Orbital roof defects are rare congenital osseous abnormalities that cause protrusion of intracranial contents into the orbit, resulting in a condition known as the orbital meningoencephalocele, a rare cause of pulsatile proptosis. We present a case of a 25-year-old lady, presented to us with complaints of left frontal headache, left eye protrusion and double vision from the left eye for the past three months. Her higher mental functions were intact. Local examination revealed non-axial pulsatile proptosis and an outward depression of the left eyeball along with diplopia. Extraocular movements and pupillary light response were normal with no bruit or orbital tenderness. Computed tomography (CT) scan of the brain and orbit with contrast showed deficient bone on the left orbital roof and floor with left frontal gliotic brain compressing the eyeball. Magnetic resonance imaging (MRI) of the brain and orbit with contrast showed an asymmetrical deformity of the skull and left cerebral hemisphere which was bulging towards the left orbit. We planned a two-staged surgical reconstruction. The orbital roof was first reconstructed using a titanium mesh. Within two weeks of surgery her pulsatile proptosis, diplopia, and headache had considerably improved and the proptosis had resolved with no visible pulsations. She is scheduled for second stage surgery after three months for reconstruction of the orbital floor. Congenital orbital osseous defects are a rare cause of orbital meningoencephalocele where the intracranial contents protrude through the bony defects into the orbit giving rise to pulsatile proptosis. The defect may be in anywhere in the orbital roof or floor and the medial or lateral orbital wall. Herniation can also be through the natural openings, e.g., the optic foramen or the sphenoid fissure. Intracranial contents passing through these defects give rise to varying degrees of proptosis. Although proptosis is seen in many pathological conditions, the causes of pulsatile proptosis are few and can also occur in some cases of Von Recklinghausen's disease . Apart fA 25-year-old female with no known co-morbidities or history of previous illness or trauma presented to the neurosurgery outpatient department. According to her, she was well three months back, when she suddenly developed episodes of severe left frontal headache which were continuous for hours to days. It was throbbing in character not associated with symptoms of nausea, vomiting, dizziness or fits. At many occasions, she complained of double vision with severe headache. For the same length of time, she also noticed that her left eye was bulging outwards along with pulsation in the same protruded eye which gradually increased and since one month it had been so obvious that anyone looking at her could appreciate it.Her general physical examination was unremarkable. She was conscious and well oriented to time, place and person. Her higher mental functions, gross motor, fine motor and sensory functions were intact. On local examination of the left eye, there was non-axial pulsatile proptosis with outward depression of the left eyeball. On visual acuity, she was assessed to be emmetropic with diplopia in her left eye. Ocular movements in both eyes were normal. There was no tenderness on palpation but pulsations were felt on left eye. No bruit was heard on auscultation.Computed tomography (CT) scan of the brain with contrast study showed deficient bone on left orbital roof and floor with left frontal gliotic brain compressing the eyeball causing it to move out and down Figure . Her magWith this information, a working diagnosis of orbital meningoencephalocele was made and a maxillofacial consult was taken. The patient and her family were counseled regarding the condition and about our plan of surgical intervention that included a two-staged procedure. Left frontal craniotomy was done as a part of the first stage. Per-operatively after opening the dura, frontal lobe was retracted without violation of the arachnoid. The retraction assisted in determining the bony defect. Then a titanium mesh was used to reconstruct the orbital roof and was fixed with screws over the inner surface of superior orbital rim. Duroplasty was performed and the frontal bone flap was secured. A subgaleal drain was then placed and the wound was closed in layers. The drain was removed at the first post-operative day and she was mobilized and discharged on third post-operative day. Her post-operative CT scan with three-dimensional 3-D) reconstruction confirmed the appropriate placement of metallic prosthesis covering the bony defect in the roof of the left orbit  and posterior. Anterior type is located between the frontal, lacrimal, cribriform bone and the nasal process of the maxilla. Sometimes it is evident at the base of the nose. The posterior type is less common. There may be a defect in the roof, floor or the orbital walls causing non-axial proptosis. Small defects are rarely symptomatic. The herniated intracranial content may either be a meningocele, encephalocele, or hydroencephalocele. In the majority of cases the brain substance, which is usually atrophic and oedematous, is incorporated. There may be associated chemosis and edema of the lids especially the upper lid. The proptosis is because of the widening of the ventricle and raised unopposed pressure of the cerebrospinal fluid that gradually pushes the brain forward through the defect, causing it to herniate into the orbit and forcing orbital contents downwards and outwards. The pulsation is due to the direct transmission of the pulsation of the brain to the eyeball. Proptosis usually increases on coughing, bending or straining. Those associated with bruit are usually because of carotid cavernous fistula or congenital arteriovenous malformation seen either in isolation or as part of Wyburn-Mason Syndrome. Rare causes of pulsatile proptosis have been reported in metastatic disease of the orbit from carcinoma of thyroid and renal cell carcinoma , 4.A correct diagnosis is of substantial importance for management. The final diagnosis of the disorder, however, builds upon radiological examination of the skull and brain. The semblance is discrete and not likely to be confused with any other condition. While, bony deformity may vary from case to case, in our patient, there is an asymmetrical deformity of skull and left cerebral hemisphere, which is bulging towards the left orbit, causing extrinsic pressure on the eyeball resulted in proptosis. Large bony defects demand aesthetic skills for closure and require either bone graft, a dural substitute (pericranium or fascia lata), or gel film. Synthetic material like titanium has been used. In this case, we used a titanium mesh to reconstruct the orbital roof although outer calvarial bone grafts have also been used in some cases .It seems probable that some of these cases never develop symptoms which call for treatment and go through life without the condition being recognized. Certainly, the degree of pulsation of the globe in our case was such that there was diplopia and the proptosis was severe.Orbital roof defects are rare congenital osseous abnormalities that cause protrusion of intracranial contents into the orbit, resulting in a condition known as the orbital meningoencephalocele, a rare cause of pulsatile proptosis. A correct diagnosis is of substantial importance for management. The final diagnosis of the disorder, however, builds upon radiological examination of the skull and brain. It seems probable that some of these cases never develop symptoms which call for treatment and go through life without the condition being recognized, however, once symptomatic, they require prompt surgical reconstruction. Large bony defects demand aesthetic skills for closure and require either bone graft, a dural substitute (pericranium or fascia lata), or gel film. Synthetic material like titanium mesh can also be used.


Treatments with dental surgery seek to displace tooth to the correct position within the dental arch. To report a clinical case that took advantage of an unerupted third molar. A male patient, 18 years of age, was referred by his dentist to evaluate the third molars. The clinical exam revealed no visible lower third molars. The computed tomography (CT) exam showed the presence of a supernumerary tooth in the region of the mandibular ramus, on the left side, and impaction of the third molar, which was causing root resorption on the second molar, thus making it impossible to remain in the buccal cavity. The preferred option, therefore, was to remove both second molar and the supernumerary tooth, in addition to attaching a device to the third molar during surgery for further traction. After 12 months, the third molar reached the proper position. When a mandibular second permanent molar shows an atypical root resorption, an impacted third molar can effectively substitute the tooth by using an appropriate orthodontic-surgical approach. Dental eruption occurs with great precision in the great majority of human beings. The deciduous and permanent teeth are formed inside the maxillary bone and, at a certain point, erupt to fully develop chewing and speech functions.Failure on dental eruption is a common clinical situation that represents an unfavorable aspect from the esthetic and functional points of view. Among the regions that are affected by this problem, a greater occurrence is observed in the third molars and canines, but this event can also be found in other regions of the oral cavity.The third molars are most frequently impacted (20 to 30%) because they are the last teeth to erupt in the oral cavity, followed by the maxillary canines , mandibular second premolars, and central maxillary incisors.Orthodontic traction of an impacted tooth can be performed in many ways, including: the direct attachment of brackets, buttons, or wires to the retained teeth and with an application of force placed on fixed and removable appliances. The anchorage of fixed devices offers a greater control and effectiveness of the applied force, thus minimizing undesired effects, although they may still occur.Few studies in the literature report on the orthodontic traction of third molars. Nevertheless, in some cases, taking advantage of third molars shows a good alternative to compensate second molar loss. Orthodontists should be aware of the third molars, even when these teeth do not interfere directly in the planning and treatment.Therefore, the present study sought to report a case that took advantage of an unerupted mandibular third molar.A male patient, 18 years of age, sought the Surgery and Oral/Maxillofacial Traumatology service for an evaluation of third molars. The patient was referred by the orthodontist, who requested the extraction of lower left second molar, due to root resorption caused by impaction of the third molar crown. The extraction of the supernumerary tooth located in the region of the mandibular ramus on the left side was also indicated. Then, the patient underwent an orthodontic traction of the third molar.In the overall evaluation, the patient reported no systemic changes or the use of any type of medications.After undergoing an extraoral exam, the patient presented no visible or palpable changes. In the intraoral evaluation, the absence of tooth #38 was observed. A subsequent computed tomography (CT) exam showed that the tooth #38 was unerupted and impacted by tooth #37. The accentuated resorption of the distal root indicated the tooth for extraction .In this case, an orthodontic surgical treatment was recommended, opting for the removal of tooth #37 and the supernumerary tooth. In addition, a button was bonded onto the buccal surface of the tooth #38, which was anchored through a wire (0.3mm) to the orthodontic appliance, for further orthodontic traction of the tooth #38 .The procedure was performed under local anesthesia, using 2% lidocaine with vasoconstrictor. A conventional incision was made to remove the third molar, with total mucoperiosteal displacement, for the appropriate exposure of the teeth. After the procedure, the patient was medicated with antibiotics for seven days, and anti-inflammatory and analgesic medications for three days.A detailed follow-up of the patient was performed. After 12 months, tooth #38 was observed in its proper position within the dental arch .Patients must be aware of all risks inherent to their treatment and should be well-advised by the dentist. All possibilities should be discussed in advance, including possible complications, especially in orthodontic-surgery combined treatments.Tooth impactions occur as a result of displacements in the normal sequence of occlusion development. In addition, impacted teeth can cause various problems, such as roots resorption of neighboring teeth, a loss of length in the dental arch, the formation of a dentigerous cyst, local infections, reflex pain, etc. In the present case, tooth #38 caused the loss of tooth #37 due to the resorption of the distal root. The tooth submitted to traction presented no divergent roots, root lacerations or other conditions that contraindicated orthodontic traction. The technical difficulty in achieving the proper force to tractioning teeth with divergent roots contraindicates this procedure.,Another point that should be observed is the periodontal tissues condition, since the tooth will be displaced great distances for a long period of time. For this reason, it is essential to maintain a good oral hygiene, in such a way that the alveolar bone and the soft tissue follow along the tooth, in order to prevent gingival recession. The present patient was quite cooperative and aware of his role in the treatment results, presenting a favorable oral hygiene, with no history of periodontal problems.The appropriate removal of the bone tissue surrounding the crown of the pulled tooth constitute an important step toward a successful treatment. In the present case, an osteotomy was performed in the cementoenamel junction, for the correct insertion of the orthodontic device, providing a better distribution of the orthodontic forces.Well-planned and executed orthodontic traction represents a safe procedure whose minimal consequences are clinically controllable. Thus, this is a safe procedure, with minimal consequences. Even when associated with surgically induced dislocation, which is also well-planned and conscientiously executed, orthodontic traction still represents a safe procedure.It is not common taking advantage of unerupted and impacted third molar, due to location and difficult traction. Few reports show the treatment with successful results, especially due to the inappropriate exposure of the crown and the improper application of orthodontic traction. This type of problem leads many professionals to fail to take full advantage of this procedure.It is also important to remember that taking advantage of this tooth allows correct homeostasis within the region, regardless of prosthetic rehabilitation, which would certainly promote a lasting and effective rehabilitation, without the need for future interventions.In the present case, CT exam enabled to verify, with greater precision, the relationship between the impacted third molar and the adjacent teeth and noble structures.The CT exam, in this case, was very important as a diagnostic aid. This exam provided a better observation of the relationship of tooth #38 with the noble structures, such as the inferior alveolar nerve, as well as its relationship with the distal surface of tooth #37. The morphology of the root and crown of tooth #38 were also observed, thus determining the viability of the orthodontic traction of this tooth. In this context, the chances of complications in the trans-operative period and during orthodontic traction were reduced.,Previous studies reported means of performing orthodontic traction of unerupted teeth, such as the attachment of orthodontic devices, buttons or brackets, and the use of fixing plates for anchoring.The use of tooth #38 was considered to be quick and effective. However, what should be taken into consideration is the importance of having sufficient space in the dental arch and a favorable position of the impacted tooth before beginning such treatment. The prognosis will depend on the patient’s age, in addition to complementary exams, to determine the position and the possible complication that can hinder orthodontic traction. Hence, when a mandibular second permanent molar shows an atypical root resorption, an impacted third molar can effectively substitute the tooth by using an appropriate orthodontic-surgical approach.


