively frequent, especially with colorectal cancer. Oncogenic activation in the PIK3CA-dependent pathway may contribute substantially to pathogenesis of the different solid tumors in the same patient.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they do not have any competing interests.FP and PP were major contributors in writing the manuscript; AC performed the histological examination and description of the specimens, GP and MC performed the molecular analysis described, and FS and MT performed the surgical operation and final revision of the manuscript. All authors read and approved the final manuscript.


Gastric teratomas are extremely rare tumors. A 15-day-old neonate presented with abdominal mass. Ultrasound of abdomen showed mixed echogenicity lesion. CT scan showed a mass with solid and cystic components and internal calcifications. At operation a tumor arising from the posterior wall of the stomach found. It was exogastric as well as endogastric in location. Biopsy report was suggestive of mature teratoma. Gastric teratomas are extremely rare tumors. They mostly present as exogastric growths, but can occur as a mix of exogastric as well as endogastric extension. They are classified into mature and immature teratomas based upon presence and differentiation of neuroglial tissue. Mature gastric teratomas are benign and have good prognosis after complete surgical excision . A neonaA 15-day-old male baby presented with a palpable abdominal mass in left upper abdomen noted by parents since birth. There were no other complaints besides occasional non-bilious vomiting. Ultrasound of the abdomen showed a mixed echogenicity mass with solid and cystic areas. Computed tomography (CT) scan revealed a solid and cystic mass with internal calcifications suggestive of teratoma ; endogastric growths are present in 30% of cases. Mixed exogastric and endogastric growths are rare . In our The main clinical features are abdominal distension, a palpable mass in the epigastrium and left abdomen, vomiting, and respiratory distress. In case of endogastric component there may be additional upper alimentary tract bleeding (hemetemesis and melena), and pain abdomen , 2, 3. IAbdominal radiograph, ultrasonography, CT/MRI, and endoscopy are important diagnostic tools. In most of the cases the preoperative diagnosis of gastric teratomas is difficult. Our preoperative diagnosis was gastric teratoma based upon our previous experience of dealing with immature gastric teratoma, age of the patient, and location in relation to the stomach.Gastroscopy in case of endogastric component may aid in the preoperative diagnosis. Complete excision with tumor free margins is the goal. Long term follow up for recurrence is important. Recurrence in a case of completely excised mature gastric teratoma is seldom reported .Source of Support: NilConflict of Interest: None declared


Pterygopalatine fossa (PPF) is a difficult-to-access anatomic area located behind the posterior wall of the maxillary sinus. Juvenile nasopharyngeal angiofibroma (JNA) often affects this area, and the management of feeding artery to the tumor is important in the surgery. Endoscopic endonasal approach to the PPF without endangering all other nasal structures is useful in the management of JNA. We describe a new approach to the PPF, endoscopic transturbinate approach, which is effective in the management of JNA. Submucous inferior turbinoplasty was performed, and sphenopalatine artery, the feeder to the tumor, was identified at the sphenopalatine foramen. The posterior wall of maxillary sinus was removed. Internal maxillary artery was identified in the PPF and was ligated with a hemoclip. The tumor in the PPF was pushed into the nasal cavity. These procedures were all performed via submucous turbinate tunnel. Then, the tumor was successfully removed in en bloc from the nasal cavity by transnasal approach without ethmoidectomy. This approach improves accessibility and visualization in the PPF and potential to reduce intraoperative bleeding due to ligation of the feeder safely without touching the tumor. Endoscopic transturbinate approach is effective in the management of early stage of JNA. Pterygopalatine fossa (PPF) is a difficult-to-access anatomic area located behind the posterior wall of the maxillary sinus and is bordered by the pterygoid plates posteriorly and middle cranial fossa superiorly . Juvenil A 25-year-old man presented with