Spontaneous rectus sheath hematoma (SRSH) is an uncommon medical emergency in the elderly. We present a case of SRSH with an atypical clinical presentation and discuss literature regarding diagnosis and proper management. A 75-year-old female patient was transferred to the emergency department due to acute dyspnoea and confusion. Her medical history revealed a viral infection of the upper respiratory tract, and no coughing or use of anticoagulants. The clinical examination showed tenderness of the left lower abdomen, although palpation was misleading due to patient's obesity. Laboratory investigations showed light anaemia. Ultrasonography and computed tomography revealed a large rectus sheath hematoma of the left abdominal wall. Despite further deterioration of the patient, conservative management including bed rest, fluid replacement, blood products transfusion, and proper analgesia was successful. No surgical intervention was needed. Prompt diagnosis and management of SRSH plays significant role in the prognosis, especially in elder patients. Independently of size and severity, conservative management remains the first therapeutic choice. Only by failure of supportive management, progressive and large hematoma or uncontrollable hemodynamic patients, interventional management including surgery or less invasive newer techniques is indicated. Spontaneous rectus sheath hematoma (SRSH) is an uncommon entity . The agiWe present a case of an elder patient with SRSH and an unusual clinical presentation. We highlight the importance of prompt diagnosis and management of this medical emergency and include a review of the literature.A 75-year-old woman was transferred to our emergency department due to sudden onset of dyspnoea and confusion. She reported nasal congestion, sneezing, and malaise during the last three days, indicating a possible viral infection. The patient reported no significant comorbidities except from arterial hypertension under therapy, no coughing, and no anticoagulant use.On examination, her temperature was 37.0°C, blood pressure 110/85 mmHg, pulse rate 100 beats/min, and respiratory rate over 25 breaths/min. The auscultation of the thorax did not reveal any abnormal respiratory or cardiac sounds. The abdominal examination revealed sensitivity in the lower left abdomen with active bowel sounds. Due to patient's obesity (BMI = 39.1), no mass was palpable during examination. Guarding was felt mainly on the same side, whereas the right side was not tender. The remaining physical examination was unremarkable. The patient was examined by a surgeon as well.3), a light anaemia (33.5%), and normal platelet number (300000/mm3). The rest of laboratory studies were as follows: ALT 25 U/L, AST 32 U/L, glucose 120 mg/dL, alkaline phosphatase 225 U/L, total bilirubin 1.3 mg/dL with a direct fraction of 0.5 mg/dL, creatinine 1.3 mg/dL, and albumin 6/mg/dL. Electrolytes, analysis of urine, bleeding and clotting time, chest X-ray, and electrocardiogram were normal. An abdominal ultrasonography study was ordered demonstrating a left sided rectus sheath hematoma measuring 10 cm × 10 cm. Computed tomography confirmed these findings.Laboratory findings revealed a light leukocytosis  and further deterioration. Resuscitation with fluid replacement and adequate analgesia followed. Diuresis was not decreased significantly. The patient became stable and remained in the hospital for monitoring. During her hospital stay, the patient showed a further decrease in her hematocrit levels which improved after transfusion of blood products. Overall, no surgical intervention was needed.SRSH is more common in elderly women than in men, the proportion being 2-3/1 . AlthougRegarding diagnosis, our case presented with an unusual clinical picture that misguided the diagnosis. Common presenting signs and symptoms of SRSH are abdominal pain, palpable abdominal wall mass, abdominal wall ecchymosis, nausea, vomiting, tachycardia, peritoneal irritation, fever, abdominal distention, and abdominal cramping , 8. FothOur case confirms that laboratory findings may demonstrate a decrease in the haemoglobin level, although this may be misleading early in the course. Leukocytosis, thrombocytosis, and prolonged clotting studies in patients on oral anticoagulation may also be present . PatientBoth ultrasound examination (US) and computed tomography (CT) are the indicated diagnostic modalities; in our case, they identified the hematoma without doubt , 13. BotThe difficulties in the correct diagnosis frequently lead to delay in treatment or unneeded surgery. Our patient corresponded to conservative management, as most cases do , 16. RevClinical improvement is usually rapid in patients with type I or II SRSH, although our patient was fully stabilized after a week. Spontaneous complete resolution, especially in large hematomas, may take up to three months . In hemoSurgical intervention includes evacuation of the hematoma and ligation of the offending epigastric vessel. Surgery is indicated, by most authors, only for cases that do not respond to supportive management, progressive large hematoma, or uncontrollable hemodynamic patients , 20. HowRegarding alternative interventional options, new methods have become more popular lately. Arteriography is able to identify the precise location of the persistent bleeding while open surgery can be limited by inability to localize and ligate the bleeding vessel . ArterioRegarding prognosis, prompt diagnosis leads usually to a good outcome and resolution without sequelae is the rule, as it was in our case . In someIn conclusion, SRSH in elderly is a medical emergency, where the atypical clinical presentation of this entity can delay diagnosis and proper management. The early diagnosis of SRSH is the most important factor of low mortality in the elderly, preventing the unnecessary surgical intervention and determining the success of conservative treatment. Conservative management remains the first therapeutic choice for the elderly.


Schwannoma is a relatively common benign spinal cord and/or cauda equina tumor; however, giant cauda equina schwannoma with extensive scalloping of the lumbar vertebral body is a rare pathology, and the treatment strategy, including the use of surgical procedures, is controversial. In this report, we present a rare case of a giant lumbar schwannoma of the cauda equina with extremely large scalloping of the vertebral body, and we discuss the surgical strategy we used to treat this pathology.A 42-year-old Japanese man presented to our department with complaints of a gait disturbance and muscle weakness in the left lower limb. His muscle strength in the proximal part of the left lower limb was grade 2 or 3/5, and he exhibited a mild urinary disturbance on the first visit. X-ray and computed tomography myelography of the lumbar spine showed an extremely large erosive lesion at the L3 vertebral body. Magnetic resonance imaging of the lumbar spine showed a large soft-tissue mass in the spinal canal at L2-L3 and the vertebral body at L3. A one-stage complete tumor resection and instrumented circumferential fusion were performed via a posterior approach, and a good outcome was achieved after the surgery.We performed one-stage posterior surgery in a patient with a giant cauda equina schwannoma with extensive scalloping of the vertebral body, and a good post-operative outcome was achieved. In particular, giant lumbar schwannoma of the cauda equina is very rare compared to sacral lesions–10. Moreer onset. In thisA 42-year-old Japanese man presented to our department with the chief complaints of gait disturbance and muscle weakness in the left lower limb of a few months’ duration. He had no previous medical or surgical history. A physical examination performed at the first visit revealed that his muscle strength was grade 2-3/5 in the proximal portion of the left lower limb. He had full muscle strength in his right lower limb and the distal portion of his left lower limb. In addition, we observed a mild urinary disturbance. X-rays and computed tomography (CT) myelography images of the lumbar spine showed large scalloping of the L3 vertebral body Figure . Magnetiet al. reported the case of a 57-year-old woman with a giant cauda equina schwannoma with scalloping of the posterior surface of the vertebral body from L3 to the sacrum in whom they performed laminectomy and incomplete resection of the tumor via the posterior approach[Spinal schwannoma is a representative benign intradural spinal tumor; however, giant lumbar schwannoma of the cauda equina is a rare pathology, and the treatment strategy, including the use of surgical procedures, for this condition is controversial. Kagaya approach. Furtherapproach. Therefoet al. reported the case of a 65-year-old woman with giant schwannoma of the cauda equina from T12 to L3 in whom they performed complete en bloc resection of the tumor without fusion, despite the fact that the tumor exhibited extensive scalloping of the vertebral bodies of L2 and L3[et al. reported the case of a 49-year-old man with a giant spinal schwannoma at L3 and an extensive scalloping lesion of the L3 vertebral body. They performed complete resection of the tumor and one-stage circumferential fusion using pedicle screws and an expandable titanium cage to treat the vertebral body lesion via a posterior approach. The patient experienced no side effects of motor dysfunction, despite the presence of a sensory disturbance post-operatively[Saito We found that most of our patient’s nerve roots were compressed by the tumor and, as a result, had accumulated in the lateral spinal canal, with the exception of two nerve roots entering the tumor. Although we sacrificed the two nerve roots to perform complete tumor resection, the patient exhibited no severe neurological deficits after the surgery. Moreover, pre-operative CT myelography and MRI of the lumbar spine showed an extensive scalloping lesion at the L3 vertebral body. Therefore, we recognized the need to reconstruct the anterior vertebral body via a posterolateral approach, thus sacrificing an extensive lateral portion of L2 and L3, including L2-L3 facetectomy, or an anterior approach using a different skin incision pre-operatively. However, we confirmed the presence of accumulation of most of the cauda equina in the lateral spinal canal space in addition to a large defect in the ventral dura mater due to tumor invasion. Therefore, we placed a titanium cage in the L3 vertebral body lesion through the dural defect. This allowed us to perform anterior vertebral body reconstruction using a titanium cage via the posterior transdural approach. Hence, we think that the potential use of a posterior transdural approach for anterior vertebral body reconstruction should be taken into account in cases involving giant lumbar schwannomas of the cauda equina because the easy access to the vertebral body makes it possible to achieve circumferential fusion.We performed one-stage complete tumor resection and circumferential fusion using pedicle screws and a titanium cage via the posterior approach in a patient with giant cauda equina schwannoma with extensive scalloping of the vertebral body and achieved a good outcome post-operatively.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