Purpose. Sinonasal malignant mucosal melanoma is a rare, aggressive tumour. Nasal obstruction and epistaxis are the most commonly reported symptoms, although symptomatology may develop late and be nonspecific, which tends to delay diagnosis, resulting in a poorer prognosis. Case Report. This report describes a 64-year-old male patient with nasal obstruction and epistaxis. Computed tomography of the facial sinuses revealed a large lesion in the right nasal cavity, with infiltration into the left cavity, ethmoidal cells, and erosion of the cribriform plate. Initial incisional biopsy revealed an undifferentiated carcinoma of the right maxillary sinus, staged as T4aN0M0. Induction chemotherapy was initiated with cisplatin and etoposide. Response to treatment was complete. The patient was then submitted to radiotherapy with concomitant cisplatin. Immunochemical analysis revealed positivity for vimentin, S100, and HMB-45 (human melanoma black 45), a result compatible with a diagnosis of malignant melanoma. Discussion. Due to the rarity of the tumour and the patient's complete response to chemotherapy and since no blackened lesion had been found at the previous exam, treatment was continued as planned. The patient remains healthy, with no metastasis or recurrence. He is currently being monitored by the clinical oncology team. First described by Lucke in 1869 –8. Its i Irmandade da Santa Casa de Misericórdia de Vitória approved this report on June 28, 2016, under reference number 56846116.9.0000.5065. The patient gave his written informed consent.This report on a case of malignant mucosal melanoma includes a brief literature review. The internal review board of theA 64-year-old white male consulted at a public healthcare facility in Mantena, Minas Gerais, Brazil, in February 2013 with pain on the right side of his face and nose. He reported sporadic episodes of epistaxis and progressive ipsilateral nasal obstruction beginning about a year previously.Computed tomography (CT) of the facial sinuses revealed a large, solid, voluminous mass in the right nasal cavity, measuring 3.8 × 3.1 × 5.1 cm along its largest axes and extending into the left nasal cavity, ethmoidal cells, and cribriform plate, causing erosion of the plate. Incisional biopsy was performed and histology revealed an undifferentiated carcinoma of the right maxillary sinus. Santa Casa de Misericórdia Hospital in Vitória, Espírito Santo, Brazil. Since surgical resection proved impossible, the condition was treated clinically. Symptomatology persisted and diplopia developed. The patient's general health was good, with no asthenia, anorexia, or weight loss. Karnofsky index was 90%. Induction chemotherapy consisted of three cycles of etoposide and cisplatin and was followed by six cycles of cisplatin concomitantly with radiotherapy.The patient was referred to theThe histopathological report was reviewed, revealing an undifferentiated ulcerated neoplasm. CT of the brain and paranasal sinuses showed a solid lesion affecting the ethmoidal cells, nasal cavity, and right sphenoid sinus and extending as far as the floor of the sella turcica on the right side, causing erosion; there were no signs of pituitary involvement. Infiltration into the sphenoethmoidal recess causes widening. Thickening of the mucosal tissue occupied the maxillary sinuses bilaterally, predominantly on the right. The lesions were lodged in the right nasal cavity, deviating the septum to the left. CT of the neck showed no lymph node involvement. The patient was staged as cT4aN0M0 . ImmunohTNM Staging in Cases of Sinonasal MelanomaPrimary Tumour (T)x: the primary tumour cannot be evaluated.TT3: disease restricted to the mucosa.T4a: disease moderately advanced: tumour deeply involving soft tissue, cartilage, bone, or overlying skin.T4b: very advanced disease: tumour involving brain, dura mater, skull base, cranial nerves , masticator space, carotid artery, prevertebral space, or the mediastinal structure.Regional Lymph Nodes (N)x: the regional lymph nodes cannot be evaluated.NN0: there is no evidence of regional nodal metastases.N1: presence of regional nodal metastases.Distant Metastases (M)M0: no distant metastases.M1: distant metastases.In view of the rarity of the tumour, the patient's complete response to induction chemotherapy, and the fact that no blackened lesion had been found at the previous exam, treatment proceeded as planned, even after diagnosis was changed during chemotherapy. Over the 21 months of follow-up with CT scans every 3 months, there were no signs of recurrence or metastasis and an improvement was seen in the patient's right sphenoid and maxillary sinus, with almost complete resolution of the lesions detected in previous exams. The patient remains healthy and is being followed up.Primary mucosal sinonasal melanoma corresponds to 0.5–2% of all malignant melanomas and approximately 4% of melanomas of the head and neck . Mean 5-The condition predominantly affects individuals ≥60 years of age. Although possible at any age, it is extremely rare in young people  and sligEtiopathogenesis remains unclear; however, mucosal melanomas are known to develop from melanocytes present in mucosal tissue originating from the migration of cells from the neural crest during the embryonic period –29.No specific risk factors are known. The long-term presence of melanosis is the association that has been most clearly demonstrated in the case of oral melanoma . OccupatThe absence of symptoms in the initial stages delays diagnosis. Furthermore, it is often difficult to differentiate primary melanoma from metastases . The mosAnterior rhinoscopy and fibre optic nasolaryngoscopy may reveal blackish-blue, pale yellow, or, in the case of amelanotic melanomas, translucent polypoid masses . The priNeck nodal metastasis occurs in 10–50% of patients , 35, 36.Definitive diagnosis is based on immunohistochemistry. Great care should be taken with diagnosis if immunohistochemistry is not performed . In the Most mucosal melanomas are of the lentiginous, superficial, or nodular types , 40. HisFirst-line treatment consists of surgical resection . ProphylFor patients with sinonasal melanoma, prognosis is poor, since this tumour is aggressive and normally diagnosed at more advanced stages. More than 50% of patients die within three years of diagnosis , some wi


Paediatric bilateral multiple trigger fingers are extremely rare. The underlying etiopathogenesis and hence the surgical principles of management of trigger finger in children are different from those of pediatric trigger thumb and adult trigger finger. In this paper, we report the case of a 5 year old girl with congenital trigger digits involving the middle, ring and little fingers of both hands. She did not have any episode of trauma, viral or bacterial infections or any metabolic disorder. Following lack of any improvement with a physiotherapy and a splintage regime for 6 weeks, we offered surgical management for the affected digits. Release was done in step-wise pattern. We present the intraoperative findings and surgical management of congenital trigger finger. Initially the partly bent fingers could be straightened with “a pop” (triggering). Gradually a flexed posture of the Middle, Ring and Little fingers of both hands was noticed. There was no history of perinatal trauma, viral or bacterial infections, or rheumatologic or metabolic disorders, nor was there any other developmental anomaly.The physical examination revealed flexion deformity ranging from 10-20 degrees in both proximal and distal interphalangeal joints of the middle, ring and little fingers of both hands. Triggering was absent. Non-tender nodules could be palpated on the volar aspect at the level of the proximal interphalangeal joints of all affected fingers. Radiographs of both hands were normal. A high frequency focused ultrasound of both hands showed mild thickening of A1 pulley in all fingers of the right hand and three fingers of the left hand. Following 6 weeks of physiotherapy with no discernible benefit, considering the age of the patient and the nonreducible nature of the deformity, we decided to proceed with surgical release.Surgical exploration of the middle and ring fingers of the right hand was performed using Brunner’s incision. In both fingers considerable thickening of the A1 pulleys was noted. In the middle finger, fusiform enlargement of FDP tendon was found at the level of C1 pulley. In the ring finger, a fibrous band of adhesion was found between FDP and FDS tendons proximal to A1 pulley in addition to the above findings . After r1 genetic disorders. Spontaneous resolution was reported in patients with mild symptoms or in young ages (24 months old or less).1 Most authors recommend surgical treatment, particularly if trigger finger persists after conservative treatment.3-5 Ultrasound scan has not proven a useful tool for predicting the site of triggering pre-operatively.3Multiple aetiologies causing congenital trigger finger have been reported including anatomical variation, trauma, viral infection, inflammation,et al.3 reviewed literature and found that the predisposing anatomic problem can either be over crowding of the contents of the sheath  or a narrow pulley system . In our case, thickening A1 pulley was noted in both fingers. In addition, fusiform swelling of FDP of middle finger and adhesion between FDS and FDP of ring finger was found. Release of A1 pulley and partial A2 pulley lead to correction of deformity of both fingers. Thus surgical release of multiple trigger finger is different than in pediatric trigger thumb and adult trigger finger release. In pediatric trigger thumb and adult trigger finger release of A1 pulley is sufficient for correction of deformity. A more extensive surgical exposure is required in these cases to confirm release of all structures responsible for deformity. The operative algorithm given by Schaverien et al.3 suggests a step-wise approach to check all structures that may cause the symptom of triggering, whilst avoiding unnecessary dissection. With help of this algorithm we were able to achieve complete correction in this child.Schaverien The authors declare no conflict of interest.