recurrent right epistaxis. Nasal endoscopy revealed a red, easily bleeding mass that filled the posterior part of the right nasal cavity. Enhanced computed tomography (CT) showed strongly enhanced tumor in the right nasal cavity with the extension to the PPF. The tumor also extended to the sphenoid sinus with bone erosion . The tum Endoscopic sinus surgery was performed under general anesthesia. Firstly, submucous inferior turbinoplasty (SIT) was performed. Vertical incision was made along the anterior margin of the inferior turbinate to expose the turbinate bone. The covering mucosa was elevated from the bone, and the inferior turbinate bone was removed with preserving the mucosa. Mucosal elevation was continued to expose the uncinate process. The inferior half of the uncinate process together with the horizontal portion of the inferior turbinate bone was then removed. SIT provided improved visualization and wide working space in the posterior part of the nasal cavity Figures . The infNo nasal packing was needed, and the postoperative course was uneventful. Postoperative histologic examination confirmed the diagnosis of JNA. No recurrences were observed at the 2-year follow-up examination. The anatomical structure of the nasal cavity and the physiological function of the nose could be preserved by this surgical technique. JNA is a benign tumor, but is locally aggressive and destructive disease. The extremely rich vascularization together with the complex anatomical structures on the cranium, including cranium nerves and vascular structures, and the aggressive growth pattern of the tumor makes its treatment a challenge. Surgical excision is the treatment of choice for JNA –5. The t PPF is a difficult-to-access anatomic area located behind the posterior wall of the maxillary sinus, and is bordered by the pterygoid plates posteriorly and the greater sphenoid wing and middle cranial fossa superiorly . ConventAlthough preoperative selective embolization of feeding arteries has decreased intraopreative blood loss and made it possible to resect even the large tumors, recently, endoscopic surgery for the early stage of JNA has been reported to be performed without preoperative embolization . Endosco In conclusion, endoscopic endonasal transturbinate approach to the PPF is effective for the management of the early stage of JNA.


Primary intracardiac tumors are rare and approximately 50% are myxomas. The majority of myxomas are located in the left atrium and have variable clinical presentation. We report a case of a large myxoma in the right atrium, which is an uncommon location for this type of tumor.A 45-year-old Caucasian woman with a history of palpitation had dyspnea on great exertion and discrete weight loss. A cardiac evaluation showed splitting of S1. An echocardiogram showed a large mass in the right atrium, suggesting myxoma; chest computed tomography confirmed the diagnostic hypothesis. Our patient underwent surgical treatment with excision of a 10 cm multilobulated mass. She presented with supraventricular tachycardia during the operation. She was placed in the intensive care unit and her condition improved after the use of amiodarone. The diagnosis of myxoma was confirmed by histopathological study.In this case report, we emphasize the rarity of large myxomas in the right atrium and the difficulty of differential diagnosis given their dimension and location. Cardiac tumors represent 0.2% of all tumors found in humans . These tMyxomas are located in the left atrium in 75% to 80% of cases and are almost always present with signs and symptoms of mitral valve disease or thromboembolic events. They may arise in other locations, such as the right atrium (RA) (18% of cases) ,6. The dMyxomas affect patients within a wide age range (15 to 80 years), and the average age is approximately 50 years. There is a female predominance in the sporadic form . MyxomasIn regard to the macroscopic aspect, the surface of myxomas may have smooth or lobulated macroscopic features. Oval, rounded, and irregular shapes have been described, and a brownish color appears to be predominant. The consistency of myxomas is also variable, from firm to gelatinous.Microscopic features of myxomas are characterized by a myxoid matrix rich in mucopolysaccharides, in which polygonal cells with an eosinophilic cytoplasm can be detected. The polygonal cells may appear as a star or nest shape or may be multinucleated. Microscopic