The patella is an uncommon location for cancerous occurrence and development. The majority of tumors of the patella are benign, with a significant incidence of giant cell tumors and chondroblastoma. With the development of modern diagnostic technologies, there appear however many other histological types which raise challenges of diagnosis and treatment. In this article, we review the reported histological types of primary patellar tumors. Specifically, epidemiology, symptomatology, imageology, histopathology, and treatment options for these histological lesions will be discussed, respectively. As there is an increasing focus on the diagnosis and the treatment of these lesions, the availability of the integrated information about primary patellar tumors becomes more significant. A low incidence of tumors is found in the patella with the vast majority being giant cell tumor and chondroblastoma . DiagnosGiant cell tumor (GCT) is the most common type of the diagnosed patellar tumors  .Clinically, the patients with the patellar GCTs usually complained about knee pains and/or swelling -5. But tThe laboratory findings on some severe patients showed an increase of serum alkaline phosphatase (AP) ,7 and erRadiographs Figure  revealedA needle biopsy or incisional biopsy of the patella is recommended for the diagnosis. The histopathological features of the patellar GCT, which mainly include numerous giant cells, short spindle-shaped cells, bone tissue calcification, and a few mitotic figures , should Surgery is the primary mode of treatment. GCT is a locally bony lesion, a tumor considered as having low recurrence rates using standard meticulous surgical technique according to Meyerding’s outline . For smaChondroblastoma (CB) can also be found in the patella but at a very low occurrence rate (2%) . It is tCB appears as lesions on the patella that caused pain either with or without swelling -16. The Most of routine laboratory tests were within normal ranges. The increased serum calcium and AP were only found in the patients with patella fracture .Plain radiographs Figure  demonstrHistological examination revealed sheets of proliferating chondroblast with chondroid matrix and some multinucleated giant cells in places admixed with focal areas of coarse calcification -17. WithCB treatment included curettage, curettage with burring, and filling of the lesion cavity with bone graft and/or bone cement . For theAlthough the patella is a rare site for osteoid osteoma (OO) with only an incidence of 5% in all patellar tumors , a few cLong-term pain was the main complaint of the patellar OO patients, which was presented throughout the daytime and exacerbated at nighttime and after physical exercises; which was well localized at the patella and could be relieved by analgesic drugs -24. SwelLaboratory studies of reported cases were normal -24.X-ray, CT, MRI, and bone scintigraphy were meaningful to the determination of the patellar OO, for they showed a well-defined lesion with nidus and mineralization in the affected location of the patella -24 Figu.Figure 3Several researchers suggested that the natural history of osteoid osteoma be a process of spontaneous healing. But studies indicated that it would take a long time to fully resolve symptoms if the osteoid osteoma was not excised. Non-steroidal anti-inflammatory drugs can reduce the time of recovery period. OO was traditionally treated with excision of the nidus, so did patellar OO. Curettage nidus or resection was performed to resolve symptoms (Enneking stage 1) ,23-25.Less than 1% aneurysmal bone cyst (ABC) of all the known cases occurs in the patella . PatellaPatellar ABC patients frequently presented complaints of pain in the anterior aspect of the affected knee and/or swelling of the knee joint -30. The Hemogram, c-reaction protein (CRP), and ESR were completely within normal ranges .Radiographs of the knee showed a ‘geographic osteolysis’ all over the affected patella, that is, cortical thinning, smooth borders, and intact articular surface -28. MildHistopathologic examination of the biopsy material revealed empty bloody cavities or the presence of red blood cells -29. The The best choice of ABC treatment is surgical saucerization and curettage with removal of the cyst lining followed by bone grafting. Bone grafting or bone cement following curettage with burring by a mid-line surgical approach was employed to remove the lesion (Enneking stage 2) -29. PateChondroma (CH), including both enchondroma (EC) and juxtacortical chondroma (JC), is a rare lesion with approximately 5% of incidence in patellar tumors .Clinically, CH patients would state their ache, swelling, and patellar mass ,32. One Routine laboratory studies and ESR were within normal ranges .Radiographs of the knee revealed an oval lytic defect within the patella in EC patients . The mixHistologic examination revealed diseased tissue of hyaline cartilage containing chondrocytes with small, condensed nuclei, which conformed to characteristic of chondroma .Lammot  reportedOsteochondroma (OC) in the patella is rare with an approximate 2% incidence of patellar tumors .Clinically, patellar OC might cause the symptoms of knee pain and mass ,35. UponRoutine laboratory studies were within normal ranges .Upon radiographs, an irregular calcification neoformation that stretched from the edge of the affected patella was detected -35. SomeAnatomic pathologically, the lesion was a pale polypoid tissue with the cartilage cap. Microscopically, the outer layer was composed of connective tissues and the deep layer of cartilage cells. Between these two layers, there were normal spongy bone tissues . The resAlthough the surgery to excise the tumor is not essential to all OC cases, it is still a rational method for patients who suffered from some OC symptoms and malignant transformation. The OC surgery involved curettage, burring, bone grafting, and bone cementing. The simple resection was often found to inhibit the tumor growth and to resolve the symptoms in Enneking stage 2 cases ,35. DuriOsteoblastoma (OB) in the patella is very rare  , and onlThe common symptoms of the patellar OB patients are pain and swelling ,39. LocaRadiograph of the patella revealed an osteolytic lesion surrounded by a sclerotic rim without evidence of extra-articular involvement ,37,38. OThe pathologic examination demonstrated that the lesion comprised irregular trabeculae of immature woven bone, lined by plump osteoblasts within a loosely textured fibrovascular stroma containing numerous giant cells ,38. In ten bloc) are the options for the OB treatment. Intralesional curettage can be effective but is associated with a high rate of recurrence. The treatment method for local recurrences should be wide en bloc resection [Generally, both conservative surgery and complete resection  is very rare with only an incidence of 2% in patellar tumors .Clinically, complaints of the patellar OH patients could include aching, swelling, and limitation of movements of the knee .Routine laboratory findings, such as blood count, Mantoux test, ESR, blood calcium, blood phosphorus, and AP, proved to be normal .Upon radiographs of the knee joint, there is a multilocular cystic lesion all over a part of patella with possible surroundings of sharp margin, thinned cortex, and sclerotic rim ,40. A sePathological findings showed that the loculated lesion containing fluid and clotted blood was traversed by thin bony septa. Existence of a fracture could be judged by visual inspection. Microscopically, the cyst consisted of large and small spaces filled with endothelium and blood. Reactive bone formation in the bony trabeculae running across the lesion could be indicated, as well .Patellectomy was performed in the two patellar OH cases (Enneking stage 1 to 2), and the treated patients were finally capable of full movements of the knee joints .Simple bone cyst (SBC) comprises about 3% of all biopsied primary bone tumors and accounts for 4% of patellar tumors .The patellar SBC patients usually complained about knee pain and swelling -43. The Radiographs showed a radiolucent lesion with well-defined margins in the affected location of the patella -44. TherLaboratory investigations did not reveal any abnormal findings .Macroscopically, the lesion included a cyst lined with a thin membrane and a smooth inner surface, full of clear or yellowish fluid ,42. MicrThe patellar SBC lesions were eliminated by autogenous grafting following curettage (Enneking stage 1)  or patelOther benign patellar tumors that have been reported include lipoma, osteitis fibrosa cystica, ganglion, osteoma, and non-ossifying fibroma. Because of the limited case reports, more details cannot be obtained. Known information thereof is shown in Table Osteosarcoma (OS) is the most common type of primary malignant osseous tumor. It mainly affects the long bones of the extremities but also affects other bones like patella. The patellar OS has an incidence of 6% in patellar tumors .The patellar OS patients complained about the increasing anterior knee pain and swelling in the affected knee -49. MostPlain radiographs Figure  showed lOnly in one case, ESR and white blood cell (WBC) count were increased .Photomicrograph of histology revealed the diffuse proliferation of malignant cells that led to abundant osteoid formation. The cells were spindle shaped, atypical with large, hyperchromatic nuclei and prominent nucleoli -50. IrreSurgery is still the main therapeutic approach. For patellar OS, surgical methods, including patellectomy  ,50, wideOriginating from cartilage tissues, chondrosarcoma (CS) is the second most common primary bone tumor. It may occur at any endochondral ossification of the whole body bones, especially at the pelvis, femurs, humeruses, and scapulas . CS occuClinically, increased mass, pain, swelling, and lameness of the affected knee might be the main complaint of the patellar CS patients ,54. The Radiographs of the affected knee joint showed a lytic lesion shadow on the patella -55. The Hematological tests of one case , includiMacroscopically, part of the patella had been replaced by the lesion, and certain necrosis of its surrounding bones and soft tissues could be found ,54. The Adjuvant chemotherapeutic drugs or radiotherapy is useless to CS due to its poor blood supply and lymph circulation. The wide surgical resection is still regarded as a mainstay method for treating CS . The surGenerally, lymphomas arise in lymph nodes, while their formation in extranodal sites is less common. The patellar POL accounts for approximately 4% of all the patellar tumors.One patient with the patellar POL presented pain, due to a pathologic fracture . A physiThe patella exhibited moth-eaten appearance and multiple lytic lesions on knee through X-ray inspection ,56,57. AHematological and biochemical tests, bone marrow biopsy, and synovial fluid were unremarkable ,57.Excised lesion revealed the diffuse infiltration of tumor and/or surrounding tissues ,57. In tThe common treatments for primary bone lymphoma included the following: a combination of chemotherapy and radiotherapy, chemotherapy alone, and radiotherapy alone . The POLMalignant fibrous histiocytoma (MFH), which arises in both the soft tissues and the bones, is the most frequent sarcoma of malignant soft tissue that is characterized by aggressive biological behavior. Patellar MFH, which originates in some unusual location, accounts for 1% of all the patellar tumors [The patient’s complaints include pain, swelling, decreased range of motion, anorexia, and weight loss ,60. PhysThe radiographs showed multiple radiolucent lesion and permeating destruction in the patella ,60. SeptThe only abnormal laboratory finding was the increased level of ESR ,60. The Generally, specimen showed grayish, whorled tissue with thin, calcified septa and thinned cortex . There wThe treatments of osseous MFH included wide resection, additional radiotherapy, adjuvant chemotherapy, systemic treatment approach, or the combination . The firOther sporadic malignant cases that were ever reported, such as leiomyosarcoma Figure , angiosaThe patella is defined as a sesamoid originating in the quadriceps tendon. It develops from a cartilaginous precursor in the third month of gestation and ossifies about 3 years of age . Its ossThe most common symptom of primary patellar tumors is knee pain that usually lasts a long period of time from pain onset to determination upon diagnosis . For benIn patients with anterior knee pain, plain radiograph is the most common way to find patellar anomalous changes and can accelerate the diagnosis of a patellar tumor . In mostThe past reviews on the topic of patellar tumors are short of supports by laboratory tests, due to the insufficient reports and unusual changes. But as relative cases are increasing, a certain amount of data has been made available. Although the routine laboratory tests are usually within the normal ranges, a pathological fracture may lead to the increased levels of serum calcium and AP. A rise in levels of WBC and ESR can be detected in some malignant cases after laboratory tests. This may pertain to the secondary inflammatory reaction. Synovial fluid examination has been used, which is of certain significance to some cases. Therefore, laboratory tests must not be overlooked.Radiographic features of primary patellar tumors are atypical of a specific histotype. Histological examination is preferred, but in view of the risk of contamination, a needle biopsy is a better option. An incisional biopsy is recommended if the diagnosis and the radiographic features are paradoxical. Two key points should be noticed: 1) In needle biopsy, the joint must not be entered; (2) in incisional biopsy, the patellar and quadriceps tendon, the joint cavity, and the synovial membrane must not be contaminated  In needl-49.Surgery is the most effective treatment for most of the symptomatic patients who have a patellar tumor. Surgeons prefer to outline a course of treatment according to Enneking stages . Some adAlthough anterior knee pain seems simple, the primary tumors originating in the patella as a possible lesion cannot be neglected. More types of unreported primary patellar tumors will be confirmed. Due to the insufficient availability of the patellar characteristics, the integrated diagnosis of primary patellar tumors should be seriously considered. The extended application of MRI and CT is necessary. And noninvasive biopsy is worth studying in order to avoid contamination. While experts are inclined to outline a course of treatment for primary patellar tumors according to Enneking stages, new adjuvant therapy and improved methods of functional reconstruction may do more good to the patients.