Unilateral lower abdominal pain and/or sciatic nerve pain is a common presentation in the elderly population. The prevalence of broad ligament leiomyoma is <1 % with the prevalence declining after the menopause and it is rare for broad ligament leiomyomas to be clinically significant. Thus, we highlight a case of symptomatic broad ligament leiomyoma in a postmenopausal woman whose symptoms improved after definitive treatment.A 62-year-old postmenopausal Macedonian woman was referred to our gynecological department with unexplained pain in her left leg and left iliac fossa region on walking. There was minimal relief with increasing analgesia use prescribed by the family physician. Investigations revealed an ipsilateral adnexal mass and subsequent treatment with laparoscopic broad ligament myomectomy helped to alleviate her symptoms.Our case highlights the importance of staying mindful of alternate diagnoses when presented with a common presentation of iliac fossa pain and pain in the leg. Although broad ligament leiomyomas are benign tumors, the uncommon symptomatic presentation led us to report and focus some attention on this type of tumor. Pathology of the broad ligament is rare and the prevalence of broad ligament leiomyoma is <1 % . While lA 62-year-old Macedonian secundipara woman was referred to our gynecological department for 2-year history of unexplained left iliac fossa pain and pain in her left leg on walking. Our patient was 15 years postmenopausal with no history of hormonal replacement therapy use. There had been no symptoms referable to the bladder or bowels or abnormal per vaginam bleeding. Our patient’s family and surgical history was unremarkable. The family physician has attributed her pain to lumbar canal stenosis based on clinical examination and subsequently prescribed increasing doses of analgesia with minimal effect.On physical examination, there was minimal abdominal tenderness. A speculum examination showed atrophy consistent with postmenopausal status. A bimanual pelvic examination revealed a non-tender, mobile mass in the left adnexal region with no other signs suggestive of malignancy.Her serum level of cancer antigen (CA)-125 was 5 U/mL. A computed tomography (CT) scan of her abdomen and pelvis revealed a solid well-defined mass in the left adnexa. Her liver, pancreas, spleen, and adrenal glands were normal and no ascites or lymphadenopathy was noted. There was lumbar canal stenosis at L4 and L5 levels, more severe on the right than the left, though this did not seem to explain our patient’s symptoms. Following the CT scan findings, a pelvic ultrasound (US) was organized and showed a 9 × 6.3 × 4.9 cm solid mass in the left adnexal region.Our patient underwent laparoscopic myomectomy; the mass originated from the left broad ligament of the uterus. Intraoperatively, there was no connection among the uterus, the left ovary, and the mass. On pathologic examination of the specimen, the tumor was diagnosed as a large 93 × 58 × 40 mm leiomyoma with no significant surrounding non-neoplastic tissue.Our patient is of interest for a number of reasons. Her age of 62 years with postmenopausal status is unusually late for patients with broad ligament or other gynecologic leiomyomas. In a postmenopausal woman, the presence of an adnexal abnormality raises the possibility of malignancy such as leiomyosarcoma  or ovariFurther, symptoms of leiomyoma are typically those of abnormal uterine bleeding, menstrual disturbances, pelvic pain, and pressure symptoms such as bloating, urinary frequency or bowel disturbances . UnilateSurgically, laparoscopic broad ligament myomectomy is difficult to perform for leiomyomas lying low on the sacrum as these may receive sacral blood supply. Although prospective randomized trials have shown that complication rates for laparoscopic myomectomy are comparable to abdominal myomectomy , 8, thesUnilateral lower abdominal pain or sciatic nerve pain is a common presentation in the elderly population. Although broad ligament leiomyoma is a rare cause of such presentation, our case highlights the importance of staying mindful of alternate diagnoses.Postoperative course was uneventful and at 6 months postoperative review, she reported alleviation of her symptoms after surgery. Our patient was grateful for the investigations and procedures performed. She felt she was listened to and was not just “another lady with chronic back pain”.


We describe a case of bowel strangulation caused by massive peritoneal adhesion as a result of effective chemotherapy. A 71-year-old man, who had obstructive descending colon cancer with massive peritoneal metastases and, therefore, received palliative surgery consisting of diverting colostomy and sampling of peritoneal nodules, developed bowel strangulation on day 4 of the 2nd course of chemotherapy, including irinotecan, l-leucovorin, and 5-fluorouracil. Emergent celiotomy showed a massive intraperitoneal adhesion formed around several intestinal loops, which were not observed at the prior surgery. One loop was strangled, but recovered by adhesiotomy alone. Intestinal loops were formed around aggregates of peritoneal nodules as the centers, several of which were then sampled. We closed the abdomen after all intestinal loops were eradicated by total enterolysis. Fortunately, the patient has been doing well and received chemotherapy without recurrent bowel obstruction 10 months after the present episode. Histological findings of the aggregates causing intestinal loops demonstrated extensive necrosis of cancerous tissue surrounded by fibrosis with abundant lymphocyte infiltration. These findings were not observed in the specimen sampled before chemotherapy, suggesting that intestinal loops were caused by inflammatory adhesion occurring around the peritoneal metastases as a result of effectiveness of chemotherapy. Up to the 1990s, colorectal cancer patients with peritoneal metastases were considered terminally ill and, therefore, these patients generally received only best supportive care in many situations. Median survival time (MST) of these patients was reported to be approximately 6 months, and efficacy of chemotherapy for colorectal peritoneal metastases (CPM) was reported to be disappointing, with 7-9 months of gained MST . AccordiWe herein report a case of bowel strangulation due to massive intraperitoneal adhesion as a result of effectiveness of chemotherapy for multiple peritoneal metastases originating from colorectal cancer.A 71-year-old man was seen at our hospital for complaints of abdominal fullness and a hard large tumor identified palpably in the left lower abdomen. He had a history of receiving surgery for rectal cancer, which was performed more than 10 years previously. After careful workups, descending colon cancer, causing bowel stenosis and multiple peritoneal metastases, was diagnosed  was started on postoperative day 7 and given biweekly. Any severe adverse effects, such as watery diarrhea, refractory constipation, or uncontrollable nausea, were not found other than mild anorexia until day 4 (25 days after surgery) of the 2nd course of FOLFIRI, when the patient developed sudden abdominal fullness causing epigastric pain and vomiting. Findings of abdominal X-ray and CT suggested intestinal obstruction Fig. . AdhesioHistology findings of peritoneal nodules sampled at the second urgent surgery showed that cancerous tissue was nearly eradicated and fibrous tissue was formed surrounding the peritoneal nodules Fig. . FurtherFortunately, the patient has been doing well and receiving chemotherapy with targeted agent (bevacizumab), which was added from the 4th course, 10 months after the diagnosis of descending colon cancer without recurrent intestinal obstruction. At this stage, efficacy of chemotherapy was judged to be partial response of Response Evaluation Criteria in Solid Tumors version 1. 1 .During last 15 years, markedly effective cytotoxic chemotherapies and biological targeted agents were developed and prognosis of patients with unresectable colorectal cancer has been dramatically improved. However, although peritoneal metastasis is the second most frequent distant metastasis of colorectal cancer following liver metastasis, treatment outcomes of patients with CPM were dismal in the previous decades when no effective drugs were present other than 5-fluorouracil –10, 12. Based on the comparison of histological findings between peritoneal nodules sampled before and after the induction of chemotherapy in the present case, bowel strangulation was considered to result from the effect of chemotherapy, which was essentially beneficial, but not adverse. It was reported that tumor cell death induced intra- and/or peri-tumoral inflammatory cell infiltration, mainly composed of lymphocyte, for scavenging necrotic tumor tissue , 15. In