characteristics including mitoses, necrosis, or pleomorphism are usually not detected or is eventually present as mild findings.Although this tumor presents with benign histological features, the signs and symptoms caused by myxomas are atypical and highly variable and can result in a difficult diagnosis of this neoplasia ,8. AccorThe classic triad found in patients with cardiac myxoma is characterized by obstruction of blood flow, constitutional symptoms, and thromboembolic events ,4,7. TheRoutine laboratory assessment may show non-specific changes such as anemia, increased erythrocyte sedimentation rate, increased levels of globulin and C-reactive protein, leukocytosis, thrombocytopenia, and polycythemia . Recent Although transthoracic echocardiography is less invasive and presents an excellent sensitivity in detecting 95% of myxomas, the sensitivity increases to 100% when followed by transesophageal echocardiography . ComputeOnce a cardiac myxoma is diagnosed, surgical excision should be performed without delays because of the constant risk of thromboembolic events ,10. GeneA 45-year-old Caucasian woman had a 14-year history of palpitations, which became more frequent and intense in the last two months prior to admission and which were associated with dyspnea on exertion and weight loss of two pounds per month. She was on atenolol 50 mg/day and acetylsalicylic acid 200 mg/day.Cardiovascular examination revealed that her heart rhythm was regular with splitting of S1 and no murmurs. The results of an examination of other systems were normal. A chest X-ray showed clear lung fields with RA enlargement Figure  attachedA total abdominal CT after oral and intravenous administration of iodinated contrast showed a normal-size liver and a heterogeneous enhancement of parenchyma associated with dilatation of the hepatic veins and inferior vena cava (cardiogenic liver). A chest CT scan after intravenous administration of iodinated contrast showed an enlarged cardiac silhouette with an intra-atrial hypodensity measuring approximately 6.1 × 5.6 cm and avoided filling of the RA with the contrast medium, suggesting the diagnosis of myxoma Figure .After providing written informed consent, our patient underwent median sternotomy under general anesthesia. Cardiopulmonary bypass (CPB) was established with conventional mild hypothermia (34.0°C). Cardiomegaly due to RA and right ventricular enlargement was observed. During an anoxic arrest for 17 minutes with single aortic cross-clamping, the tumor was completely excised through a right longitudinal atriotomy. The tumor was mobile, pedunculated, lobulated, clear, and gelatinous, with implantation at the atrial superior vena cava junction. The resected mass was sent for histological assessment. CPB was discontinued without any problems and our patient remained hemodynamically stable. The procedure was concluded in the usual fashion. Our patient was transferred to the intensive care unit (ICU) in good hemodynamic condition.Postoperative complications included supraventricular tachycardia, which was reversed with intravenous amiodarone. Subsequently, our patient developed bradycardia for which cardiac pacing was required in the VVI (ventricular-based) mode. Sinus rhythm was resumed three hours later. Our patient was discharged from the ICU on the second postoperative day and from the ward on the fifth postoperative day. She was readmitted to the hospital twice while complaining of palpitations. However, in both admissions, the results of blood tests and imaging exams were normal and there was no evidence of recurrence of the tumor.Macroscopically, the tumor presented as a 10 × 6 × 8 cm lobulated grayish mass weighing 100 g and had an irregular surface and polypoid areas of elastic consistency and spots of loose and friable tissue Figure . On micrPrimary cardiac neoplasms are rare and occur with an estimated incidence of 0.0017% to 0.19%, representing less than 5% of all heart tumors . Myxoma fossa ovalis or base of the interatrial septum [RA myxomas usually originate in the l septum , but in l septum . In thisIn a recent publication reporting 19 years of experience with surgical treatment of primary intracardiac myxoma, seven (17%) cases out of 41 originated from the RA. However, in this series, the mean maximal diameter of the tumors was 5.1 ± 1.8 cm . To the The signs and symptoms of RA myxomas are atypical and highly variable, depending on