Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare autosomal recessive congenital anomaly characterized by horizontal gaze limitation and progressive scoliosis. We investigated the underlying pathogenesis by incorporating diffusion tensor imaging and an electrophysiological study.A 55-year-old female patient presented to our clinic due to a chronic history of eye movement limitation since childhood. Her eye problem was followed by a progressive scoliotic change in her torso during junior high school. Neurological examinations revealed remarkable conjugate horizontal but not vertical gaze palsy. Her pupils were isocoric, with a prompt response to light reflex and convergence. Her vision, including visual acuity and field, were normal. No pathological signs of muscle tone, muscle power, deep tendon reflex or coordination were revealed. There was no associated family history, and no diseases involving other systems were noted. On reviewing her past medical history, X-rays revealed scoliotic changes of her thoracic and lumbar spine. Brain magnetic resonance imaging showed a midline cleavage at the tegmentum (split pons sign) and butterfly configuration of the medulla, consistent with HGPPS. Color-coded diffusion tensor imaging in our patient revealed absence of decussation of the superior cerebellar peduncle. In tractography, the pontocerebellar tracts and fibers within the inferior cerebellar peduncle, deemed to be primarily dorsal spinocerebellar and vestibulocerebellar tracts, appeared to be agenetic. The tegmentum was compromised secondary to dorsal displacement of the corticospinal tracts. Of note, the bilateral corticospinal tracts remained uncrossed at the level presumed to be the pyramidal decussation. A somatosensory evoked potential study also revealed predominantly ipsilateral cortical sensory responses.Our study confirmed that a compromised tegmentum secondary to dorsal displacement of the corticospinal tracts and poorly-developed afferent fibers within the pontocerebellar tracts and inferior cerebellar peduncle to be the main neuroanatomical anomalies responsible for the clinical presentations of HGPPS. In addition, the uncrossed nature of the majority of pyramidal and proprioceptive sensory systems was confirmed.The online version of this article (10.1186/s12883-018-1081-9) contains supplementary material, which is available to authorized users. ROBO3 gene, as the ROBO3 protein determines axon path finding, crossing, and resultant hindbrain morphogenesis in human [Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare congenital disorder with autosomal recessive inherence, characterized by impaired conjugate horizontal eye movements and progressive scoliosis developing in childhood and adolescence , 2. Genein human , 4. Althin human , 4, studin human , 6 and ein human  are limiA 55-year-old female patient presented to our clinic due to a chronic history of eye movement limitation since childhood. She had difficulty in moving her eyes to the right or left, and therefore relied heavily on turning her neck. This debilitating condition was followed by progressive scoliotic changes in torso during junior high school. Her cognition was intact, and she had no history of seizures or vision loss. Neurological examinations revealed remarkable conjugated horizontal but not vertical gaze palsy Fig.  . (AVI 2703 kb)2, plus one b = 0 image. The imaging parameters were: TR/TE = 8000/82 ms, matrix size = 128 × 128, field of view = 240 mm, slice thickness = 3 mm with no intersection gap, number of excitations = 2, number of slices = 67, scan time = 5 min and 58 s. The post-processing software Functool  was used to generate fiber tracts. The seed points were placed in the brainstem based on T2-weighted images. The cutoff value for fractional anisotropy was 0.20 . In color-coded DTI, the transverse pontine fibers  which separated the corticospinal tracts anteriorly from the medial lemniscus and medial longitudinal fasciculus posteriorly in the normal healthy control, were abnormally located anterior to the corticospinal tracts  , 19, but reports , 6, 8, 9 reports –17.This case report supports the hypothesis that maldevelopment of the tegmentum plays a crucial role in the pathogenesis of HGPPS. Horizontal gaze palsy could be explained by a compromised medial longitudinal fasciculus, whereas scoliosis could be due to agenesis of the pontocerebellar tracts and afferent fibers within the inferior cerebellar peduncles. In addition, the uncrossed nature of the majority of both the pyramidal and proprioceptive sensory systems was confirmed.