A 22-year-old woman with myasthenia gravis (MG) presented with ptosis and mild muscle weakness symptoms for one year. Computed tomography (CT) presented a diffuse bilobulate enlargement gland with a high density of soft tissue. Magnetic resonance imaging (MRI) showed the gland with no suppression on the opposed-phase chemical shift. After the thymic tumor diagnosis, she underwent thoracoscopic surgery for tumor resection. The postoperative histopathological finding was thymic lymphoid hyperplasia. This case suggests chemical shift MRI is not enough in distinguishing, and supplementary examination is essential to avoid unnecessary thymic biopsy and surgery. Thymic lymphoid hyperplasia (TLH) is very common in patients with myasthenia gravis. Contrary to true thymic hyperplasia, thymic lymphoid hyperplasia has diverse types and shapes. It can exhibit a normal shape and size, diffuse enlargement of both lobes, or a focal soft tissue mass . TherefoA 22-year-old woman had ptosis and mild muscle weakness symptoms for one year. The symptoms were mild in the morning, severe in the evening, worse on exertion, and improved with rest. She had not diplopia, dyspnea, or dysphagia symptoms. Prostigmine and repetitive nerve stimulation tests were positive. She was diagnosed with myasthenia gravis and treated with corticosteroids and mytelase and her symptoms got better. After CT and MRI examination, she was diagnosed with a thymic tumor and underwent thoracoscopic surgery for tumor resection. The postoperative histopathological finding was thymic lymphoid hyperplasia.CT examination was performed using a 2-section CT system  in a single-breath hold at end inspiration. Technical parameters included 120 kVp, 180 mAs, pitch of 1, section thickness of 5 mm, contiguous section interval, and 512 × 512 matrix without contrast agent intravenous injection. Observation was performed on soft tissue window W350, L100 HU.MRI examination was obtained using a 1.5 T MRI unit . She underwent transverse gradient-echo T1-weighted non-dual-echo in-phase and opposed-phase imaging, using an anterior-to-posterior phase-encoding direction, in separate breath holds. Imaging parameters included 350 mm field of view, 256 × 256 image matrix, 5 mm section thickness, 151 ms time repetition (TR), and in-phase and opposed-phase time echo (TE) of 4.6 and 2.3 ms. She also underwent axial T1-weighted and T2-weighted imaging without and with fat suppression and black blood technique with cardiac gate. Imaging parameters included TR 1000, 2000 ms, TE 10, 60 ms, and section thickness of 6 mm.CT findings showed the relative homogeneous bilobulate gland with a high density of soft tissue (60.7 HU). The left lobe with a thickness of 13 mm was greater than the right lobe. The gland had straight margins. MRI findings presented the gland with an increased homogenous intensity on both T1-weighted and T2-weighted imaging. It was greater than in the wall muscle but lower than in the adipose tissue. Chemical shift magnetic resonance images demonstrated no decrease in signal intensity of the gland on the opposed-phase images relative to the in-phase images, suggesting the absence of fat component. The mean chemical shift ratio (CSR) was 1.0 .On gross examination, the resected thymus weighed 55 g, and the external surface was smooth and lobulated, triangle shaped, and yellowish, with a size of 25 × 20 × 10 mm. A microscopic examination revealed numerous reactive lymphoid follicles with large germinal centers in the thymic medulla. Lobules were separated by a thin fibrous septum and had a little fat tissue. In addition, there were an increased number of Hassall's corpuscles .Myasthenia gravis is an acquired autoimmune disease caused by an immune response in which IgG autoantibodies are produced against the acetylcholine receptors of the neuromuscular junction postsynaptic membrane. The thymic gland plays a very important role in the pathogenesis of MG. Above 90% of myasthenia gravis cases had abnormal thymus including 70% thymic lymphoid hyperplasia and 20% thymoma . DiffereBesides CSR, another index also used to quantify fat tissue is signal intensity index (SII). The SII is often used when chemical shift imaging is obtained by a dual-echo technique. According to Priola et al., reference tissue was not only unnecessary but also incorrect because the tissue may contain a determined amount of fat . The stuAckman et al. presented a pathologically proven case of normal thymus in a 21-year-old woman that demonstrated no fat replacement on the opposed-phase chemical shift MRI (CSR = 1.1) . Priola −3 mm2/s and 2.47 × 10−3 mm2/s [−3 mm2/sec with Se 96.8% and Sp 79.2% [With thymic hyperplasia cases without fat as our case, Priola's studies have suggested that diffusion-weighted MRI could be valuable because of its capability to reflect cell density and cellular architecture and to detect malignant tissues by demonstrating restricted diffusion and low (ADC) values. He reported two thymic hyperplasia cases without fat that was not detected on chemical shift imaging but was found on diffusion-weighted MRI due to the high ADC values 1.97 × 10−3 mm2/s , 10. HisSp 79.2% . HoweverSp 79.2% , 14, UsuSp 79.2% , Seki etSp 79.2% , and GümSp 79.2%  always uIn thymic hyperplasia, histopathological characteristics revealed numerous reactive lymphoid follicles with prominent germinal centers in the thymic medulla. In adults, both normal thymus and thymic hyperplasia contain a great amount of fat tissue. However, in this case, postoperative histopathological findings showed only a few fat cells, which was not sufficient to detect the decrease of signal intensity on chemical shift MRI .In conclusion, the normal and hyperplasia thymus glands present great fat infiltration. Conversely, thymoma does not show adipose tissue. Chemical shift magnetic resonance imaging is helpful in differentiating thymic lymphoid hyperplasia from thymic neoplasm. However, in a few cases, especially in young women, the chemical shift magnetic resonance imaging is not enough in distinguishing. Diffusion-weighted MRI and supplementary examination in these cases are essential to eliminate unnecessary thymic biopsy and thymectomy.


We present a 48-year-old caucasian woman presenting with anal pain, discharge and difficulty in defecation due to recurrent Buschke-Lowenstein tumor with rectal involvement discuss it in the light of literature. A 48-year old caucasian woman was referred to our institute with anal mass causing pain, discharge and difficulty in defecation. She initially had simple excision and electrocoterisation 3 and 15 years before at different centers. At physical examination, multiple vegetative mass lesions presented as a cauliflower-like tumor were seen at perianal region. Colonoscopy showed an inflamated, vegetative mass covering all mucosa annularly and starting from 2 cm away from anal verge and reaching until 20 cm was seen. Due to the large extent of tumor invasion in this case, curative surgery would have been achieved only by wide local surgical excision and abdominoperineal resection due to rectal involvement. This severe mutilation was refused by the patient. Thus, patient was referred to medical oncology for radiochemotherapy. Wide radical excision of Buschke-Lowenstein tumor (BLT) is the preferred treatment for achieving local control, but excision alone often is ineffective treatment. Abdominoperineal resection is necessary in cases with infiltration involving the sphincter muscles or rectum, especialy for recurrent cases. A Buschke-Lowenstein tumor  was first described by Buschke and Loewenstein as penile condyloma like carcinoma without true microscopic invasion findings . It may In this article, we present a 48-year-old caucasian woman presenting with anal pain, discharge and difficulty in defecation due to recurrent BLT with rectal involvement which was locally excised 3 and 15 years ago in different centers and discuss it in the light of current literature.A 48-year old caucasian woman was referred to our institute with anal mass causing pain, discharge and difficulty in defecation. This lesion had been increasing slowly for more than sixteen years. She initially had simple excision and electrocoterisation 3 and 15 years before at different centers. At physical examination, multiple vegetative mass lesions presented as a cauliflower-like tumor were seen at perianal region, the biggest one sizing 5 x 3 cm, causing multiple perianal fistula formation , it was BLT is a rare sexually transmitted disease, triggered by human papilloma virus (HPV), usually genotype 6 or 11 . They haBLT has been reported to transform into malignant cancer in 30-50% of patients. It remains unknown which viral factors  or host factors  may affect the oncogenic potential of HPV to cause the giant condyloma to turn squamous cell carcinoma (SCC). The mean time of malignant transformation from benign BLT to histologic malignancy is approximately five years . DiffereThere are still many problems relevant with the treatment for BLT because of the high recurrence rates (up to 50%) and the lack of adequate series of patients following the same procedure . SurgicaRadiation therapy and topical or systemic chemotherapy without surgery have also been used with minimal response, and should only be considered as palliative treatment in patients that present with nonresectable tumours , 6. SomeThe most important problems with the treatment of BLT are recurrence after inadequate surgery, anorectal involvement and malignant transformation; as it is mentioned in the literature and as in our case. In our patient, the initial simple excision and electrocoterisation by the first surgeon was inadequate, which caused recurrent BLT twelve years later. Another surgeon (gynecologist) performed local excision three years ago.As a result, wide radical excision of BLT is the preferred treatment for achieving local control, but excision alone often is ineffective treatment for BLT. Abdominoperineal resection is necessary in cases with infiltration involving the sphincter muscles or rectum, especialy for recurrent cases.