Extrarenal angiomyolipoma (AML) is an extremely uncommon lesion, accounting for less than 9% of all angiomyolipomas. We present a previously unreported case of a rarely located gigantic extrarenal angiomyolipoma at the posterolateral chest wall of a 35-year-old woman. Clinically, the lesion had all the characteristics of a benign tumor, being soft in palpation, painless, and growing in size in a slow rate. Histologically, the lesion consisted of convoluted thick-walled blood vessels without an elastic layer, interlacing fascicles of smooth muscle, and mature adipose tissue, features consistent with an angiomyolipoma. The mass was surgically removed, without any postoperative complications, and the patient has an uneventful postoperative course. Signs of local recurrence have not been observed. The purpose of this brief report is to point out the necessity of including angiomyolipoma in the differential diagnosis of adipose layer lesions. Angiomyolipoma (AML) is a clonal mesenchymal tumour with malignant potential, usually located at the kidney and most commonly found in middle-aged women. Renal angiomyolipoma is strongly associated with tuberous sclerosis complex (TSC), a group of autosomal dominant genetic disorders, and lymphangioleiomyomatosis. It is an invasive and recurring lesion, is prone to spontaneous bleeding, and consists of variable portions of interlacing fascicles of epithelioid smooth muscle, convoluted thick-walled blood vessels, and mature fat. Extrarenal manifestation of angiomyolipoma is uncommon and has been mostly reported in the liver. It is very rare and presents with male predominance, no association with TSC or lymphangioleiomyomatosis, and no signs of local recurrence. Extrarenal angiomyolipoma is also solitary and well circumscribed and is characterized by the lack of epithelioid tissue. The purpose of this report is to describe a case of angiomyolipoma in the posterior chest wall with a diameter of 22 cm, a location and size that have never been reported previously in the literature.The patient is a 35-year-old, otherwise healthy, white female, who presented to our clinic with a large tender mass in the left posterolateral chest wall. The physical examination revealed a solitary and painful nodule adherent to deep layers and covered with normal skin. The patient's medical history included a resection of a cystic formation of the right lung in childhood. She did not receive any systemic treatment and had no family history of tuberous sclerosis complex or other inherited conditions. MRI was performed and the existence of a 22 cm lobular mass was confirmed. It was located just over the lower limit of the left scapula and extended posterolaterally roughly down to the rim of the left 10th-11th rib . The lesOriginally, angiomyolipoma was known as a hamartomatous lesion. It is now considered to be a mesenchymal tumour with malignant potential , usuallyMRI imaging is the most important diagnostic tool in distinguishing angiomyolipomas, since the proportion of fat in the lesion defines the ability for imaging. Macroscopic fat has high signal intensity on T1 and T2 sequence and leads to high sensitivity in the detection of extrarenal angiomyolipomas . AccordiThe angiomyolipoma diagnosed in our patient in the posterolateral chest wall is generally observed in middle-aged men, is noninvasive, and shows no signs of recurrence or metastasis. The size of a regular angiomyolipoma defines the symptoms, since lesions with diameter less than four cm are usually asymptomatic, whereas those with diameter bigger than four cm may cause pain or lead to bleeding. The appropriate treatment for an extrarenal angiomyolipoma is surgical resection, whereas the absence of malignancy makes any adjuvant therapy unnecessary. The posterolateral chest wall has great extent and permits the presence of a sizeable angiomyolipoma (diameter of 22 cm). Apparently, the location of the lesion plays a significant role in its size. A year after the surgical resection, our patient is healthy and has not presented any signs of local recurrence.We presented a previously unreported case of a rarely located gigantic extrarenal angiomyolipoma of the posterolateral chest wall and pointed out that angiomyolipoma should be included in the differential diagnosis of adipose layer lesions.


Dens evaginatus (DE) is a rare developmental anomaly characterized by presence of an extra cusp arising from occlusal or lingual surfaces. Isolated occurrence or concomitant presence of DE with other dental anomalies has been reported. DE commonly affects permanent teeth and is rarely seen in primary dentition. Treatment may be conservative or radical. This article presents two unusual cases of concomitant occurrence of a supernumerary tooth and DE affecting maxillary deciduous lateral incisor and conservative management of DE occurring concurrently with a possible Oehler’s type I invagination in maxillary permanent lateral incisor. Dens evaginatus and dens invaginatus are developmental variations of the shape of a tooth.7 Other terms used to describe this anomaly include accessory cusp, leong’s premolar, evaginatus odontomas, occlusal pearl, and talon cusp .38DE occurs normally alone, but can present itself in association with other dental anomalies such as supernumerary teeth, peg shaped incisors, bifid cingulum, and dens invaginatus.8 The evaginated tubercle usually is composed of normal enamel and dentin with varying extensions of pulp tissue, and is more prone to wear or fracture leading to early pulp pathosis. Treatment approaches to DE depends on the shape and size of the evagination and can be either conservative or radical.9While talon cusp originated as a descriptive term for DE due to its resemblance to an eagle’s talon, current concepts prefer the terminology DE, to describe this developmental aberration, as most authors agree that both DE and talon cusp share the same etiology occurring during the morpho-differentiation stage of tooth development.This article presents two unusual cases of concomitant occurrence of a supernumerary tooth and DE affecting maxillary deciduous lateral incisor and conservative management of DE occurring concurrently with a possible Oehler’s type I invagination in maxillary permanent lateral incisor.A 9-year-old female came to the pediatric dental clinic for dental treatment. The medical and family history was noncontributory. Clinical examination disclosed carious lesions in primary and permanent molars, and a prominent cusp-like structure on the palatal surface of the maxillary left lateral incisor. It was pyramidal in shape and extended from CEJ half-way to the incisal edge causing minimal occlusal interference .Clinically, the tooth was asymptomatic and responded normally to pulp vitality tests. The grooves at the junction of the cusp and palatal surface were stained and contained dental plaque. Maxillary right lateral incisor displayed a deep lingual pit suggesting type I invagination. Further, prominent bifid cingulae with deep stained fissures were apparent on the palatal aspects of both maxillary right and left central incisors. White spot lesions were visible in the invagination and around the tubercle .A periapical radiograph showed a ‘V’ shaped radiopaque structure superimposed on the affected crowns, with the ‘V’ pointing towards the incisal edge. In addition, periapi-cal radiograph revealed an invagination lined by enamel, adjacent to the tubercle indicating probable Oehler’s type I invagination in 22. Radiographically no evidence of associated pathosis was found .The treatment plan consisted of oral hygiene instructions, restoration of carious teeth, and prophylactic restorative procedures in the maxillary central incisors and lateral incisors. The prophylactic treatment consisted of selective grinding of extra cusp with flare-shaped diamond bur under water coolant using high-speed hand piece at 3-month intervals.Each grinding session was followed by the application of a desensitizing/remineralizing agent with 0.2% fluoride , to the surfaces of reduced cusp, after polishing, to reduce dentin sensitivity. The treatment lasted 12 months, during which preventive measures to caries were followed regularly.Invasive sealing of the cusp-tooth intersection in incisors was planned. After prophylaxis of affected teeth, ameloplasty was performed on the grooves/fissures near the bifid cingulum using carbide bur in a high-speed hand piece and were then sealed with an acid-etch flowable composite resin to avoid penetration of irritants and microorganisms into the invagination . After 1A 6-year-old female presented for a routine dental examination. Her medical history was noncontributory and extraoral examination did not reveal any abnormalities. On intraoral examination, the left maxillary deciduous lateral incisor (62) was slightly mobile and revealed an extra cusp which extended from CEJ halfway to incisal edge, at right angles to the mesiodistal surface of the tooth crown on the lingual surface resembling an eagle’s talon, suggesting dens evaginatus. The cusp measured 2 to 2.5 mm in length , 1 to 1.5 mm in width  and about 2 to 2.5 mm in thickness  approximately .She was born out of consanguineous marriage and family history was free of similar dental abnormalities. A periapical radiograph of 62 revealed a radiopaque structure superimposed on the crown. Further, the unerupted left maxillary permanent lateral incisor presented an invagina-tion like appearance lined by enamel with possible aspect of Oehler’s type I dens invaginatus. Additionally, an unerupted supernumerary lateral incisor  was noticed mesial to 62. No evidence of any periapical pathosis was evident radiographically .There were neither esthetic concerns nor clinical signs of any problems associated with deciduous maxillary lateral incisor. So no immediate dental intervention was advised and the patient is under regular follow-up.10 In the two cases reported here, the etiology of dental abnormalities could not be clearly associated with any genetic or environmental factors, as the family history was negative in both the cases.The exact etiology of these variations remains unclear. However, the probable role of genetics and environmental factors, such as trauma or other localized insults affecting the tooth germ have been suggested.5611 One plausible explanation for the association of extra cusp with a supernumerary tooth may be the local hyperactivity of dental lamina resulting in the occurrence of supernumerary and accessory cusps, during the bud-stage and morpho-differentiation stages, respectively.5The case-2 presented here is unique because of the association of a supernumerary tooth and a probable dens invaginatus in 22, with DE in maxillary deciduous left lateral incisor. Only few cases of this anomaly in primary teeth have been reported in the literature.12Small talon cusps are asymptomatic and require no intervention. Large talon cusps may cause clinical problems including occlusal interference, irritation of the tongue during speech and mastication, carious lesions in the developmental grooves that delineate the cusp, pulp necrosis, periapical pathosis, attrition of the opposing tooth, and periodontal problems due to excessive occlusal forces.13On the contrary, the invagination in DI frequently communicates with the oral cavity, allowing the penetration of irritants and microorganisms into the pulp and may eventually lead to pulp necrosis. The treatment options for dens in-vaginatus include preventive sealing of the invagination, root canal treatment, endodontic apical surgery and extraction.414 Treatment strategies are different for the deciduous and the permanent dentition. Oehlers and Colleagues (1967)15 advocated intermittent grinding of the tubercle to encourage reparative dentin formation. Myers 16 reported a case of talon cusp where in two-thirds of the cusp reduction was done followed by topical fluoride application at 4 month intervals over 5 years. Richardson (1985) 17 had suggested a preventive approach, which included pumice prophylaxis and acid-etching with phosphoric acid, followed by placement of a composite material.The treatment of DE merits clinical consideration and objectives could include pulp vitality preservation, caries prevention, elimination of tongue irritation, and meeting esthetic and occlusal requirements.9 Further, it increases tooth resistance to acid dissolution, promotes remineralization, and also inhibits the cariogenic microbial process.Prophylactic treatment can be considered as the preferred mode of approach. Application of desensitizing/rematerial-izing agent containing 0.2% sodium fluoride following the gradual and periodic reduction of bulk of extra cusp, reduces sensitivity, stimulates reparative dentin formation, and allows the tooth to remain vital, especially in permanent teeth with open apices.9In case-1 reported here, we have reduced 1 to 1.5 mm of extra cusp gradually on several consecutive visits at 3 month intervals, followed by application of a desensitizer (GC tooth mousse plus) to protect the pulp and allow deposition of reparative dentin. Here, the grinding procedure was carried out over 12 months. However, in case-2, no intervention was advised as the need for treatment in a primary talon-cusped tooth which will eventually exfoliate, other than for esthetic reasons, is low. Pit and fissure sealants are recommended as preventive measures, whilst composite resin and amalgam restorations have also been advocated as DE is prone to caries. Root canal therapy and extraction have been suggested in selected cases.Dens evaginatus deserves clinical importance as it provides chances of early pulp pathosis. Radiologic examination plays a vital role in diagnosis in conjunction with clinical examination. Early and the correct diagnosis of dental abnormalities by clinicians are essential to preserve the vitality of the pulp.