Lipomatous lesions are common musculoskeletal lesions that can arise within the soft tissues, bone, neurovascular structures, and synovium. The majority of these lesions are benign, and many of the benign lesions can be diagnosed by radiologic evaluation. However, radiologic differences between benign and malignant lipomatous lesions may be subtle and pathologic correlation is often needed. The use of sonography, computed tomography (CT), and magnetic resonance imaging (MRI) is useful not only in portraying fat within the lesion, but also for evaluating the presence and extent of soft tissue components. Lipomas make up most soft tissue lipomatous lesions, but careful evaluation must be performed to distinguish these lesions from a low-grade liposarcoma. In addition to the imaging appearance, the location of the lesion and the patient demographics can be utilized to help diagnose other soft tissue lipomatous lesions, such as elastofibroma dorsi, angiolipoma, lipoblastoma, and hibernoma. Osseous lipomatous lesions such as a parosteal lipoma and intraosseous lipoma occur less commonly as their soft tissue counterpart, but are also benign. Neurovascular and synovial lipomatous lesions are much rarer lesions but demonstrate more classic radiologic findings, particularly on MRI. A review of the clinical, radiologic, and pathologic characteristics of these lesions is presented. Musculoskeletal lipomatous lesions form a diverse group of entities which arise from a broad range of tissues and range from benign to malignant. The majority of these lesions arise within the soft tissues, but lipomatous lesions within the bone, neurovascular structures, and synovium are also seen. Many of these lesions demonstrate a classic imaging appearance which can confidently make a diagnosis by imaging alone. However, other lipomatous lesions exist on a spectrum where imaging alone is not enough to differentiate the malignant lesions from the benign. The clinical, imaging, and pathologic characteristics of a variety of these lesions are presented and discussed.Lipomas are benign soft tissue tumors composed of mature adipose tissue. These lesions are the most common soft tissue tumor, making up nearly 50% of all soft tissue tumors . LipomasThough these lesions are usually solitary, they can be multiple in 5–15% of patients , 5. ApprSuperficial lipomas can almost always be diagnosed clinically. In cases where imaging is obtained, lipomas have a characteristic appearance on ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI). Large lipomas may appear as a radiolucency on radiographs, but the finding is not diagnostic. Sonographically, the lesions appear as well-defined, oblong, echogenic mass without posterior acoustic enhancement. In the larger lesions, fine linear striations may be seen coursing parallel to the skin . Deep liOn non-contrast CT, the classic appearance of a lipoma is a circumscribed, homogeneously low (fat) density mass ranging from −120 to −65 Hounsfield units , 10. SimAnother complicating feature of some lipomas is mineralization, which is present in up to 11% of imaged lesions , 9. ThisHistopathologically lipomas are encapsulated tumors composed of mature adipocytes. Fibrous connective tissue may be seen which correlates with the thin septa seen on imaging. Lipomas have a rich vascular supply, though the tenuous vessels are often compressed by the large adipocytes. Marginal surgical excision is performed for symptomatic lesions , 12. In The main differential consideration for lipomas are liposarcomas, as some of these lesions can have a similar imaging and histologic appearance. Liposarcoma makes up 10–35% of soft tissue sarcomas , 16–19, The most common liposarcoma subtype is a well-differentiated liposarcoma, which makes up approximately half of these lesions , 17, 19.Myxoid liposarcoma, the next most common class of liposarcoma, occurs in the intermuscular space in up to 80% of the cases . This suLess common types of liposarcoma include pleomorphic, dedifferentiated, and mixed-type. Pleomorphic and dedifferentiated subtypes are both high-grade malignancies. Pleomorphic liposarcomas are large, multinodular, well-defined masses. Heterogeneity on imaging is related to internal hemorrhage and necrosis. These lesions are often difficult to diagnose by imaging given their relative lack of adipose tissue , 23, 25.Another benign fat-containing soft tissue lesion is an elastofibroma dorsi. This lesion classically occurs in the infrascapular region, deep to serratus anterior and latissimus dorsi. These lesions are bilateral in up to 60% of cases and when unilateral are more often seen on the right than left . Less coOne of the many subtypes of a lipoma is an angiolipoma, which is a soft tissue tumor composed of mature fat and small blood vessels. Angiolipomas are also known as hemangiolipomas, vascular lipomas, or fibromyolipomas. Originally, up to 17% of lipomas were felt to be angiolipomas , though Lipoblastomas are rare, benign mesenchymal tumors, which are discovered almost exclusively before three years of age . OriginaA hibernoma is a rare lipomatous lesion composed of brown fat affecting