the size, position, and mobility of the tumor, and are modified according to physical activity and body position of the patient . RA myxoPatients may also present with atypical chest pain, syncope, lethargy, malaise, palpitation, peripheral edema, pulmonary embolism, and hemoptysis. However, the most common manifestation is dyspnea (in 80% of patients), and right heart failure has been reported. Palpitations and dyspnea on exertion were found in this case. An abdominal CT scan showed hepatic congestion, a sign of right heart failure, but our patient had no ascites or edema in the lower extremities. Echocardiography remains the best diagnostic method for locating and assessing the extent of myxomas and for detecting their recurrence, with a sensitivity of up to 100%. However, transthoracic echocardiogram may not identify tumors smaller than 5 mm in diameter, and a transesophageal echocardiogram is required when there is suspicion of a very small tumor . In thisAlthough echocardiography is the modality of choice for screening cardiac masses, magnetic resonance imaging and CT provide information regarding tissue characteristics and allow an excellent overview of cardiac and paracardiac morphology. CT, in this case, showed an enlarged cardiac silhouette with an expansive ovoid mass in the RA with a density lower than that in the heart muscle .The treatment of choice for myxomas is surgical removal ,10. CompThe recurrence rate of sporadic tumors is very low: between 1% and 3% . The opeThough a rare location for a large myxoma, the RA should always be considered in the differential diagnosis of a right-sided heart mass, especially when the patient shows signs and symptoms of heart failure with uncertain etiology. The findings in our case report suggest that cardiologists and surgeons need to make an early diagnosis and treat patients with these tumors to improve the prognosis.CPB: cardiopulmonary bypass; CT: computed tomography; ICU: intensive care unit; RA: right atrium.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.VJSN analyzed and interpreted the patient data regarding the cardiac mass and performed the final review of the manuscript. NACS helped to conduct the review of the literature and made a major contribution to the writing of the manuscript. SFDG helped to conduct the review of the literature and wrote the case report. TLR helped to conduct the review of the literature and wrote the introduction. ECF described the surgical aspects of the procedure. RVAHN analyzed and interpreted the patient imagining data. JSL described the macroscopic examination of the tumor. FACCS translated and reviewed the manuscript. NSF performed the histological examination. All authors read and approved the final manuscript.


Extra-adrenal myelolipoma is a well-described entity in adult population, however it is extremely rare in paediatric age group. An unusual case of intra-peritoneal extra-adrenal myelolipoma in an 8-year-old child is presented here. The lesion was incidentally detected while evaluating the patient for spasmodic abdominal pain. Ultrasonography followed by CT scan and MRI imaging suggested the diagnosis which was confirmed by histopathology. A non-surgical approach was adopted and there was no progression of the lesion on follow-up imaging. Extra-adrenal myelolipoma is a rare benign tumour with only about 50 cases reported previously.[1] Histologically, myelolipomas consist of haematopoietic elements and mature adipose tissue. Cases of extra-adrenal myelolipomas have been described previously only in middle aged and elderly individuals. Extra-adrenal myelolipomas are exceedingly rare in paediatric age-group.[2]An 8-year old girl presented with spasmodic pain in abdomen for one week. The pain was not localised to one particular region of the abdomen. It was not associated with fever, vomiting, diarrhoea or constipation. There was no significant past medical or surgical history. The general physical examination revealed mild tenderness on palpation in the right hypochondrium. Rest of the general and systemic examination was unremarkable. Ultrasonography of abdomen revealed an echogenic mass lesion in the right subhepatic region measuring 3.2 x 2.0 cm. Short term use of dicyclomine relieved the spasmodic abdominal pain. Further evaluation of the incidentally noted abnormality with computerised tomography (CT) scan revealed a well-defined intraperitoneal mass lesion in the right