Hemorrhage caused by spinal cord hemangioblastoma is rare, usually presenting as a subarachnoid hemorrhage. Intramedullary hemorrhage is an extremely rare manifestation of spinal cord hemangioblastoma.Forty-year-old Japanese male patient presented with acute paraplegia. Magnetic resonance (MR) imaging of the spinal cord revealed intramedullary hemorrhage. An intramedullary mass lesion was detected at the 8th thoracic vertebral level (T8) in a gadolinium enhanced-MR image. Spinal angiography revealed an intramedullary tumor stain at the level of T8. Therefore we diagnosed the problem as intramedullary hemorrhage caused by the hemangioblastoma. One month after the onset, extirpation of the intramedullary hemangioblastoma was performed. The tumor was completely removed. Pathological findings revealed a typical hemangioblastoma. At his final follow-up visit, the patient showed no apparent neurological recovery.Hemangioblastoma can be a cause of intramedullary hemorrhage should be considered in such cases. Hemorrhage caused by spinal cord hemangioblastoma is rare, usually presenting as a subarachnoid hemorrhage . IntrameA 40-year-old Japanese male patient presented with acute paraplegia after extending his trunk during bathing. Before onset, he was doing well without any paralysis. He had untreated diabetes. The patient was admitted to a local hospital and then transferred to our department for surgery.On admission, the patient was awake and alert, and did not complain of either headache or neck pain. There were no signs or family history of von Hippel–Lindau disease. Initial physical examination revealed that muscle strength and sensation of his upper extremities were normal. There was complete loss of sensation to pinprick below the 7th thoracic vertebral level (T7) including the perineal region; however, touch sense was preserved except for the feet bilaterally. There was complete motor loss in his right lower extremity and severe weakness in his left lower extremity, which had a manual muscle testing grade of from 1 to 2. Patellar and Achilles tendon reflexes were absent bilaterally. There was no apparent Babinski reflex.Magnetic resonance (MR) imaging of the spinal cord revealed a syrinx extending from the bulbs to the lumbar spinal cord, and intramedullary hemorrhage (Figure Therefore we diagnosed the problem as intramedullary hemorrhage caused by the hemangioblastoma. We planned to perform tumor extirpation as elected surgery in the subacute stage of hemorrhage, and we decided to wait for at least 3 to 4 weeks after the onset.After admission, the patient showed slight motor and sensory recovery. The patient was treated conservatively with physiotherapy under hospitalization. One month after the onset, he could extend his right knee and ankle, and he recovered sensation to pinprick on his trunk.One month after the onset, the patient underwent surgery. Under general anesthesia, a T7–T9 laminectomy followed by extirpation of the intramedullary hemangioblastoma was performed. The tumor was completely removed. Pathological findings revealed a typical hemangioblastoma. At his final follow-up visit, the patient showed no apparent neurological recovery.Here we report a case of acute intramedullary hemorrhage caused by hemangioblastoma of the thoracic spinal cord. Despite complete removal of the tumor, the patient obtained only slight motor and sensory recovery.Previous reports describing intramedullary hemorrhage caused by spinal cord hemangioblastoma showed acute paraplegia after the onset, and poor recovery even after complete removal of the tumor. Preexisting intramedullary hemangioblastoma might cause subclinical spinal cord damage resulting in additive catastrophic damage caused by intramedullary hemorrhage.et al. reported the clinical outcome of conservative and surgical treatment for intramedullary cavernous hemangioma with hemorrhage, and indicated that the intramedullary tumor removal operation presents a risk of additional damage to the acutely paralyzed spinal cord [Whether a better outcome can be obtained by emergent tumor removal remains unclear. Steiger nal cord . In thisOnce bleeding occurs, it leads to a catastrophic sequela of paralysis that not even tumor extirpation can rescue. Tumor removal surgery should be considered for asymptomatic hemangioblastoma or hemangioblastoma with only slight symptoms. At least, the patient should be informed of the possibility that hemangioblastoma can cause intramedullary hemorrhage.In conclusion, that hemangioblastoma can be a cause of intramedullary hemorrhage should be considered in such cases.Written informed consent was obtained from the patient for publication of this Case Report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


The current study reports a case of an extremely rare tumor that presented in an uncommon location, which was successfully treated via radical resection and reconstruction. A 37-year-old female, with no notable medical history, with the exception of a cesarean delivery, was admitted to The First Affiliated Hospital of Wenzhou Medical University  due to pain and a lump of the anterior chest wall. The mass was identified on the manubrium sterni and was not tender on palpation. A chest computed tomography (CT) scan reconstruction identified the abnormal mass on the manubrium sterni  and positron emission tomography-CT interpretation strongly indicated a type of well-differentiated malignant tumor, such as a giant cell tumor. An aspiration needle biopsy was not conducted, however, the patient underwent tumor radical resection and sternal reconstruction using steel wire and titanium mesh. Histopathological examination of the surgical specimen determined the diagnosis of chondrosarcoma. A postoperative chest X-ray revealed that the sternal defect had repaired well, therefore, this procedure may be highly beneficial in future for repairing defects in the sternum. Primary sternal tumors are a rare type of bone and soft tissue tumor, with chondrosarcoma being the most common. Generally, the precise evaluation of such tumors requires a thorough radiological examination since the clinically appreciable mass usually involves the sternum . However18F-fluorodeoxyglucose (FDG)-avid, however, there was no evidence that the distant metastases were FDG-avid. On the basis of the anatomic and metabolic findings, the PET-CT interpretation strongly indicated a type of well-differentiated malignant tumor, such as a giant cell tumor.In July 2013, a 37-year-old previously healthy female, who had previously undergone one cesarean delivery, presented at The First Affiliated Hospital of Wenzhou Medical University  with a lump and pain of the anterior chest wall. On physical examination, a palpable mass was identified on the manubrium sterni, which was not tender on palpation. Laboratory tests revealed no abnormalities; however, a chest computed tomography (CT) scan reconstruction demonstrated an expansive localized, hypodense, round mass on the manubrium sterni  was performed. Radical resection of the right and left sternoclavicular joints, the second rib and the entire upper section of the sternum body was performed, and a drainage tube was placed in the mediastinum. Following surgery, the patient received antibiotics and expectorant treatment, along with parenteral nutrition. The pain subsequently diminished and became tolerable. On postoperative day five, the removal of the mediastinal drainage tube was required; the patient recovered well following surgery and was discharged on postoperative day eight. The overall duration of the hospital stay was 16 days. Follow-up CT scans were performed every three months to monitor tumor recurrence. After 12 months of follow-up no tumor reccurence was identified.Histological analysis of a surgical specimen revealed well-differentiated malignant cartilaginous cells, consistent with a grade I (considered to be low grade) chondrosarcoma . The tumPrimary sternal tumors are uncommon and account for only ~1% of primary bone neoplasms, worldwide . ChondroIn cases of chondrosarcoma, the CT scan usually indicates a hypodense, rounded mass that is located in the sternum; the bone is expansive and the cortex is thin and damaged. PET-CT may occasionally distinguish benign from malignant bone neoplasms, however, certain well-differentiated tumors may be only marginally FDG-avid on PET-CT imaging, therefore, it can be challenging to differentiate the tumor types. Although the histopathological findings of the excised mass are the only true determinant of the nature of the cancer, PET-CT remains a valuable and meaningful measure for assessing the nature and extent of the tumor.Histopathologically, chondrosarcoma exhibit a distinctive architecture, with high numbers of hyaline cartilage cells and the presence of a chondromyxoid cartilage matrix. The atypical cells are comprised of oval or round nuclei and a deeply eosinophilic cytoplasm .Bone autografts and allografts from the ribs or iliac bone have been reported as an alternative to synthetic materials for use when rebuilding chest wall defects . HoweverIn conclusion, the current study reports a rare case of primary chondrosarcoma of the sternum, which was successfully treated by radical resection and reconstruction using steel wire and titanium mesh. Therefore, in future cases, the use of titanium mesh and steel wire may be considered as highly beneficial materials for use during the reconstruction of defects of the sternum.