middle-aged patients. These lesions most commonly arise in the thigh and subcutaneous tissues of the shoulder, upper back, and neck , 39. ThoParosteal lipomas are benign lesions composed of mature adipose tissue. These lesions are much less common than their soft tissue counterparts, making up only 0.3% of all lipomas . ParosteAnother benign lipomatous lesion is an intraosseous lipoma. Patients are usually within their 4th to 5th decades of life at presentation and classically have focal pain. These lesions occur within the lower extremity up to 71% of the time ; the calA fibrolipomatous hamartoma (FLH), also known as lipomatosis of nerve by WHO, refers to adipocyte proliferation within a peripheral nerve. These lesions most commonly occur in infants and children and often present as compressive neuropathy of the affected nerve. The median nerve is involved in up to 50–85% of the cases , followeFrond-like lipomatous proliferation of the synovium is a benign process known as lipoma arborescens, occurring within joints, bursae, and tendon sheaths. Two categories are reported: the primary form of lipoma arborescens is idiopathic and is much less common than the secondary form, which occurs in the setting of osteoarthritis, rheumatoid arthritis, trauma, or synovitis , 50. BotLipomatous lesions are common throughout the musculoskeletal system and encompass both benign and malignant lesions. Though the radiographic manifestation of the lesion may be helpful in the diagnosis, radiographic findings can overlap among various lesions and histologic diagnosis may be necessary. Imaging can help in management by targeting the biopsy to the non-lipomatous components of the mass. Ultimately, the understanding of the wide range of musculoskeletal lipomatous lesions and their radiologic appearance is essential to the appropriate treatment of patients.The authors declare no conflict of interest in relation with this paper.


Leiomyosarcoma of the rectum is a rare entity that comprises less than 0.1 % of all rectal malignancies. Given the uncommon nature of this tumor and the controversy about its treatment we report one case and review the literature in an attempt to report a particular evolution and to discuss the most appropriate treatment.This case report describes the presentation of leiomyosarcoma of the rectum. A 30-year-old man from the north of Morocco presented with rectorrhagia and constipation. On physical examination we found a mass in his rectum approximately 6 cm from his anal margin. Pelvic magnetic resonance imaging showed a rectal mass with a parietal attachment that invaded the fascia and his perirectal tissue. Before any treatment he defecated spontaneously the tumor. On histopathological examination a diagnosis of leiomyosarcoma was made. An anterior resection of his rectum was performed with adjuvant radiotherapy at a dose of 50 Gy. After 1 year of surveillance, he has not presented any clinical symptoms and pelvic magnetic resonance imaging was normal.Unfortunately, histological analysis of a superficial biopsy of a rectal leiomyosarcoma may not be reflective of the entire tumor mass, and a diagnosis is based essentially on postoperative pathological examination. The optimal treatment modality in patients with rectal leiomyosarcomas is controversial. Prognosis is also poor; tumor size, histological grade, mitotic index, and local staging are the most known prognosis factors.The prognosis of rectal leiomyosarcoma is poor; more investigations are necessary to understand the progression of these tumors and to define an optimal treatment modality. Leiomyosarcoma of the rectum is a rare malignant tumor representing less than 0.1 % of all rectal malignancies [We describe the case of a 30-year-old man from the north of Morocco with no medical or surgical history and no family history of rectal disease. His dietary habits were characterized by a well-balanced consumption of meat and vegetables. He presented with a 1-year history of rectorrhagia and constipation.A digital rectal examination and rectoscopy revealed a mass in a left posterolateral position in his rectum approximately 6 cm from his anal margin that extended up to 10 cm; the tumor was not mobile. The rest of his physical examination was normal. During rectoscopy, the tumor was biopsied and histological analysis gave no evidence of malignancy. An abdominal and pelvic magnetic resonance imaging revealed an approximately 7.5-cm heterogeneous mass in his rectum. The tumor presented a parietal pedicle with an implantation base of 4 cm and invaded the fascia and the perirectal tissue. There was no evidence of distant metastasis Figs. .Fig. 1ImOn biologic examination, his hemoglobin was 10 g/dl and his white blood cells were 7000. His renal function was correct and creatinine clearance was 120 ml/minute. Hepatitis B and C serology was negative. He was hospitalized in our surgical department when he defecated spontaneously the tumor mass.Macroscopic pathology examination showed a large fleshy lobulated mass measuring 8 × 5 × 3 cm, with foci of hemorrhage. Microscopic examination revealed a cellular tumor arranged in interlacing bundles of spindle-shaped