Steatocystoma multiplex is a rare genetic disorder characterized by the presenceof hamartomatous malformations at the junction of the pilosebaceous duct. Itconsists of encapsulated cystic lesions in the dermis, with adjacent sebaceousgland. When associated with inflammation, resembling hidradenitis, it is calledsteatocystoma multiplex suppurativa, a condition rarely reported. This is thefirst case of steatocystoma multiplex suppurativa reported in the Brazilianliterature. Female patient, 23 years old, with papular and nodular cysticlesions that started in the armpits and groin, later spreading to the trunk,lower limbs, anticubital fossa, face and scalp. The presence of papular-nodularlesions associated with disseminated hidradenitis-like lesions in flexural areasand the histopathological diagnosis of steatocystoma defined the diagnosis ofsteatocystoma multiplex suppurativa. Rarely, it is found on the scalp and face.7 Lesions vary from normochromic toyellow, are movable, with slow growth and liquid content most of thetimes.4Steatocystoma multiplex (SM) is a rare genetic disorder.2 Most cases have autosomal dominant feature, but it can alsooccur sporadically.8SM appears in adolescence or early adulthood, with no predilection for sex.6 In this case, thereis spontaneous rupture of the cysts, suppuration and malodorous drainage, especiallyif secondarily colonized by bacteria.9 Abscesses can also occur. These findings resemble tolesions of acne conglobata or hidradenitis suppurativa.9 The lesions can progress to scars, causing muchdiscomfort to the patient.9Although it is a benign condition and most of the lesions are asymptomatic, there isan inflammatory variant called SM suppurativa.,Female patient, 23 years old, reported that at 11 years she noticed the appearance ofpapules and nodules in the axillary regions  and thenFigures27 The origin of cyst formation is not completelyknown. The familial form may be related to mutation in the keratin 17, a keratintype 1 found in the sebaceous glands and hair follicles.4 The same mutation is found in pachyonychia congenitatype 2 (PC-2), with which SM may be associated, a dominant autosomal disorder thatpresents with nail dystrophy, palmoplantar keratoderma, oral leukoplakia, follicularkeratosis and epidermal inclusion cysts. 8 In addition,SM can manifest with hypertrophic lichen planus, acrokeratosis verruciformis, natalteeth and other findings.8SM is a hamartomatous malformation of the junction of the pilosebaceousduct.10There are phenotypic variations within the same family, as in the case of ourpatient, and there may be overlap among subtypes. The same mutation in the keratin17 gene may manifest with SM or PC-2 alone or in association. More than 11 distinctmutations are described; however, the resulting phenotype is independent of the typeof mutation. It is possible that these manifestations are spectra of the samedisease.3The largest case series of SM comprises 64 patients with clinical, demographic andhistopathology analysis. It has been demonstrated that most of the cases weresporadic, with mean age of 26 years, and the most affected sites were the arms (35%)and chest (29%).6but they were absent in the present case.Symptoms such as pain, itching and fever may occur in the suppurative form. Theevolution of SM to the suppurative form is rare and can happen at any time duringthe course of the disease. Reports of serious infections can result due to lowsocioeconomic status and poor adherence to treatment. Cystic lesions are oftenmovable, with consistency ranging from firm to elastic. Nail disorder, such ashypertrophy, occur in most cases,6In addition to SM suppurativa, there are other reports of rare variants of SM, suchas the facial, acral, vulvar and steatocystoma simplex (single lesion).In the case reported, although no genetic evaluation was performed, the patientprobably had mutation in the keratin 17, considering it had a positive familyhistory. Furthermore, it is a rare case, because it presented suppuration in limbsassociated with facial and scalp lesions.4 SM suppurativa must be distinguished from severenodulocystic acne, acne conglobata, infected fibroadenoma, and pyoderma.6The clinic features of SM can resemble a number of diseases: vellus cysts, myxoidcyst, milia, acne conglobata, hidradenitis, and pseudofolliculitis, delaying thediagnosis and the proper monitoring.1being more accessible than the genetic evaluation, in addition to presentingpeculiar characteristics.To avoid delay in diagnosis of this condition, it is more feasible to proceed theinvestigation with histopathology,2Histologically, the steatocystomas are dermal cysts coated with typical stratifiedsquamous epithelium, whose stratum corneum shows wavy appearance, refractive andstrongly eosinophilic. Sebaceous glands are usually present in the cyst wall, andhair may occur in its cavity.2 and Erbium YAG, cryotherapy or oral isotretinoin arepotential therapies options.5The treatment is varied and, in general, unsatisfactory, because of the difficulty ofapproaching such disseminated lesions. Surgery, needle aspiration, use of laserssuch as CO6 Use of oralisotretinoin for the treatment of SM has been reported with mixed results. Mostresponsive patients had SM suppurativa.9 Isotretinoin usually does not eradicate this condition, butdecreases the size of suppurative lesions.6 This therapeutic response probably reflects theanti-inflammatory effect of retinoids.9Inflamed lesions can be addressed with injection of corticosteroids ordrainage.2 We should be alert to SM suppurativaand all the social and psychological implications it entails, seeking diagnosing asearly as possible, clarifying the benign nature of the lesions, and offeringavailable therapeutics.These therapeutic modalities may be used in isolated form or in combination,according to the availability, clinical manifestations and patient’s wish, sincemost patients seek dermatologic service for esthetic demands.


Recently, gastrointestinal stromal tumors that have developed outside of the digestive tract have been reported. These tumors are collectively termed extra-gastrointestinal stromal tumors. Extra-gastrointestinal stromal tumors can also develop in the liver. Only eight case reports involving primary GIST of the liver have been published. We report a case and review the literature regarding this disease.18F-Fluoro-2-deoxyglucose positron emission tomography revealed no other malignancies except for the liver tumor. Because the lesion was suspected of being a primary malignant hepatic tumor, lateral segmentectomy was performed. The immunohistochemical analysis supported the diagnosis of gastrointestinal stromal tumors in the liver. The patient has had no evidence of recurrence during the 10-month follow-up period; imatinib chemotherapy was not administered.A 70-year-old woman with a past history of gastric cancer visited our hospital for regular inspection. With extensive radiological imaging, a computed tomography scan revealed a mass with a size of 6.8 cm in the lateral segment of the liver. Primary hepatic gastrointestinal stromal tumors had no characteristics that distinguished them from ordinary tumors in imaging examinations. Primary gastrointestinal stromal tumors might have developed from interstitial Cajal-like cells. Gastrointestinal stromal tumors (GISTs) are thought to arise from the interstitial cells of Cajal (ICCs) located in the gastrointestinal mesenchyme. The current diagnosis of GISTs is based on histological and immunohistochemical criteria, the most important of which is the expression of the receptor tyrosine kinase, KIT CD117, c-Kit). GISTs are typically found in the gastrointestinal tract, including the stomach, small intestine, colorectum, and esophagus 17, c-Kit. The numFrom around the year 2000, many groups have become interested in whether or not ICCs are present outside of the gastrointestinal tract; peculiar interstitial cells have been found in the upper and lower urinary tracts, blood vessels, pancreas, and other sites. Such cells have been named interstitial Cajal-like cells (ICLCs) . FurtherA Japanese female patient underwent distal gastrectomy with regional lymph node dissection for gastric cancer when she was 63 years old. The cancer was a poorly differentiated adenocarcinoma with serosa invasion, and lymph node involvement was found. However, no liver tumor was detected during radiological examination at the initial operation. In addition, extensive microscopic examination of the resected stomach revealed no GISTs, with the exception of gastric adenocarcinoma.18F-Fluoro-2-deoxyglucose (18F-FDG) positron emission tomography (PET) was performed to rule out the presence of metastases throughout the body. The hepatic mass showed 18F-FDG uptake with a maximum standardized uptake value of 6.3; however, no other sites of hot 18F-FDG uptake were demonstrated  scan revealed a 6-cm mass in the left lateral segment of the liver, and peripheral enhancement in the arterial phase with a heterogenous appearance Fig. . The lefThe patient underwent left lateral segmentectomy of the liver because of the possibility of a primary hepatic malignancy. Intraoperative findings indicated that the hepatic tumor was palpable with a hard elastic consistency and had no lymph node involvement; no gastrointestinal tumor was found in the abdominal cavity. The hepatic tumor and the adjacent small intestine adhered firmly to each other, and thus, combined resection was performed. On gross evaluation, the hepatic tumor was well circumscribed and measured 6.8 × 5.5 cm. In cross section, the tumor had a whitish-tan color to the cut surface and focal necrotic and cystic portions Fig. . No maliMicroscopically, the hepatic tumor was composed of spindle cells with pleomorphic nuclei arranged into short fascicles. The mitotic count was 35–40 mitoses per 50 high-power fields (HPFs) Fig. . ImmunohAdjuvant therapy such as imatinib was not administered continuously because of adverse events involving general malaise and anorexia. The patient recovered well from the side effects and exhibited no evidence of recurrence during the 10-month follow-up period.No other tumors except for the hepatic tumor were identified pre- and intra-operatively in the present case; moreover, no neoplastic lesions were identified after surgical treatment without chemotherapy. We made a final diagnosis of primary hepatic GIST.GISTs are specific mesenchymal tumors located in regions of the gastrointestinal tract such as in the stomach (60–70 %), small intestine 20–25 %), colon and rectum (5 %), and esophagus (<5 %) 0–25 %, c–16. They18F-Fluorodeoxyglucose positron emission tomography (18FDG-PET) was performed in three patients, and high accumulation of 18FDG was found in their hepatic tumors. All patients had undergone surgery, with the exception of only one who had been treated with radiofrequency ablation. Morphologically, GISTs can be subdivided into spindle cells, epithelioid cells, and mixed types. Seven of the nine cases were the spindle cell type, one was the epithelioid cell type, and one was the mixed cell type.All hepatic GISTs demonstrated hypoattenuation on CT scans. MRI was performed for three patients, but no characteristic findings were obtained. The mitotic count in the tumor ranged from 0 to 75 per 50 HPFs, with a median of 20 per 50 HPFs. Fletcher et al.  proposedFour patients had received imatinib chemotherapy, one patient underwent adjuvant therapy, two of three patients with recurrence were effectively treated and remained alive for 36 and 66 months, and the other patient died of the disease. Patients with recurrent tumors could be expected to achieve long-term survival if they had received chemotherapies continuously.The present case can be considered a primary hepatic GIST because of the following two findings. First, preoperative gastroscopy and colonoscopy imaging studies and extensive pathological examination of the resected stomach failed to detect any other GISTs, except for a hepatic tumor. Second, there was no evidence of recurrence and no neoplastic lesions after hepatectomy in the absence of adjuvant chemotherapy. ICCs are normally present in the myenteric plexus of the gastrointestinal tract, and they have not been identified in the liver. Consequently, is it possible that primary GISTs can occur in the liver? We initially suspected that primary hepatic GISTs might originate from the biliary system in the liver. Ahmadi et al. reported that ICCs were identified in the extrahepatic bile ducts ; unfortuPrimary GIST of the liver is very rare, and only eight cases have been previously reported. Primary hepatic GISTs had no characteristics to distinguish them from ordinary ones on CT and MRI scans and also regarding histopathological findings. Primary hepatic GISTs might have developed from ICLCs, but further investigations are necessary.


Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor of intermediate biologic potential. Because of its rarity and nonspecific radiological and diverse pathological findings, AFH is often clinically misdiagnosed. However, few clinical reports have described this tumor. As reported herein, we analyzed the clinical and radiological features and clinical outcomes of AFH.We retrospectively reviewed the medical records of seven cases histopathologically diagnosed as AFH. We examined clinical features, MRI findings, histopathological diagnoses, treatments, and outcomes.These seven cases comprised five male and two female patients with ages ranging from 8 to 50 years old. The primary locations included upper extremities in 2, lower extremities in 4, and the inguinal region in one patient. Of the tumors, 4 occurred in subcutaneous tissues and 3 occurred in deep tissues. No cases were diagnosed as AFH from MRI and needle biopsy results. All cases were diagnosed histopathologically after excision. After treatment, 2 patients (29%) had tumor recurrence and metastasis, one of whom died from disease progression. These 2 aggressive cases involved both EWSR1 and CREB1 gene rearrangements as determined by FISH. The other patients were alive and well without recurrence or metastasis.AFH is a rare tumor that is difficult to diagnose. Therefore, it tends to be misdiagnosed and to be treated inadequately by referring physicians. Surgeons must therefore be mindful of the presence of AFH, learn about appropriate treatment necessary for this tumor, and conduct careful follow-up because AFH can engender poor outcomes. Angiomatoid fibrous histiocytoma (AFH), a rare soft tissue neoplasm, was described initially as “angiomatoid malignant fibrous histiocytoma” by Enzinger in 1979 . AFH oftWe retrospectively reviewed the medical records of seven cases histopathologically diagnosed as AFH at two institutions. All histopathological specimens were examined by a soft tissue pathologist to confirm the diagnosis Figs.  and 2. AClinical features related to gender, age, tumor size and site, and symptoms were obtained Table . FurtherThese seven cases were composed of five male and two female patients with an age range of 8 to 50 years . The primary locations were upper extremity in two patients, lower extremity in four patients, and the inguinal region in one patient. Regarding the tumor sites, four patients had subcutaneous lesions and three had deep lesions. Five patients presented with a painless mass, whereas one presented with a painful mass. Only one patient (case 5) had no symptoms. However, the mass had been occasionally noted as an abnormal finding after medical examinations. The average and median sizes of the tumors were 69 mm and 45 mm range, 10–303 mm), respectively : these diagnoses included myxoid liposarcoma, Ewing’s sarcoma, myxofibrosarcoma, and synovial sarcoma. Case 4 was initially diagnosed as a spindle cell tumor. The diagnoses of these five cases (cases 2–6) were amended to AFH after pathological review or additional wide resection. Only two cases were correctly diagnosed as AFH by initial excisions  to detect the rearrangement of EWSR1 or FUS and reverse transcription-polymerase chain reaction (RT-PCR) to elucidate EWSR1-CREB1, EWSR1-ATF1, or FUS-ATF1 fusion transcripts , 17, 20.A summary and comparison of the clinical outcomes of AFH cases described in earlier reports are presented in Table Costa et al. reported that an irregular tumor border as well as a head and neck location were associated with local recurrence, and the depth of the tumor was correlated with subsequent local and distant metastasis . FurtherNot only is AFH a rare soft tissue neoplasm, but its diagnosis is often difficult because of the lack of specific clinical symptoms and radiological and histopathological features. In our series, AFH occurred in older patients and was found in deep lesions. Although it appears that patients with AFH have excellent prognoses, the rates of local recurrence and metastasis might be higher than initially expected. Surgeons must therefore be aware of AFH and include it in clinical, radiological, and histological differential diagnoses.


Epigastric pain is a common complaint made by patients being evaluated in the emergency department. Spontaneous isolated visceral artery dissection is a rare cause with no reported prevalence. We present a case of a 37-year-old male evaluated in the emergency department for epigastric pain and subsequently diagnosed with a spontaneous isolated celiac artery dissection with involvement of the hepatic and splenic arteries. Recent case series suggest this disease may be managed medically in most cases. Surgical intervention may be considered for significant bleeding or signs of intestinal ischemia. A 37-year-old Caucasian male presented to an emergency department with worsening epigastric pain of 4-day duration. He stated that his pain initially began as indigestion while he was leaning forward getting out of bed and progressed to a constant aching pain with associated nausea. He stated that bending forward exacerbated the pain. He denied prandial pain, melena, hematochezia, or recent change in bowel habits. History was notable for hypertension which was being managed without need for antihypertensive medications. Surgical history was not significant for any prior abdominal surgeries. His social history was significant for tobacco abuse which consisted of a half pack of cigarettes a day for 25 years. Initial vital signs were significant for hypertension. Physical exam on presentation was significant for minimal epigastric tenderness with normoactive bowel sounds without rebound or guarding. Lab analysis was not significant for elevated lipase, liver function abnormalities, anemia, or electrolyte abnormalities. Computed tomography (CT) of the abdomen and pelvis with IV contrast was performed which showed a filling defect in the celiac artery. Due to concern for dissection, a CT angiogram of the abdomen and pelvis was performed. The CT angiogram showed a 40.1 mm dissection from the celiac origin into the hepatic artery as well as a 37.9 mm dissection from the celiac origin into the splenic artery (Figures The patient was admitted for observation, given 1000 mL unfractionated heparin bolus, and started on an unfractionated heparin infusion. The patient was evaluated by vascular surgeons and gastroenterologists. When given the choice for stenting the lesion for symptomatic control, he elected not to have the lesion stented. Prior to discharge he was started on 81 mg aspirin daily and a follow-up appointment with repeat CT angiography was scheduled in 2 months. Follow-up CT angiogram two months after his initial diagnosis showed minimal enlargement of the dissection. At that time during his vascular surgery follow-up, the patient stated improvement in his abdominal pain. At that time he was scheduled for a 6-month appointment and follow-up CTA.Visceral artery dissection typically occurs as a complication of an extension of a descending aortic dissection. However, spontaneous isolated visceral artery dissection has been described in the literature. The prevalence and etiology of this disease are unknown as it is uncommon and the literature consists only of case reports and case series. Spontaneous dissection of the superior mesenteric artery is most common, followed by celiac artery dissection –3. PatieThe most common sign of spontaneous visceral artery dissection is acute onset epigastric or abdominal pain not preceded by trauma. Associated symptoms may include nausea, vomiting, or diarrhea –8. PatieComplications of visceral arterial dissection include intestinal ischemia or infarction from stenosis of the true lumen, aneurysm formation due to vessel wall degeneration, and vessel rupture. Initial conservative medial management with anticoagulation and close follow-up can be applied in the absence of bowel ischemia or vessel rupture. Anticoagulation is thought to prevent vessel thrombosis and embolization. Surgical management with endovascular stenting can be used in patients with persistent abdominal pain. Open surgical management is rare and is typically used when the dissected vessel has ruptured.


Endometrial stromal sarcomas arising in extrauterine and extraovarian sites, in the absence of a primary uterine lesion are quite rare, especially in the absence of endometriosis. They usually present as an abdominal or pelvic mass lesion.In 2007, a 45-year-old woman underwent total hysterectomy for in situ squamous cell carcinoma of the cervix. In 2014, an upper left pulmonary lobectomy was performed for a mass, which was provisionally diagnosed as primary carcinosarcoma of the lung. A second histological revision of the lung surgical specimen was performed in the Pathology Unit of our Institute. After extensive immunohistochemical analyses, the preferred diagnosis was spindle-cell sarcoma, consistent with high-grade extragenital endometrial stromal sarcoma (EESS). A review of all slides of the hysterectomy specimen confirms the original diagnosis: no evidence of stromal tumor was found. Afterwards, the patient developed multiple and metachronous pulmonary lesions and a scapular soft tissue mass, which showed the same morphophenotypic features of the first lung mass. The patient was treated with antiblastic therapy, surgical resection and radioablation, when appropriate. To date, the patient has no signs or symptoms.The authors present the first case of primary EESS arising in the lung with no association with endometriosis published to date. Detailed clinical history and follow-up are also described. Moreover, extensive literature review is reported, along with differential diagnoses, immunohistochemical and molecular findings, pathogenetic hypotheses and treatment options. The knowledge of EESS potential extrauterine location and of its peculiar morphophenotypic aspects are required for a correct diagnosis, and for choosing the most suitable treatment. Endometrial stromal sarcoma (ESS) is an uncommon mesenchymal tumor of the uterus, which accounts for less than 1% of all uterine malignancies but it is the second most common uterine malignant mesenchymal tumor . In contEndometrial stromal sarcomas arising as primary tumors in extrauterine and extraovarian  sites are quite rare, and reported in the English medical literature as small series or case reports Table  3–29].T–29.T3–29The clinical-pathological features of primary EESS have not been widely investigated yet. To the authors’ knowledge, this is the first case reported of EESS arising in the upper left lobe of the lung in a 45-year-old woman without associated endometriosis and in the absence of a primary genital ESS. An extensive review of literature is also reported, along with differential diagnoses, immunohistochemical and molecular findings, pathogenetic hypotheses and treatment options.In 2007, a 38-year-old female was hospitalized for an ulcerated uterine cervical lesion which, after cervical biopsies, showed an in situ squamous cell carcinoma. Subsequently, an abdominal CT scan revealed an