Hemangiomas are the most frequent benign hepatic tumours and are usually found in patients aged between 40 and 60 years, more frequently in women. In 30–35% of patients, the lesions are multiple. If the lesions are larger than 4–10 cm, they are coined as “giant” hemangioma. Here, we present a case of giant hemangioma treated with enucleation of the lesion and the advantages of the procedure. Hemangiomas are the most common benign tumours affecting the liver, with the incidence of 0.4 to 20% . The exaA 40-year-old female with no medical comorbidities presented with complaints of abdominal pain confined to right hypochondria, decreased appetite with early satiety associated with bloating of abdomen after meals. Her family history was unremarkable and she denied any substance abuse. She had similar complaints in the past and was treated symptomatically. She had past history of appendicectomy eight years before the current admission.Routine investigations which involved complete blood count, urine analysis, and liver function test were within normal limits . With thPatient underwent enucleation of the lesion under general anaesthesia. Bilateral subcostal incisions were made, wound was opened in layers, and mass was excised in toto measuring 20 × 30 cms approximately . After aThe management of giant hepatic hemangioma is controversial. Several treatment strategies are available: nonsurgical, surgical treatments (open/laparoscopic) resection or enucleation . SeveralThe effectiveness of hepatic artery ligation as a treatment for hemangioma has been described anecdotally; however, its benefit is likely transient. Hepatic artery ligation or embolization does play a pivotal role in controlling haemorrhage temporarily from a hemangioma to permit the transfer of a patient to higher centres. Radiotherapy has been used successfully to treat the symptoms and induce involution of hemangioma in few institutions, but the rarity of this occurrence makes the result difficult to interpret. On the whole, the data justifying the use of radiotherapy in hemangioma is scant. However, if surgical therapy is not possible, palliative radiotherapy can be given .Of the various treatment options for giant hemangioma, surgical treatment, including resection or enucleation, provides consistently effective outcome with satisfactory results , 14.p = 0.004), less risk of bile leakage (enucleation: 0% versus resection: 8–17%), maximum preservation of functional liver parenchyma, and less overall complications [The conundrum between enucleation or resection of a giant hepatic hemangioma is dependent on various factors like the certainty of diagnosis, localisation, size and number of lesions, and growth pattern of the hemangioma , 13, 14.ications –18.With enucleation, the risk of injury of bile ducts and vessels is minimal, since enucleation is performed just outside of the fibrous capsule surrounding the hemangioma, which is composed of compressed liver parenchyma. Belli et al. showed positive results after enucleation of giant hepatic hemangioma in four patients, with the preservation of sufficient normal liver parenchyma , 18. A cWe report a giant hemangioma successfully treated with enucleation, thus reiterating the advantages of this procedure over resection and other newer modalities.


SDHB, confirming a diagnosis of Hereditary Paraganglioma-Pheochromocytoma syndrome.A young healthy postpartum mother presented with intermittent high fevers and tachycardia. Appropriate testing was done to rule out infectious causes including pan cultures but no identifiable infectious source was found. A CT of the abdomen showed a retroperitoneal mass with two small pulmonary nodules and a bony metastatic lesion. She was found to have stage 4 extra-adrenal paraganglioma with metastases to the lungs and spine. She underwent resection of the mass and is currently undergoing palliative radiation to the spine for pain control. Subsequent genetic testing identified a likely pathogenic variant in  We report a rare and unique case of a young primigravida female patient who developed postpartum fever. After appropriate diagnostic testing, the patient was found to have a retroperitoneal paraganglioma. We report this case to emphasize that pheochromocytoma or paraganglioma should be considered in the differential diagnosis of postpartum fever especially with a history of preeclampsia during pregnancy.A 21-year-old primigravida at 37-week gestation presented to the outpatient clinic for a routine obstetrical examination. Her pregnancy course had been uneventful. Her blood pressure at this visit was 184/108. She was asymptomatic except for occasional mild headaches. A urine dipstick showed 3+ proteinuria. Patient was diagnosed with preeclampsia and was admitted to the hospital for emergency induction of labor. She was started on IV magnesium for preeclampsia and labor was induced with oxytocin. She underwent an emergency cesarean section due to fetal decelerations during induction. Subsequently, she delivered a healthy male infant by low transverse C section. Four days after partum, she started spiking high fevers up to 39.5 degrees Celsius and developed tachycardia with heart rate of 140 to 150/min. She was otherwise completely asymptomatic and clinical exam was negative for any localizing signs. Her cesarean section incision looked clean; she had no breast tenderness. Infectious work-up including blood and urine cultures was negative. Her chest X-ray was normal; EKG showed sinus tachycardia. She was empirically started on broad spectrum antibiotics. After 48 hours of IV antibiotics, patient continued to spike high fevers and was still tachycardic. The differential diagnosis of endometritis and septic thrombophlebitis was high on the list even though her abdominal exam was benign. Computerized tomography (CT) scan of the abdomen and pelvis with contrast  was orde MAX, NF1, RET, SDHA, SDHAF2, SDHB, SDHC, SDHD, TMEM127, and VHL identified a likely pathogenic variant in SDHB, c.418G>T . This result confirmed a diagnosis of Hereditary Paraganglioma-Pheochromocytoma syndrome, an autosomal dominant condition with reduced penetrance.The patient underwent genetic counseling and pursued genetic testing for multiple hereditary paraganglioma syndromes. She had no known family history of paraganglioma, pheochromocytoma, kidney cancer, or thyroid cancer. Next generation sequencing ofParagangliomas, also sometimes called extra-adrenal pheochromocytomas, are rare neuroendocrine tumors arising from sympathetic and parasympathetic paraganglia outside the adrenal medulla. They can occur at almost any site in the body including head, neck, thorax, and abdominal cavity. Most of the thoracic and abdominal paragangliomas arise from sympathetic ganglia and are secretory while head and neck paragangliomas are generally nonsecretory as they arise from parasympathetic ganglia , 2. They SDHB-associated paragangliomas are malignant in up to 37% of cases [Most paragangliomas occur sporadically; however, up to 30% are due to a hereditary predisposition syndrome . All indof cases . IndividParagangliomas are seen most commonly located in the inferior para-aortic region; however, they also occur in other regions like the urinary bladder, mediastinum, head, and neck . ParaganThe occurrence of paragangliomas in pregnancy is extremely rare and has been reported to be about 5 per million pregnancies in some studies to 18.5 per million live births in others , 9, 10. Timely diagnosis and treatment are of paramount importance as the maternal and fetal mortality can be as high as 50% if this condition is undiagnosed and untreated . A hyperAfter biochemical confirmation, imaging is used to identify the location of the tumour. In pregnant females, the imaging modality of choice is Magnetic Resonance Imaging whose sensitivity can be as high as 90% in diagnosing paragangliomas and it minimizes fetal exposure to ionizing radiation , 14. UltSurgical resection is the mainstay of treatment during pregnancy . PresurgDue to the extremely high mortality rate of this rare but treatable condition, it is essential to conduct a meticulous history taking and clinical examination so that early diagnosis and treatment can save maternal and fetal lives and improve clinical outcomes.


We report a case of a 51-year-old man who was diagnosed with systemic reactive (AA) amyloidosis in association with untreated glomus jugulare and glomus caroticum tumors. He refused radiotherapy and renal replacement therapy. Paragangliomas, although rare, should be considered one of the tumors that can result in AA amyloidosis. Paragangliomas are uncommon true neoplasms that arise from paraganglionic chemoreceptor cells. Other terms used to describe these lesions include glomus tumors, nonchromaffin paragangliomas, chemodectomas, and carotid body-like tumors . SystemiA 51-year-old man with a glomus jugulare tumor was referred to our nephrology clinic with suspected renal failure and proteinuria. Twenty-nine years prior, the patient had an operation for glomus jugulare tumor that resulted in extensive hemorrhaging and required a two-month hospitalization. Since the operation, the patient had not sought any follow-up care for his glomus tumor. His brother was diagnosed as having a glomus caroticum tumor. The patient complained of pulsatile tinnitus and a mass in his right external ear canal.On admission, the patient's blood pressure was 100/70 mm Hg, pulse rate 78/min, and temperature 36.6°C. He had 1+ pitting edema in the lower extremities. On otolaryngologic exam, the patient had a red-whitish mass protruding from the middle ear to external ear, filling the external ear canal . Audiome4  is formed in the liver as an acute-phase reactant secondary to provocation of inflammation by cytokines . The tumIn patients with AA amyloidosis, if the underlying pathologic condition is controlled or eliminated, deposition of AA fibrils may be halted and perhaps even reversed . This isIn conclusion, this patient had long-standing paragangliomas that resulted in eventual complications due to tumor-associated systemic amyloidosis. Paragangliomas, although rare, should be considered one of the tumors that can result in AA amyloidosis.The patient presented here had long-standing paragangliomas that resulted in eventual complications due to tumor-associated systemic amyloidosis. Paragangliomas, although rare, should be considered one of the tumors that can result in AA amyloidosis.