subhepatic region which contained areas of fat density interspersed with areas of soft-tissue density higher than fat. No evidence of calcification was seen within the lesion. Post-contrast CT scan revealed mild enhancement of the capsule of the lesion and the intrinsic areas of soft-tissue density . No invasion of adjacent structures was seen. Magnetic Resonance Imaging (MRI) was performed out of academic interest and confirmed the presence of fat along with some other soft tissues within the lesion on T2W fat suppressed images . The laboratory investigations were within normal limits. A CT guided biopsy of the lesion was performed. Histopathological analysis revealed presence of haematopoietic cells in various stages of maturation admixed with mature adipose tissue suggestive of myelolipoma. In view of the benign nature of the lesion, absence of any attributable symptoms and reluctance of parents for any surgical intervention the lesion was not operated upon. Follow-up ultrasonography at 6 months and 1 year did not reveal any significant change in the size and appearance of the lesion.Extra-adrenal myelolipoma is an uncommon but well-described entity in medical literature. It is being diagnosed with increasing frequency because of the wide availability and use of imaging modalities. The usual locations for extra-adrenal myelolipomas are retroperitoneum, pre-sacral region and pelvis. [3] The median age for diagnosis of extra-adrenal myelolipoma is 66.5 years.[4] The unique features of the case described above are the age of presentation and the location of the lesion. To the best of our knowledge, only one case of extra-adrenal myelolipoma has been described previously in paediatric age group.[2]The aetiology is not known but endocrine disorders and chronic diseases are believed to play a role.[5] In our case, no such underlying co-morbidity was found. Many patients are asymptomatic at diagnosis, while some may present with non-specific symptoms such as abdominal discomfort, nausea and vomiting. Surgical treatment of myelolipoma is indicated only if the patient is symptomatic or the tumour is causing mass effect. In asymptomatic patients, the lesion may be monitored by periodic imaging. In our patient, the spasmodic pain was unlikely to be related to the lesion, because the pain was not localised to right hypochondrium and short term use of an antispasmodic relieved it.Though specific pre-operative diagnosis of extra-adrenal myelolipoma is difficult, imaging can be suggestive.[6] The fatty component of the lesion appears hyperechoic on ultrasonography, whereas the myeloid component appears hypoechoic. CT scan is more specific for the presence of fat within the lesion. The myeloid component shows higher attenuation as compared to fat and may also show enhancement on post-contrast CT study. Extra-adrenal myeloliomas are well-encapsulated lesions and may show capsular enhancement on post-contrast CT, as seen in our patient. Presence of fat within the lesion is confirmed on T1 weighted and fat-suppressed MRI sequences. The radiological differential diagnosis includes lipoma, dermoid, liposarcoma, myolipoma, angiomyolipoma and extramedullary haemopoiesis.[5] Core biopsy of the lesion establishes definitive diagnosis, thus preventing unnecessary surgical intervention.[6]Source of Support: NilConflict of Interest: None declared


Adenoid cystic carcinoma of Bartholin’s gland is a very rare disease.A 48-year-old premenopausal woman of Caucasian origin was delivered adjuvant pelvic and inguinal radiotherapy after prior complete left Bartholin’s gland tumor excision and inguinal lymph node dissection for adenoid cystic carcinoma of Bartholin’s gland with one metastatic inguinal lymph node.Two years after primary treatment, she presented to the Emergency Room with acute headache, hypoacousia, decrease in visual acuity, and a decrease in right leg muscle strength. A cranial magnetic resonance imaging scan demonstrated three cystic brain lesions with associated perifocal edema. Chest and abdomen computed tomography scans and a magnetic resonance imaging scan of the pelvis did not find any metastatic or residual disease elsewhere. A physical examination found no local recurrence.Stereotactic brain biopsies with pathology examination revealed the presence of adenoid cystic carcinoma metastasis. She thus received 30Gy of brain radiotherapy but, three months later, the brain lesions did not