enlarged, dishomogeneous uterus, with hypodense mass of 5 cm adjacent to the posterior wall of the bladder. Two months later, the patient underwent total abdominal hysterectomy with bilateral salpingectomy and pelvic lymphadenectomy. In the intraoperative phase, the uterus was found to be mobile and ovaries were normal. Neither enlarged lymph nodes nor peritoneal lesions or ascites were noted.On histological examination of the surgical specimen, a well-differentiated squamous cell carcinoma in situ of the cervix, measuring 4 mm in maximum diameter, with basaloid morphology, was identified. Three additional leiomyomata of the uterine corpus, besides the larger one already identified on CT scan, were also microscopically found.In 2014, the patient presented to medical observation with a pulmonary mass  on CT scan. After multidisciplinary discussion, a surgical intervention  was performed.Almost three months after surgical intervention, a new pulmonary lesion located in the right lung was detected by CT scan. The lesion was subsequently treated with radiofrequency ablation. As a consequence of the procedure, right pleural effusion after 24 h and after five days by the appearance of right pneumothorax after five days appeared. Shortly after tube insertion, additional radiographs were taken: they showeda rapid decrease in the size of the pneumothorax. The thoracic drainage was removed and patient was discharged from the hospital.After one month, a CT scan was repeated. The scan showed surgical and radiofrequency ablation effects and a new pulmonary lesion of uncertain nature, measuring 4–5 mm of maximum diameter. Hormonal therapy with progestin (acetate medroxyprogesterone 1 gr daily) was prescribed.No radiological signs of progression were noted until December 2015, when new lung lesions located in the lower lobe of the right lung and upper lobe of the left lung were detected on CT scan.In January 2016, the patient referred the appearance of a soft tissue mass, rapidly enlarging, located in the left periscapular region. A tru-cut biopsy of the soft tissue lesion was performed: histological examination revealed a neoplastic lesion with spindle cell morphology, reminiscent of the upper left lobe EESS.A second line polychemotherapy with epirubicin and ifosfamide was started. After three courses of chemotherapy, radiological evaluation through CT scan revealed a decrease of the diameter of the right lung lesion and the disappearance of the lesion in the left lung. At the follow-up visit, the patient was in good conditions well, with no clinical signs or symptoms of the disease and no pathological findings on CT scan; routine laboratory tests were normal.Signed written consent was obtained from the patient for this case report.Two and half-micron sections from formalin-fixed paraffin embedded tissue of the resected lobe and of the hysterectomy specimen were cut and immunohistochemical analysis was performed through an automated system . The following primary antibodies were used:CD117 , Estrogen receptor , Progesteron receptor , Ki67 , CD10 , CD34 , CD31 , Pankeratin , MNF116 , smooth muscle actin (SMA) , H-Caldesmon , S100 , MDM2 , D2-40 , CD99 , Myogenin , Myoglobin , Vimentin , Desmin , TTF1 .The color was developed with 3.3′-diaminobenzidine (DAB), and the slides were counterstained with Meyer’s hematoxylin. Appropriate positive and negative controls were run concurrently.The histological examination of hematoxylin and eosin stained slides of the pulmonary lesion showed uniform spindle cells organized in a diffuse pattern Fig. , b, c, wA review of all slides from the hysterectomy specimen, which included the large lesion identified on CT scan plus three additional microscopically found leiomyomas, confirmed the original diagnosis of squamous carcinoma in situ of the cervix with leiomyomas of the uterine wall. No evidence of ESS or endometrial stromal nodules was found.On immunohistochemical staining, the tumor cells showed patchy and intense immunoreactivity for CD10 Fig. , estrogeCD10 was completely negative Fig.  whereas The periscapular soft tissue recurrence showed the same histological and immunohistochemical findings, except for the absence of staining for CD10, which displayed a patchy staining on the lung specimen; therefore, its absence on a tru-cut biopsy of periscapular soft tissues could be due to sampling from a non-staining area.Endometrial stromal sarcoma is a malignant tumor closely resembling stromal cells of proliferative-phase endometrial stromal cells. It is commonly associated with a delicate network of arterioles. Even when displaying classical histological features, an unusual site of occurrence may make the diagnosis challenging. Therefore, despite its rarity EESS should always be taken into consideration in the differential diagnosis for a woman with a thoracic or abdominal tumor with uniform cytologically bland cells resembling normal endometrial stroma. On the other hand, in the gastrointestinal tract and extragenital sites, in the absence of endometriosis EESSs may be mistaken for other mesenchymal more common neoplasms , such asIn the largest group of EESS described to date, one case arising in the larynx was initially diagnosed as monophasic synovial sarcoma  which isde novo from the peritoneal/pleural (in our case) surface or the coelomic or subcoelomic multipotential epithelium [Several hypotheses are invoked to explain the pathogenesis of EESS. Since in many cases Table  endometrithelium , 31.Recent molecular studies showed that ESS can be genetically heterogeneous. The most common alteration carried by ESS is the t  p15;q21) translocation, which results in JAZF1–SUZ12 gene fusion  . However translocThe behavior of EESS, compared to genital ESS, seems to be controversial. Although results are conflicting, it seems that the only feature associated with a worse prognosis is the presence of morphological features suggestive of dedifferentiation .Given the rarity of these tumors, evidence-based data are not available to help guide treatment decisions. Surgery is generally regarded as the cornerstone of treatment for ESS. Complete cytoreductive surgery is recommended, especially in patients for whom this surgery could result in being residual-disease-free. Tumor-free margins are important in prognosis . ExtrageIn summary, we present in this study the first EESS of the lung reported to date. EESS is an uncommon tumor arising in women of any age, usually presents as a mass lesion in the abdomen or pelvic cavity. The knowledge of its potential extrauterine location and of peculiar morphophenotypic aspects are required for a correct diagnosis. Surgical resection is the most frequent treatment option, eventually followed by hormonal-therapy. EESS does not seem to have an aggressive behavior, and is likely to recur late after the initial treatment: therefore, a long-term clinical follow-up should be programmed.


Acral persistent papular mucinosis (APPM) is a rare subtype of localized lichenmyxedematosus. It consists of small papules localized exclusively on the back ofthe hands, wrists and extensor aspects of distal forearms with no other clinicalor laboratory manifestations. The lesions tend to persist and may increaseslowly in number. Histologically, hematoxylin-eosin and Alcian blue stainingdemonstrate mucin accumulation in the upper reticular dermis with separation ofcollagen fibers as a result of hyaluronic acid deposition. Treatment is rarelynecessary due to the absence of symptoms. We present a 27-year-old healthy womanwith asymptomatic papules on her upper extremities, which adequately meetclinical and pathological criteria of acral papular mucinosis. To date, there have been only 34 reported casesof this entity that strictly fulfilled the diagnostic criteria proposed byRongioletti and Rebora in 2001.1Cutaneous mucinosis is a group of disorders charaterized by an accumulation of mucinor glycosaminoglycan in the skin and its annexes.We present a 27-year-old healthy woman with asymptomatic papules on her upperextremities, which adequately meet clinical and pathological criteria for acralpersistent papular mucinosis.Our patient complained of persistent, asymptomatic and symmetrical skin lesions onthe hands and arms, which evolved over a period of one year. She had no history ofmedical problems and reported no use of oral or topical medications. She reported noprevious injuries or trauma to the affected sites and we could establish norelationship with sun exposure. No other family member had been affected.Physical examination revealed approximately 8 small (3-5 mm) firm round skin-coloredpapules located exclusively and symmetrically on the dorsum of the hands and wrists. We iden1 The condition istraditionally divided into two groups: primary mucinosis – in which mucin depositsare the main histologic feature manifested through cutaneous signs; or secondarymucinosis – additional and casual findings in the biopsy specimen of other diseases.The latter group includes: certain endocrinopathies ;toxic diseases (toxic oil syndrome); Eosinophilia-Myalgia Syndrome, nephrogenicfibrosing dermopathy; and diffuse connective tissue diseases .3Cutaneous mucinoses are a heterogeneous group of disorders in which an abnormalamount of mucin is accumulated in the skin and their etiopathogenesis is stillunknown.5 They differentiated two main groups of dermalmucinosis: the generalized form or scleromyxedema, which has a constant associationwith systemic disorders (like paraproteinemia) or less frequently with hematologicmalignancies; and the localized form, which does not have a systemic involvement,also called lichen myxedematosus (LM). Cases not meeting the criteria forscleromyxedema or localized form are classified as atypical.9 Acral persistent papular mucinosis was firstreported by Rongioletti et al. in 19868 as one of the five subtypes oflichen myxedematosus. By definition, the small papules are localized exclusively onthe back of the hands, wrists and extensor aspects of distal forearms with no otherclinical or laboratory manifestations.7 The lesions tend to persist and may increase slowly innumber.8 Histologically, hematoxylin-eosin and Alcian bluestaining demonstrate mucin accumulation in the upper reticular dermis withseparation of collagen fibers as a result of hyaluronic acid deposition. Mucinaccumulation may cause thinning of the epidermis.6 To date, 34 cases have been reported, four of whom presentedwith a past history of malignant tumors.8 Luo etal.8 summarized thereported cases and confirmed a female predominance. The main age at onset was 42.9± 15.9. The possible association with malignancies has not been clarifiedyet. Treatment is rarely necessary due to the absence of symptoms. Topicalcorticosteroids, tacrolimus and pimecrolimus have been used with somesuccess.8The latest classification of dermal mucinosis was established by Rongioletti andRebora in 2001.We presented another case of this rare type of acral mucinosis. It is important toemphazise that it is approriate excluded other systemic or secondary forms thedisease in case of finding any skin accumulation of mucin.