AIP) gene. A 50-year-old male presenting with headache was diagnosed with a large pituitary tumor by magnetic resonance imaging (MRI). His visual fields were intact and he exhibited no features of CD. Owing to an exuberant response to synacthen, an overnight dexamethasone suppression test was performed revealing inadequate suppression of plasma cortisol (419 nmol/l). Owing to tumor growth and visual field impairment, he underwent transsphenoidal surgery and developed hypocortisolemia. The pathology specimen revealed a sparsely granulated corticotrope adenoma. Postoperative MRI showed a large tumor remnant. The patient developed skin hyperpigmentation and a synacthen test demonstrated high basal and stimulated cortisol levels; an overnight dexamethasone suppression test showed no suppression (791 nmol/l) and elevated plasma ACTH levels (135 ng/l). A transcranial operation was performed followed by radiotherapy. Two months after radiotherapy, he developed secondary adrenocortical failure. Genetic testing revealed an AIP variant of unknown significance (p.R16H) without loss of the normal AIP allele in the tumor. A literature review showed ten CD patients with AIP gene variants, of whom five (including our case) were p.R16H. CD is occasionally dominated by pituitary tumor growth rather than symptoms of hypersecretion. The particular AIP gene variant identified in our patient is shared by four other reported cases of CD. Future studies are needed to assess whether the reported AIP gene variant is more than just coincidental.A patient of Cushing's disease (CD) characterized by a large tumor and only subtle symptoms of hormonal hypersecretion was examined. The patient had a germline variant in the aryl hydrocarbon receptor-interacting protein (CD is occasionally dominated by pituitary tumor growth rather than symptoms of hypersecretion.Resolution of both tumor remnant and hormonal hypersecretion may occur within 2 months after postoperative radiotherapy.The particular AIP gene variant identified in our patient is shared by four other reported cases of CD. Cushing's syndrome refers to the clinical manifestations induced by chronic exposure to excess glucocorticoids. It is a rare condition with an annual incidence of 3/million. It is, however, a frequently entertained differential diagnosis as it shares symptoms and signs with more prevalent conditions, such as type 2 diabetes and polycystic ovary syndrome. In addition, both the diagnosis and treatment of Cushing's syndrome are challenging, which have important prognostic implications. Cushing's disease (CD), defined as pituitary adrenocorticotropic hormone (ACTH) excess, is the most frequent cause of endogenous Cushing's syndrome with an incidence of 2/million per year. It is usually due to a microadenoma  and may be difficult to visualize; in contrast, macroadenomas  comprise only 4–10% of diagnosed patients AIP) gene have been shown to play a casual role in the development of pituitary adenomas, in particular large somatotropinomas occurring in young patients The etiology and pathogenesis of corticotrope adenomas remain unclear and the majority appears to be sporadic. In a minority of cases, Cushing's syndrome is associated with genetic syndromes such as McCune–Albright syndrome, MEN1, and Carney complex. More recently, germline mutations in the aryl hydrocarbon receptor-interacting protein  and exhibited no clinical features of anterior pituitary insufficiency, CD or acromegaly, but he did present symptoms of diabetes insipidus with pronounced thirst, polydipsia and polyuria (∼6 l/24 h), also during the night. Iodothyronine and thyroid-stimulating hormone levels were in the low normal range; a short synacthen test showed no evidence of ACTH insufficiency (cortisol levels (nmol/l): 654  and 981 (30 min)). The patient was started on oral desmopressin, and within 1 month, he was also substituted with levothyroxine and testosterone. A neuro-ophthalmological examination revealed intact visual fields. Owing to a moderately elevated serum insulin-like growth factor 1 (IGF1) level  and the exuberant response to ACTH stimulation, both a growth hormone (GH) profile during an oral glucose load and an overnight dexamethasone suppression test were performed. The former disclosed a near-normal nadir GH level of 0.32 μg/l (<0.30 μg/l), but the patient failed to suppress his morning plasma cortisol (nmol/l) to overnight dexamethasone (648 (afternoon) and 419 (morning)) combined with an unsuppressed ACTH level of 62 ng/l (7–64 ng/l). An MRI performed 7 months after the first one indicated tumor growth  performed due to a 1-year history of headaches. Twelve months before this, the patient underwent unilateral orchidectomy for a testicular seminoma and was in remission. In addition, the patient had a history of transitory cerebral ischemia and has been recently diagnosed with type 2 diabetes. The initial MRI revealed a pituitary macroadenoma with suprasellar extension a. The par growth b. Human 2). A postoperative GH profile with an oral glucose load showed a slightly elevated nadir GH level (0.37 μg/l) and serum IGF1 level of 248 μg/l (55–220 μg/l). An MRI performed 4 months after the operation demonstrated a large suprasellar tumor remnant; somatostatin analog treatment (Sandostatin LAR 10 mg/4 weeks) was attempted to allow for tumor shrinkage and normalization of GH. A repeat MRI after 6 months showed tumor growth but normal visual function, and treatment with a dopamine agonist (cabergoline 0.5 mg twice weekly) was added. The patient also appeared hyperpigmented and a synacthen test demonstrated high basal (797 nmol/l) and stimulated (1084 nmol/l) cortisol levels. Hydrocortisone treatment was discontinued and a subsequent overnight dexamethasone suppression test again showed lack of suppression (791 nmol/l) and elevated plasma ACTH levels (135 ng/l (reference range: 7–64 ng/l)). His glycemic control deteriorated and insulin treatment was started. The patient was underweight . The pathology specimen from the initial operation was re-evaluated and was shown to contain periodic acid-Schiff (PAS) positivity and ACTH immunoreactivity in a pattern consistent with a sparsely granulated corticotrope adenoma (AIP mutation and a variant of unknown significance was identified (p.R16H). The patient had no family history of pituitary disease. The seminoma and the pituitary adenoma tissue did not show any loss of the normal AIP allele  and ACTH levels (135 ng/l). Immunohistochemistry showed PAS positivity and ACTH immunoreactivity in a pattern consistent with a sparsely granulated corticotrope adenoma. Genetic testing indicated the MRI after the transcranial operation revealed a suprasellar tumor remnant and fractionated conventional radiotherapy was initiated 3 months postoperatively.A synacthen test was performed 2 months after the conventional radiotherapy and revealed secondary adrenocortical failure. The patient developed permanent panhypopituitarism and is currently treated with hydrocortisone, levothyroxine, testosterone, GH, and desmopressin.This case of CD is educational in several respects. The presenting symptom was headache, which led to an MRI revealing a pituitary tumor. It is important to recognize the spectrum of pituitary ACTH excess associated with corticotrope adenomas. The majority of patients with CD are diagnosed on the basis of classic symptoms and signs of hypercortisolism; the traditional challenge in the context of CD is rather the opposite than in our case: a patient presenting with persuasive symptoms and signs but without a distinct pituitary tumor. Most ACTH-producing pituitary adenomas are microadenomas (<10 mm in diameter); these small tumors are usually composed of basophilic, densely granulated corticotropes. In contrast, a less frequent scenario is that of a macroadenoma associated with only subtle features of glucocorticoid excess; these ‘whispering’ adenomas, distinct from the truly silent corticotrope adenomas, are almost always composed of chromophobic cells with faint immunoreactivity for ACTH that can be easily missed Our patient is unusual in that his pituitary adenoma caused central diabetes insipidus, which is usually attributed to other mass lesions involving the pituitary stalk or occurs following pituitary surgery.The initial endocrine evaluation included an elevated serum IGF1 measurement and a subsequent GH profile following an oral glucose load demonstrated a slightly elevated nadir GH level indicative of acromegaly despite the absence of classical symptoms and signs. Only few cases of pituitary tumors secreting both GH and ACTH are reported; in our case, the tumor tissue submitted for pathology examination was completely negative for Pit1 and GH and had no features of a somatotrope adenoma. We therefore find it unlikely that the tumor co-secreted GH, and it should be noted that elevated serum IGF1 levels have previously been reported in Cushing's syndrome (CS) Given the fact that the patient had an ACTH-producing adenoma and only a small proportion of the tumor was removed, it was also unexpected that hypocortisolemia developed after the first operation. The rapid resolution (4 months) of hypercortisolemia following the conventional radiation therapy targeting the suprasellar tumor remnant, and development of secondary adrenocortical failure are also unusual, as the effects of conventional radiation therapy on hypersecretion and tumor shrinkage usually set in after several years. We suggest that an infarction developed in the pituitary tumor remnant, which, to our knowledge, has been reported in only one previous case – also a patient with CD – following radiation therapy AIP gene c.47G>A, p.R16H. AIP mutations are associated with familial pituitary adenomas and more than 200 cases have been described displaying over 70 different mutations AIP mutations are predominantly GH-producing macroadenomas. Thus far, ten cases of CD have been reported AIP variant reported in our case is considered of unclear pathological significance. It has been identified in 0.3% of European Americans and 0.08% of African Americans in the general population. An in vitro functional study based on binding to one of the partners of AIP phosphodiesterase subtype 4A5 did not demonstrate a significant functional change attributable to this gene variant. Nevertheless, this gene variant has been reported in four additional patients with CD  has been linked to CD Finally, our case was diagnosed with a variant in the  with CD . In accoWritten informed consent was obtained from the patient.AIP mutation. S L Asa was responsible for pituitary tumor histology, the histological perspective of the case rapport. M Korbonits was responsible for the genetic perspective of the case rapport. J O L Jørgensen was in charge of the patient treatment and the CD perspective of case rapport.P T Dinesen and J Dal were involved in writing the case report and data collection. P Gabrovska, K Stals, and J Denes performed genetic examination of the pituitary adenoma tissue. M Gaustadnes and C H Gravholt performed genetic testing for