decrease in size and left mid lobular lung lesions appeared on her chest computed tomography scan. A mid left lobe lung excision was undertaken followed by chemotherapy consisting of six cycles of cyclophosphamide, adriamycin and cisplatin. Five months after beginning chemotherapy, the brain disease progressed and our patient died.Our case report shows the difficulty in managing brain and lung metastasis of Bartholin’s gland adenoid cystic carcinoma as no consensus on the optimal treatment exists. Bartholin’s gland carcinoma is a rare disease, accounting for 0.1% to 5% of all vulval carcinomas and representing barely 0.001% of gynecological cancers . The meaIn May 2009, a 48-year-old gravida 1 para 1 premenopausal woman of Caucasian origin presented with a tender mass in the area of the left Bartholin’s gland, associated with a left palpable inguinal lymph node, which had been slowly evolving for two years. Following the tumor’s recent increase in size and vulval pain, a left Bartholin’s gland excision (bartholinectomy) was performed under general anesthesia.The pathology examination revealed a primitive ACC of Bartholin’s gland measuring 30mm but with positive margins.Upon diagnosis of this unexpected malignant lesion, a systematic search for metastatic disease was undertaken. Chest, abdomen and pelvis computed tomography (CT) scans with intravenous contrast along with a magnetic resonance imaging (MRI) scan of the pelvis showed a highly suspicious left inguinal lymph node demonstrating high metabolic activity upon whole body positron emission tomography (PET) scan. No other site of metastatic disease was detected.Next, our patient underwent radical tumor re-excision with left inguinofemoral lymph node dissection. The postoperative pathology examination found two remaining tumors of 6mm close to the surgical margin. Tumor cavity vaginal and vestibular re-excisions were clearly disease-free. Among the seven inguinofemoral lymph nodes collected, one of them, measuring 45mm with an intact capsule, had metastatic disease.Postoperatively, after multidisciplinary discussion of the case, adjuvant pelvic and inguinal radiotherapy was recommended. She thus obtained 66Gy of radiation.Follow-up visits were organized every three months with CT scans of the chest, abdomen and pelvis performed every six months. In June 2011, two years after her primary treatment, she presented to the Emergency Room with acute headache, hypoacousia, decrease in visual acuity, and a decrease in right leg muscle strength.A cranial MRI scan demonstrated three cystic brain lesions in the right parietal, left parieto-occipital and left temporal region with associated perifocal edema Figure . Chest aStereotactic brain biopsies were undertaken. A pathology examination revealed the presence of ACC metastasis with characteristic tumor proliferation in a cribriform pattern composed of nests and columns of cells arranged concentrically around gland-like spaces filled with eosinophilic periodic acid-Schiff-positive material. These results were confirmed after immunological staining with specific antibodies, which showed that the brain lesions had similar characteristics as the initial ACC of Bartholin’s gland that was diagnosed two years earlier Figure . ImmunohOur patient’s case was once again discussed at a multidisciplinary meeting where whole brain radiotherapy was advocated. The patient thus received 30Gy of brain radiotherapy ending in August 2011. A cranial MRI scan, chest and abdomen CT scans, and a MRI scan of the pelvis were performed three months later to evaluate the efficacy of the treatment. The brain lesions had not decreased in size and right middle lobe lung lesions appeared on the chest CT scan  code of conduct. Every effort was made to protect the identity of our patient and there is no reason to believe that our patient would have objected to publication.ACC: Adenoid cystic carcinoma; CK: Cytokeratin; CT: Computed tomography; EMA: Epithelial membrane antigen; MRI: Magnetic resonance imaging; PET: Positron emission tomography.The authors declare that they have no competing interests.RR analyzed data and wrote the manuscript. EAN and CB performed the literature search. DR reviewed the manuscript. All authors read and approved the final manuscript.RR is a gynecologist specialized in vulval cancer treatment and research. He is a fellow of the International Society for the Study of Vulvovaginal Disease (ISSVD).