Background: Adrenal incidentaloma refers to the incidental finding of a tumor in the adrenal gland, where nonfunctional forms are the most common variant. Myelolipoma is a rare (0.08-0.4%) occurrence characterized by adipose and hematopoietic tissue. The aim of this case report is to describe the diagnosis and appropriate management of a myelolipoma in an asymptomatic patient, which was originally considered an incidental hepatic hemangioma prior to being identified as a giant adrenal adenoma.Case description: The patient was a 54 year old obese female with a recent diagnosis of diabetes type II and dyslipidemia with recent ultrasound imaging suggestive of a hepatic hemangioma. An MRI was performed revealing a 7x6cm lesion in the right adrenal area indicating a giant adrenal adenoma. An adrenalectomy was performed without complications. The pathology report identified a myelolipoma.Discussion: The incidence of myelolipoma has recently increased due to advances in radiological techniques. Its etiology is unclear and the most accepted theories support a myeloid cell metaplasia in the embryonic stage as a result of stress, infections, or adrenocorticotropic hormone or erythropoietin stimulus. Contributing components may include bone morphogenetic protein 2 and β-catenin, as well as the presence of the chromosomal translocation  . Despite its benign nature, the association with other adrenal lipomas must be ruled out. A biochemical evaluation is essential for detecting subclinical states, such as Cushing syndrome and pheochromocytoma.Conclusion: Adrenal myelolipomas are rare benign tumors that are generally asymptomatic. Uncertainty still exists surrounding their etiology. Surgical management depends on hormone production, tumor size, high risk features on imaging and patient consent.  Additional information is needed to better understand myelolipomas, their etiology, and clinical management.  Incidentalomas may confuse the physician and patient. Ensuring proper multidisciplinary management based on the clinical guidelines of endocrinology allowed a satisfactory resolution of this case. When discussing adrenal incidentalomas (AIs), this refers to a finding of a visible adrenal mass greater than 1cm in diameter found on imaging performed for other medical causes2. In general, adrenal tumors are detected in 0.4% of abdominal ultrasounds and occur with ten times greater frequency in those with a positive cancer history3. Exclusion criteria for AIs include patients who present with manifestations of adrenal dysfunction2 and those with extra-adrenal cancers in the process of stratification4.The term incidentaloma is derived from “3. Incidental adrenal masses are found in 2–4% of abdominal CT scans and the frequency increases in correlation with the patient’s age - adding 0.2% in the third decade of life, up to 7% in those greater than 70 years old4. Among these, non-functional adenoma remains the most frequent (60–85%), while a minority present as functional adenomas (5–16%)5. Of functional masses, 6% consist of pheochromocytomas, 5% are subclinical Cushing Syndrome, 5% are adrenal carcinoma, 2% prove to be a metastasis, and the rest belong to other etiologies, such as myelolipomas, hematomas, cysts, or lymphomas7.Advances in modern diagnostic methods have produced a greater prevalence of AIs, especially due to advances in CT and MRI technology8. According to endocrinology guidelines, both hormonal and radiographic evaluation must be performed in order to rule out subclinical states10. In general, masses ≥4cm are removed surgically, independent of functionality. Furthermore, all functional tumors and those with malignant characteristics undergo an adrenalectomy under endocrinologic supervision. Non-functional adenomas, small myelolipomas, and benign asymptomatic cysts do not require surgical intervention10.Nevertheless, the retroperitoneal location increases the difficulty of detection during a standard physical exam. This often leads to the late diagnosis of such tumors only when clinical systemic manifestation is present - in the case of functional incidentalomas - or the compromise of the adjacent tissues secondary to abnormal gland growth6. Additionally, asking “Are there malignant characteristics?” proves equally important. This is determined by the radiologic imaging that look for heterogeneity, poorly delimited borders, the presence of necrosis, hemorrhage, calcification, or an attenuation coefficient greater than 20 Hounsfield Units7.With this in mind, providers must remember two primary questions, first asking “Is the mass hormonally active?” as this differentiates between functional and nonfunctional massesThis case report describes a giant right upper quadrant incidentaloma in an asymptomatic patient that was initially thought to be a hepatic hemangioma, due to its size and location, which was later confirmed to be an adrenal tumor.A 54 year old asymptomatic female patient was seen by her family physician in Marcaibo, Venezuela, for her annual health exam in January 2014 in a primary care center. She had no complaints, except for recent unintended weight gain. Her past medical and surgical history are notable for a left breast lumpectomy (1973), a salpingectomy (1994), a hysterectomy without oophorectomy for NIC III (2005), and a left unilateral oophorectomy for ovarian torsion (2007). The patient used no medications and has no known allergies, and denied tobacco, alcohol, or drug use. The patient is monogamous and happily married. Her family history is notable for a sister who died of Hodgkin Lymphoma.On physical exam, the patient was afebrile with normal vital signs. Her weight was 92.5 kg, 1.74 meters tall, with a BMI of 30.6. She appeared well hydrated with moist mucous membranes. She had an unremarkable exam - no findings of violaceous striae, acanthosis, acrochordons, or signs of virilization.®, 50/1000mg) 1 tab daily, ezetimibe/simvastatin  1 tab daily, gemfibrozil  1 tab daily, and orlistat  1 cap daily, was initiated.Laboratory results showed a normal complete blood count, mixed dyslipidemia, fasting blood glucose levels >125 mg/dl  on more than two occasions, and HOMA1-IR index >2.5  ; meetingSimultaneously, a right upper quadrant ultrasound was ordered showing slight hepatic steatosis, as well as a round space occupying lesion with well-defined hyperechoic borders measuring 5.6×7.3cm in segment V of the right lobe suggestive of a hemangioma. Of note, a bilateral non-obstructive nephrolithiasis was observed . Due to In April 2014, a right subcostal adrenalectomy was performed in at a level three hospital so as to ensure the presence of an intensive care unit due to the potential bleeding risk. The pathology report described a 4×7×6cm adrenal mass with a grey-yellow surface covered partially with a thick grey capsule with brown areas with a hemorrhagic and yellow adipose center. The microscopic evaluation showed an external layer of clear cortical cells of the adrenal granulosa; a center made of mature adipocytes and all three hematopoietic cell lines without calcifications or fibrosis. The final diagnosis was determined to be an adrenal myelolipoma .The patient experienced no post-surgical complications. She has subsequently completed regular physical activity and continues with the same treatment at the same dosage. Standard laboratory checks at three months showed notable improvement in all parameters.11 and later named by the French pathologist Charles Oberling in 192913. These tumors are metabolically inactive - or nonfunctional - and composed of adipose and hematopoietic cells originating from the adrenal stroma. They are predominantly asymptomatic and tend to be discovered incidentally15.Adrenal myelolipoma is a rare encapsulated benign tumor described first in 1905 by Gierke12, although they comprise 15% of the AIs discovered due to advances in radiographic anchorry13. They frequently present between the fifth and seventh decades of life without a predominance in either sex - though there is a greater incidence in the right adrenal gland15. Though the adrenal location predominates, there have been discoveries in other locations with a preference for the presacral region, and less frequently in gastric, hepatic, ganglionic lymphatics, cranium, and spleen locations16. These statistics are in accordance with this case report.The incidence of these tumors is between 0.08–0.4%17. This metaplasia may occur as a response to necrosis, stress, infections, or prolonged adrenocorticotropic hormone (ACTH) stimulation18. For example, Al-Bahriet al.19 reported a case of a large bilateral myelolipoma in a 39 year old male with a history of congenital adrenal hyperplasia secondary to a 21-α hydroxylase deficiency treated with steroids starting in childhood. This was later stopped during adolescence with a subsequent myelolipoma development - supporting the theory that ACTH stimulation causes adrenocortical metaplasia. Finally, giant myelolipomas usually are associated with hematologic disorders, like hereditary spherocytosis, thalassemia, and falciform anemia, as a response to adrenal stimulation from erythropoietin20.The etiology for adrenal myelolipoma is not clear with numerous theories being proposed. Some suggest a metaplasia of the adrenal and myeloid cells that migrated during embryogenesis, extramedullary hematopoiesis, and embolization of osseous medulla elementset al. described an extremely rare case with the presence of osseous tissue with cells similar to osteoblasts21. Upon immunohistochemical analysis, there were positive results for bone morphogenetic protein 2 (BMP2), which acts as an inductor for osseous formation and the β-catenin that intervenes in the signal pathway. This finding can help give insight into myelolipoma tumorigenesis.Recent cytogenetic analyses propose that myelolipomas are out-of-place masses of myeloid cells. Mitsui18. Because of this, some consider myelolipomas as variants of multiple endocrine neoplasias22, while others recommend that they be grouped with other tumors, such as lipomas, teratomas, liposarcomas, or angiomyolipomas24. Despite its benign characteristics, the pathological studies and immunohistochemical evaluation (not performed due to lack of reagents) was recommended, because of the patient's personal and family history that increased risk for malignant results.Researchers have also identified  chromosomal translocations in patients with myelolipomas and hematological neoplasias25, there may be the coexistence of myelolipoma with hyperplasia in any of the three adrenal cortical zones27. For these cases, treatment is adrenalectomy (just as in any case of myelolipomas >6cm) independent of its functionality, due to the risk of intratumoral necrosis, hemorrhage from rupture or compression of adjacent structures due to mass effect28. Alternatively, nonfunctional tumors ≤4cm with benign characteristics are recommended to be periodically monitored with radiological and biochemical evaluations. For masses between 4 and 6cm, the surgical intervention should be based on presenting characteristics, growth rate, and the patient’s preference29.Though these tumors are nonfunctional19. In the present case, the patient’s hormone values were within normal parameters - ruling out subclinical states, including Conn's Syndrome , Cushing Syndrome or pheochromocytoma. Nevertheless, the presence of myelolipoma is associated with obesity, DM2 and dyslipidemia warranting pharmacological intervention30. This was further emphasized through a retrospective review of 34 AIs in patients of both sexes over the age of 50, where over half suffered from hypertension, 20.6% had DM2, and 37% had obesity. Of these, 80% were histopathologically confirmed to be adenomas with one being a myelolipoma30.It is estimated that 20% of AIs will have subclinical hormone production and these patients represent an at-risk population with greater risk of metabolic disorders and cardiovascular diseaseAs strengths, we can point out the collaboration between different levels of medical attention and the shared effort of the family and the patient to travel to another state to complete this medical Care. Despite the Venezuelan medical assistance crisis, a relatively quick resolution of the case was achieved. Lastly, we emphasize the compliance with the protocol for proper management of adrenal tumors.The limitations include the inability to perform the hormonal profile and determine whether the tumor was functional or not. Additionally, the choice of imaging could have been better. Specifically, the use of MRI instead of CT is not the first choice for the diagnosis of the myelolipoma; however, this occurred because the initial diagnosis was directed towards a hepatic hemangioma.26. In general, surgical management depends on hormone production, tumor size, high risk features on imaging and patient consent. Yet additional studies and information are needed to better understand myelolipomas, their etiology, and clinical management.Adrenal myelolipomas are rare benign tumors that are generally asymptomatic, whose size ranges from a few millimeters to over a dozen centimetres. Much uncertainty exists surrounding the etiology of these masses with continued debate in the current literature on whether or not they are true neoplasms or manifestations secondary to a reactive processLastly, this case demonstrates how family physicians can manage various aspects of patient care through the facilitation of medical treatments, surgical interventions, and ensuring a proper multidisciplinary approach based on the endocrinology clinical guidelines.Written informed consent was obtained from the patient for the publication of the patient’s details and accompanying anchors. This case provides an extensive and comprehensive background and description of a case of an adrenal myelolipoma. As this is not a common condition, sharing the knowledge on a rare case may facilitate other practitioners to reach the correct diagnosis. It also includes description of relevant differential diagnosis as well as treatment.I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. et al. rightfully have pointed it out. The clinical case was well studied according to accepted guidelines. I consider that the manuscript is well written. Perhaps the only thing that would call my attention is in the Discussion section since it seems to me unnecessarily extensive, but it depends on journal requirements and policies.I think the case report fulfills diagnostic criteria for Adrenal Incidentaloma as Kim and Zeiger One suggestion in the abstract: Use Arabic number to describe the type of Diabetes.We have read this submission. We believe that we have an appropriate level of expertise to confirm that it is of an acceptable scientific standard.