The posterior reversible encephalopathy syndrome (PRES) is a rare clinical-radiological entity well described with typical clinical and radiological manifestations. Atypical presentation, especially in imaging, exists. The authors report here two cases of posterior reversible encephalopathy in which imaging aspects were atypical, mimicking, in the first case, hemorrhagic cerebral metastasis of cholangiocarcinoma and, in the second case, a brain tumor. The diagnosis has been retrospectively rectified due to clinical and radiological outcome. Described for the first time in 1996 by Hinchey et al. . In imagWe report here two cases of posterior reversible encephalopathy in which imaging aspects were atypical, mimicking, in the first case, hemorrhagic cerebral metastasis of cholangiocarcinoma and, in the second case, a brain tumor because of its unilateral localization.A 71-year-old male patient with known hypertension and history of myocardial infarction that required a double bypass surgery 38 years ago, cardiac arrhythmias due to atrial fibrillation (AF), and an old thrombocytopenia has been urgently admitted for onset of neurological disorders manifested as visual disturbances. Clinical examination found an acute hypertensive episode with an elevated blood pressure of 180/110 mmHg.A brain noncontrast CT-scan was performed and found bilateral occipital nodular hematomas, predominantly in the right hemisphere, surrounded by edematous hypodense white matter . Faced wA brain MRI-scan performed 6 weeks later Figures  and anotGiven the evolution of radiological images and the disappearance of neurological disorders in the absence of a specific treatment except the one for controlling the hypertension and the chemotherapy for his cholangiocarcinoma, the final diagnosis was intracerebral hemorrhagic complicated form of PRES. For his hepatobiliary tumor he had initially benefited from chemotherapy by GEMOX that allowed stabilizing the lesions and in a second phase he had a right hepatectomy.A 77-year-old woman was admitted in emergency room for persistent headache in a context of high blood pressure at 175/115 mmHg. Noncontrast and contrast brain CT-scans were performed and showed a diffuse area of unilateral hypoattenuation in the right white matter in the parietooccipital lobes finger in glove appearance with a discrete mass effect on the occipital horn of the lateral ventricle, without contrast enhancement, in favour of a space occupying lesion of the brain Figures . The braPosterior reversible encephalopathy syndrome (PRES) is a clinical-radiological entity that was well described by Hinchey et al.  in 1996 The pathophysiology of PRES remains controversial. The two main hypotheses contradict each other. One involves impaired cerebral autoregulation responsible for an increase in cerebral blood flow (CBF), whereas the other involves endothelial dysfunction with cerebral hypoperfusion . This hyTypically, PRES presents as widespread, symmetrical, and bilateral, usually reversible vasogenic edema, cortical and subcortical, which backs up into the deep white matter as it becomes more severe , 7. PredAlthough typically symmetrical and bilateral, asymmetrical or unilateral distributions of vasogenic edema in PRES can also be seen. This is designed as “tumefactive PRES” by McKinney et al. . That is∗, but it is antiquated relative to SWI (susceptibility-weighted imaging) and other newer and postprocessed sequences. The incidence of hemorrhage (particularly microhemorrhage) is higher; its frequency is more than 50%; McKinney et al. found 58.1% at presentation and 64.7% at follow-up [Our first case has been diagnosed with an intracerebral haemorrhagic complicated form of PRES. The aspect was bilateral multiple focal hematomas. Cerebral hemorrhage is a potential complication that is known to occur in PRES. Hemorrhage is said to be an uncommon complication in PRES (5%  to 17%  of patieollow-up . The patollow-up , 14. In In all of our two cases, the diagnostic of PRES has been made after partial or complete regression of the initial clinical and radiological abnormalities. In some cases, the diagnosis of PRES remains in doubt. In this situation, regression of the clinical and radiological abnormalities with appropriate treatment supports the diagnosis. Thus, repeated brain imaging is helpful .The posterior reversible encephalopathy syndrome is a rare radioclinical syndrome. This diagnosis is easy when the clinical and radiological presentation is typical. In atypical forms, it poses the problem of differential diagnosis, which can be confused with a primary brain tumor or brain metastasis. It is often the evolution that allows rectifying the diagnosis.


Pyonephrosis is an uncommon disease that is associated with suppurative destruction of the renal parenchyma in adults. Upper urinary tract infection and obstruction play a role in its etiology. Immunosuppression from medications (steroids), diseases , and anatomic variations  may also be risk factors for pyonephrosis. Fever, shivering, and flank pain are frequent clinical symptoms. On physical examination, a palpable abdominal mass may be associated with the hydronephrotic kidney. Septic shock and death can occur if the disorder is not treated with urgent surgery. After the acute phase, most patients are treated with nephrectomy. In this paper, we share the etiology, clinical features, diagnosis and treatment of pyonephrosis using the background of a case with giant pyonephrosis developing due to a kidney stone, the most common cause of upper urinary tract obstruction. Pyonephrosis is a disease causing suppurative destruction of the renal parenchyma. If it is not diagnosed early, it can worsen rapidly and cause the death of the patient with the development of septic shock. Clinical findings of the patients vary from asymptomatic bacteriuria (15%) to sepsis. Fever, chills, and flank pain are most commonly seen.Radiological tests such as ultrasound (USG), computed tomography (CT), urography, and magnetic resonance imaging (MRI) are used in the diagnosis of pyonephrosis. If the pus detected as a result of the investigations is not surgically drained, antibiotics may not be very effective. In this context percutaneous or open nephrostomy or ureteral catheter insertion is appropriate. However, nephrectomy can be considered as a good treatment option if the contralateral kidney is intact in case of a damaged kidney that has lost most of its functions , 2.Our case was a 65-year-old male who suffers from left flank pain for 4 years and developed abdominal distention 1 month ago. Left hydronephrosis was found by the investigations performed .Whole abdomen CT revealed a normal right kidney but a cystic mass 13 × 24 × 34 cm in size including calcified areas in the cystic density, without a solid component but with multiple thin septa completely filling the abdomen and passing from the midline to the right in the left kidney region. The patient underwent radical nephrectomy with a diagnosis of nonfunctioning left kidney due to giant pyonephrosis caused by a stone .The report from our pathology laboratory was “hydronephrosis and chronic pyelonephritis” . The macroscopic appearance was nephrectomy material 1150 g in weight, 32 × 15 × 5 cm in size, and pink brown in color, showing cystic enlarged nodularity .Two stones of 1 cm diameter were removed during the operation. Seven liters of purulent material was also drained . No growEscherichia coli, Enterococcus species, Candida, Klebsiella, Proteus, etc.) in the infection group and stones (staghorn in 75%), yeast balls, metastatic tumors , pregnancy, and ureteropelvic junction (UPJ) obstruction in the obstruction group. It may also appear as a complication of past urologic surgery and chronic pyelonephritis.Pyonephrosis is a rare disease and upper urinary tract system infections and obstructions play a role in its etiology. There are multiple infectious agents  to sepsis. Fever, chills, and flank pain are most commonly seen. Rabii et al. found lumbar pain in 70% of 14 pyonephrosis cases, together with painful lumbar region on examination in 5 cases and fever, chills, and pyuria in all cases . The etiUSG and CT are the methods generally used for the diagnosis of pyonephrosis. However, CT is more effective than USG as it identifies renal function, causes of obstruction , and abdominal pathologies such as hydronephrosis better. Fultz et al. found that CT was a very sensitive radiological diagnostic method in the study they conducted on 17 pyonephrosis plus hydronephrosis cases . We alsoAntibiotics have no effect in pyonephrosis unless the pus is surgically drained. Percutaneous nephrostomy and urethral catheter insertion are therefore necessary. Studies show percutaneous drainage to be a fast, trusted, and effective diagnostic and therapeutic method , 3, 5.Radical nephrectomy can be the preferred treatment for a kidney that has lost most of its function if the contralateral kidney is normal. Nephrectomy has been found to have fewer complications compared to other treatments , 7. LeftWe did not find any other case with pyonephrosis of a similar size in the literature. Giant pyonephrosis is rare now due to the advanced diagnostic methods and medical and surgical treatment.In conclusion, pyonephrosis should be diagnosed early and surgery should be performed immediately. Radical nephrectomy can still be considered as a potential treatment that is curative. However, the best treatment is to detect and treat the stones that play a major role in the etiology.


Intestinal neuronal dysplasia (IND) type B is characterized by malformation of parasympathetic plexus and manifests at more than 6 month of age with progressive severe constipation. We report a case of IND type B presented with bowel dilatation on antenatal scan and neonatal intestinal obstruction which is unusual with this type of IND. A full-term male neonate weighing 2290gm born to a 21-year old woman  by spontaneous vaginal delivery, was admitted to with suspicion of intestinal atresia. Antenatal scan, done at 26-week of gestation, showed marked dilatation of bowel loops (16mm). After 4 weeks, follow-up ultrasonography suggested an increase in the diameter and the number of loops (20mm). On physical examination, mild tachypnea and abdominal distension were observed. Abdominal x-ray showed small bowel dilatation. Because of suspicion of intestinal atresia, an exploratory laparotomy was performed. At surgery approximately 50 cm of bowel distal to the ligament of Treitz was dilated, and an obstruction was found at the end of this segment. The area of obstruction consisted of a dilated meconium-filled proximal bowel segment, an abrupt transition zone, and a distal bowel that was small in caliber . Jejunal enterotomy was performed and bowel was decompressed. The 10-cm long distal jejunal segment including transitional zone was resected. Stoma was not made and end-to-end anastomosis was done. The disparity of lumen between the proximal and distal segments was five fold. Distal bowel wash-outs with gastrograffin were started on postoperative day five. Genetic study for cystic fibrosis was negative. Histopathological examination of the resected bowel segment established the diagnosis of IND  on the basis of hyperganglionosis with giant ganglia, ectopic ganglion cells in the lamina propria, and positive immune-histochemical staining for the S-100 protein. Unfortunately, enzyme histochemistry for acetylcholinesterase (ACH) was not performed owing to lack of fresh tissue. A final diagnosis of type B IND was made. Oral feeding was started on day 10 after the operation, and was gradually increased. Patient was discharged on postnatal day 27. He is doing fine on 6-month follow-up.IND was first described in 1971 by Meyer-Ruge as a hyperplastic malformation of the enteric plexus.[1] IND is classified into two clinical and histologically subtypes as types A or B.[2] Type A of IND is congenital hypogenesis or agenesis of the sympathetic innervation, which occur in less than 5% of all IND cases. Type B of IND is characterized by hyperplasia of the parasympathetic plexus and accounts for 95% of all IND cases. IND type A manifests with episodes of abdominal distension, bowel obstruction and diarrhea with blood in stool in neonatal period. Type B IND presents with chronic constipation with or without abdominal distension after 6 months of age.[3] In our case, the presence of antenatal dilated bowel loops was suggestive of fetal bowel obstruction; moreover patient presented with neonatal inetstinal obstruction. Antenatal identification reduces the perinatal morbidity and mortality by early resuscitation and suitable surgical interventions except in rare situations.[4] Our differential diagnosis on the basis of history and abdominal radiograph was that of intestinal atresia.Typical histological features of IND type B are hyperganglionosis, giant ganglia, and ectopic ganglion cells and increased ACH enzyme activity in the lamina propria and around submucosal blood vessels.[5] Our patient had similar findings however ACH enzyme was not performed. To summarize, IND type B presenting with fetal bowel dilations and neonatal intestinal obstruction is a rare occurrence. IND must be kept in differential diagnosis of fetal bowel dilatation on antenatal scan.Source of Support: NilConflict of Interest: None declared