Angioleiomyomas are benign mesenchymal tumours commonly occurring in the subcutis of extremities. They are typically composed of interlacing fascicles of smooth muscle cells with intersecting vascular channels. Angioleiomyomas of the uterus are rare with only very few case reports available in literature. Herein, we report a case of this rare entity in a 47-year-old woman owing to its highly unusual features of cellular atypia, raised CA-125 levels, and pseudo Meigs syndrome. Angioleiomyoma is a rare and distinct variant of leiomyoma . UterineA 47-year-old female presented to the gynaecology outpatient department with a one-year history of abdominal distension. She also had a history of menorrhagia, dyspepsia, and weight loss. On clinical examination, she was noted to have massive ascites with a palpable lower central abdominal mass. On vaginal examination, the uterus could not be felt separately from the mass. Her CA-125 level was 477.1 IU/mL . The possibility of an ovarian tumor or uterine sarcoma was raised clinically.Ultrasound scan revealed a large mass posterior to the uterus along with massive ascites. CT scan performed showed a large predominantly solid pelvic mass demonstrating loss of fat plane with the uterine myometrium. The ovaries were not localized. There was massive ascites along with minimal left-sided pleural effusion. The diagnostic possibilities considered were those of a solid ovarian neoplasm infiltrating the uterus and a pedunculated uterine fibroid with sarcomatous change. The cytological examination of her ascitic fluid revealed no malignant cells. The patient underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy. About ten liters of serous ascitic fluid was drained. Preoperatively, a large irregular tumor with hemorrhage and degeneration was seen arising from the uterine isthmus.Grossly, the specimen with the attached tumor weighed 1.5 kgms. The uterus and cervix together measured 9 × 6 × 4 cm. A large hemorrhagic mass measuring 18 × 15 × 8 cm was seen to arise from the lower posterior part of the uterus . On sectHistological examination of the tumor showed a moderately cellular neoplasm composed of interlacing fascicles of spindle cells with interspersed abundant thick muscular walled vessels . Bland sOn immunohistochemistry, the tumor cells showed positivity for smooth muscle actin (SMA)  and desmThe patient has had no complications or further symptoms on followup for 6 months post-op and to date.Angioleiomyomas are benign, relatively common neoplasms described in the lower extremities, head, and trunk. However, only very few cases of angioleiomyomas have been described in the uterus  and theyKawagishi et al.  and J. ACA-125 is a valuable serum marker that aids in the diagnosis of ovarian cancer. However, CA-125 levels are not entirely specific for ovarian carcinomas and can be seen elevated in several nonneoplastic and other neoplastic conditions including benign ovarian fibromas  and uterPseudo Meigs syndrome encompasses the clinical triad of hydrothorax, ascites, and pelvic tumours/ovarian tumours (excluding ovarian fibromas). In contrast to Meigs syndrome, which has been typically described in association with benign ovarian fibromas, pseudo Meigs syndrome can be seen with benign and malignant tumours including uterine leiomyomas , leiomyoThe study on vascular system of intramural leiomyomas by Walocha et al.  revealedOne of the differential diagnoses considered in this case was a PEComa. In our case, the tumor cells showed perivascular accentuation around thick walled vessels but lacked the clear or eosinophilic granular cytoplasm described in the latter. The cells were negative for HMB-45 and positive for SMA and desmin, thereby ruling out the possibility of a PEComa. In cases of angioleiomyomas that exhibit atypia, the possibility of a leiomyosarcoma should be excluded. This case illustrates the importance of thorough sampling of such tumors to identify foci of coagulative tumor cell necrosis, increased mitotic activity, and atypical mitoses, which are diagnostic features of leiomyosarcoma. The other differential diagnosis includes symplastic leiomyoma, which also exhibits cellular atypia but lacks abundant thick walled blood vessels with perivascular spindle cell swirling.In conclusion, this is a unique case of a uterine angioleiomyoma with atypia in a middle-aged woman who presented with clinically worrisome massive ascites and markedly elevated CA-125 levels. Atypia of tumor cells in such a clinical context can be alarming, but diligent sampling and histopathological examination of these tumors can provide crucial diagnostic pointers to the benign nature of these lesions.