Cranial-retained surgical sponges (gossypiboma or textiloma) are rare incidents and mostly asymptomatic. However, they can be confused with other masses such as a hematoma abscess or tumor. During early stages, some gossypibomas can cause infection or abscess formation.A 22-year-old Turkish female who had frontal lobe brain surgery to remove an abscess 2 months previously was admitted with complaints of headache and vomiting.Gossypiboma was confirmed in the patient. Following cranial surgery, gossypiboma should be considered as a differential diagnosis of recurrence of previous surgical operations. Although rare, retained surgical sponges can occasionally be found after neurosurgical operations. A “textiloma” describes a mass lesion consisting of a surgical sponge. “Gossypiboma” describes both the mass of sponge and a foreign-body reaction around it , 2. ThesA 22-year-old Turkish female was admitted with headache and vomiting. She presented with a history of frontal lobe brain abscess which had been operated on in the previous 2 months. The abscess was surgically resected using resorbable hemostatic agents in the operation area. After the initial surgery, third-generation cephalosporin was started and continued for 10 days. The neurologic examination was normal. There was no fever and routine laboratory tests  were all normal. Microbiologic and pathologic investigations of the abscess revealed no pathogens, only exudative encapsulation. Two months after the operation, the patient admitted to our clinic with increasing frequency of severe headaches. Subsequent computed tomography (CT) and magnetic resonance imaging (MRI) scans revealed a new contrast enhancing mass in the frontal lobe at the site of her prior abscess which was associated with extensive edema in the surrounding brain , gossypiboma  gauzoma  and muslinoma (from muslin) are the historical terms that have been given to foreign-body related inflammatory pseudo-tumors. Specifically, these terms refer to tumors arising from a retained, non-absorbable cotton matrix that is either inadvertently or deliberately left behind during surgery, together with the associated inflammatory reaction.All classes of resorbable and non-resorbable hemostatic agents may produce textilomas as an allergic response. Textilomas may present with neuroimaging features that mimic recurrent tumor, abscess, and hematoma. In the differential diagnosis of a mass lesion arising after prior intracranial surgery, the possibility of textiloma should be considered along with recurrent tumor, radiation necrosis, and abscess.Awide variety of synthetic materials may be left in place during intracranial procedures, e.g., silicone coated sheets, which are used as a dura mater substitute for repair of dural defects. Microscopic remnants of cotton gauze of no clinical consequence are often inadvertently left in the surgical field and subsequently identified incidentally on microscopic examination of a specimen obtained at repeat surgery. These agents and other foreign substances that are deliberately introduced to the central nervous system may induce an excessive inflammatory foreign-body reaction –6.Many different kinds of hemostatic agents, absorbable and non-absorbable, are used to control intraoperative bleeding in neurosurgical operations. Non-absorbable materials include various forms of cotton pledgets and synthetic hemostats, which should be removed before surgical closure .In the general surgical literature, the incidence of textiloma is highest following abdominal surgery, followed by orthopedic procedures. Textilomas have been reported in all major anatomic compartments: chest, retroperitoneum, extremities, head and neck , 9. The After surgery, the body can react to foreign bodies such as retained sponges in two ways: (1) exudative tissue reaction which leads to acute abscess formation, and (2) aseptic fibrous tissue reaction, which involves slow adhesion formation such as encapsulation and granuloma formation .Hemostatic agents may produce clinically symptomatic, radiologically apparent mass lesions. When considering a mass lesion arising after intracranial surgery, the differential diagnosis should include textiloma along with recurrent tumor abscess and radiation necrosis.CT scans can be useful in cases of suspected lesions. However, gossypibomas may not be easily recognized even on CT scans. In our case, the CT scan showed a hyper-dense mass lesion, although it was not diagnostic for the lesion , 13. MRIIn patients with history of cranial operation, gossypiboma should always be a consideration in the differential diagnosis of newly found lesions, as it is believed that they are much more common than reported. MRI is the best radiologic modality for the diagnosis. However, no pathognomonic radiologic characteristics are defined for these lesions. For this reason, the definitive diagnosis must be mainly aided by the physician and the intraoperative findings. Moreover, although gossypibomas are rare, their occurrence is still a possibility. Thus it must be remembered that careful inspection of the surgical field before closure is still an important basic rule in surgery.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


To date, there have been less than a 100 confirmed case reports of primary cardiac malignant fibrous histiocytomas, a rare form of sarcoma. In this report, we discuss the case of a 15-year-old girl who initially presented with a histiocytic cerebral sarcoma that was treated with aggressive resection and chemotherapy. Three years later, the same patient developed increasing shortness of breath and was found to have a high-grade pleomorphic undifferentiated cardiac sarcoma that likely represents the primary tumour from which the cerebral lesion metastasised. This represents an extremely unique case; in 2010, a research group in Germany claimed the very first description of a true cardiac sarcoma with brain metastasis . Neverth This report highlights the case of a young patient who presented with an extremely rare histiocytoma brain tumour that was treated with surgery and extensive chemotherapy. During follow–up, two years later, however, she was found to have a cardiac sarcoma that likely represented the primary tumour of which the original brain deposit was a metastasis. We will present the literature on histiocytomas and primary cardiac and cerebral sarcomas that are all rare and form conclusions to help assist everyday practice when faced with these tumours.Cardiac tumours are rare, studies at autopsy have shown a prevalence of 0.001–0.03% , and of Our case report starts in April 2013 when a 15-year-old girl presented to her local accident and emergency department with a two-month history of an occipital headache, three weeks of intermittent diplopia and a one-week history of vomiting on multiple occasions daily. An insightful emergency doctor ordered an MRI scan with IV contrast that was performed the same day . This reThe following day, neurosurgeons performed a right-sided frontal-pterional craniotomy to remove the space occupying lesion. Unfortunately, post-operative scans showed significant residual disease and the case was taken to neurosurgical MDT mid-May. Histology, available at this stage, showed lymphocytic infiltration and a high-grade tumour that was INI1, vimentin, and histiocytic markers positive. The likely diagnosis of the brain tumour was suggested to be a histiocytic sarcoma, and further, imaging was suggested to exclude metastases or another primary location. Primary cerebral histiocytomas are even rarer than cardiac histiocytomas and as of 2013, only 10 have ever been reported . CounterA CT scan of the patient’s thorax, abdomen, and pelvis was performed to look for other sites of sarcoma, and this was reported as showing no evidence of lesions in either lung, liver, spleen, or kidneys. There was no lymphadenopathy or free fluid in the abdomen and pelvis. The patient was then treated as per UK CCLG High-Grade Glioma guidelines with concomitant focal radiotherapy and temozolomide followed by seven cycles of temozolomide where the patient was able to tolerate 3–4 out of five daily doses in each cycle. The patient concluded treated in March 2014 with no evidence of reoccurrence or any residual disease.In May 2015, the patient now 17 years old presented to her oncology follow-up clinic appointment complaining of increasing shortness of breath over a one-month period combined with lethargy and an isolated anaemia. She was assessed by haematology and diagnosed with Iron deficiency anaemia thought to be secondary to heavy menstruation that was subsequently treated. MRI brain with contrast was performed in mid-June, the frontal lobe appearances were unaltered from the preceding scan, and there was no evidence of disease relapse. However, in August 2015, the patient had a further drop in her haemoglobin this time without iron deficiency and had started having evening and night time fevers. A CT scan was performed to look for a source of the pyrexia of unknown origin. It showed a 4.6 × 3.5 × 3 cm filling defect in the left atrium . The radFive days later, a cardiac MRI scan was performed showing a tumour mass attached to the posterior wall of the left atrium lying very close to but not occluding the left lower pulmonary vein . The posPost-operatively, there was an initial deterioration in the patient’s ejection fraction from a pre-operative 51% down to 33%; however, later, in the month, this had an increased back to 48% all values measured by Simpson’s method. A post-operative cardiac MRI did not show any signs of post-operative re-occurrence. The patient has now begun doxycycline/ifosfamide chemotherapy and has had four cycles up until this point.A Sarcoma centre published 13-year data in 2007; of 1429 patients with sarcoma 14 were primary cardiacsarcomas and only a single one had a cerebral metastasis . AggressIn conclusion, this case, albeit of a rare presentation of sarcoma, highlights the need for extensive investigation at presentation, especially if an unusual primary location has been found. PET imaging at the time of diagnosis or after initial surgery would be extremely useful in these cases to assist in the identification of other metastatic sites or primary tumour location that may be missed on plain CT imaging. We would also recommend consideration of cardiac MRI imaging, if there is any potential concern that a primary heart tumour may be involved.


Dear SirA 4-month-old boy, first in birth order, born by spontaneous vaginal birth at full term, was brought with a complaint of flexion deformity of the left ring finger. His mother stated that the deformity had been noticed by the primary care physician during routine neonatal check-up at birth. There was no history of peri-natal trauma, infections, or rheumatologic / metabolic disorders and development milestones had been achieved normally. No prior intervention had been done for the complaint. On physical examination, there was triggering of left ring finger (4th digit) with a snap on flexion to about 60° . Palpation revealed a non-tender, firm nodule over the palmar side of the metacarpophalangeal joints of the affected finger. There was no other anomaly. Plain radiographs of both hands were normal. Parents were counselled and attached to the services of hand physiotherapy/orthotics division where a nocturnal splint was advised with passive daytime, home exercises of finger flexion/extension. However, due to lack of compliance, no benefit could be appreciated after follow up at 8 weeks of presentation. Hence, after informed consent, surgical release of A-1 pulley was undertaken under general anaesthesia and proximal tourniquet control . The deformity was markedly reduced after surgical release , but the final outcome could not be ascertained due to loss of patient to follow-up. Trigger finger (TF) is a rare disorder in paediatric age group [1] though TF is relatively common but the two represent distinct conditions [2]. TF tend to develop earlier in life than trigger thumb and the spontaneous recovery rate was higher in former. The aetiology has been suggested to be genetic though other identifiable causes may be occasionally present [3] and it is still controversial as to whether the congenital trigger digit is truly congenital or an early acquired condition [4]. TF may affect one or more digits [3] and usually affects one hand but bilateral cases are rarely reported in literature .The first line of management is conservative with dedicated home based physiotherapy and splintage  to maintain the IP joint in neutral extension or hyperextension [5]. Most of the cases respond. In case of failed conservative management, surgical exploration of flexor mechanism is undertaken to release A-1 pulley, with or without resection of one or both limbs of the sublimis tendon or an A-3 pulley release [6]. A-2 pulley release may be required in extremely rare situations when clinical examination and exploratory findings reveal triggering at A-2 pulley [7]. In our case, our conservative line of management failed primarily due to non-compliance on the part of guardians. The non-compliance remained the issue, even after the surgical release and hence, proper counselling of the guardians is stressed to ensure their cooperation in achieving successful outcome.Source of Support: NilConflict of Interest: None