cells, with a marked cellular pleomorphism, a large and hyperchromatic nuclei, and abundant eosinophilic cytoplasm. Mitotic figures (12 per 10 high-power fields) and atypical mitotic figures were also noted. Immunohistochemistry revealed that neoplastic cells expressed desmin, h-caldesmon, and smooth muscle actin, and exhibited negative staining for CD117, CD34, and S-100. Subsequently, a diagnosis of grade II leiomyosarcoma was retained , the total prescribed dose was 50 Gy in 2 Gy per fraction, and the treatment duration was 36 days. At the end of radiotherapy, he had follow-up consultation every 3 months. At 1-year post-radiotherapy he is doing well and has no symptoms. Magnetic resonance imaging of his pelvis performed at 6 months post-radiotherapy showed no sign of recurrence . In fact, leiomyosarcomas are composed of cells that consistently express smooth cell actin and desmin, but they are negative for CD34 and CD117, these last are positive in the case of GIST [et al., in a review of 135 cases of rectal leiomyosarcoma, found a local recurrence rate of 67.5 % with local excision, compared to only 19.5 % with abdominoperineal resection, but there were no differences in survival rates between the two surgery modalities [et al. stated that local excisions of rectal leiomyosarcomas were almost always followed by recurrence no matter what grade of malignancy [The optimal treatment modality in patients with rectal leiomyosarcomas remains controversial. Radical surgery, such as anterior resection or abdominoperineal resection, is preferred to wide local excision. In fact, radical surgery is associated with a lower recurrence rate than wide local excision. Khalifa dalities –10. In alignancy . Howeverlignancy .et al. adjuvant radiation therapy is not effective [et al. [Because leiomyosarcoma is a relatively radioresistant tumor, and patients with rectal leiomyosarcoma can develop early hematogenous metastases, adjuvant pelvic radiation therapy is generally considered unsuccessful, as for Tjandra ffective . In cont [et al.  reported [et al. . Histopa [et al. .In our case, the evolution was unusual compared to other reported cases, with spontaneous expulsion of the entire tumor through the rectum, it may be explained by the necrosis of the tumor pedicle. We did not find cases with such an evolution in the literature, but we found some cases of spontaneous expulsion of ileal lipoma per rectum , 18. As Leiomyosarcomas of the rectum are rare tumors with few cases published in the literature. The diagnosis is confirmed by postoperative pathological examination; the overall prognosis is poor with reported survival of 20 to 40 % at 5 years . In view


Intracranial enterogenous cysts are rare and mainly occur in the posterior fossa. These cysts are usually extra-axial, midline, anterior to the brainstem, or at the cerebellopontine angle. We report a case of an enterogenous cyst in which diagnosis was difficult because the lesion showed atypical pathologic findings.A healthy 41-year-old man complained of paroxysmal occipital headaches lasting over a week, with increased severity for 3 days accompanied by slight dizziness and mild nausea. Magnetic resonance imaging showed a cystic lesion between clivus and brainstem. The patient underwent surgery for removal of the lesion via the right-sided far-later approach, and the lesion was resected totally. Although pathologic examinations showed a cyst had a mono-to-multilayered squamous epithelium, which are not accord with typical enterogenous cyst, the diagnosis was finally made based on the presence of basement membrane and immunohistochemical results.To confirm the diagnosis of enterogenous cyst, further pathologic examinations were performed and immunohistochemical characters were summarized. Chemical meningitis, a rare complication of enterogenous cyst, happened in current case. Use a syringe and aspirate the contents before incision might be a procedure to prevent chemical meningitis.To our knowledge, this is the first report of an enterogenous cyst associated with mono-to-multilayered squamous epithelium. Although during the follow-up time, no recurrence happened, long-term follow-up is needed. Enterogenous cysts are rare, benign, congenital lesions, which account for approximately 0.01% of CNS tumors, and commonly locate in the spinal canal, especially at the cervical or upper thoracic level  of brain revealed a hyper-dense lesion in the prepontine cistern  based on histological features (Wilkins and Odom Type A cysts have an epithelial lining composed of pseudostratified cuboidal or columnar epithelial cells mimicking the respiratory or gastrointestinal epithelium.Type B cysts in addition to the above structure, may be arranged in complex invaginations and have associated glands producing mucinous or serous fluid. These cysts may be composed of a range of associated tissue including smooth muscle, striated muscle, fat, cartilage, bone, elastic fibers, lymphoid tissue, nerve fibers, ganglion cells, or Vater Pacini corpuscles.Type C cysts, in addition to the findings in Type B, may be associated with glial elements such as ependymal cells of the wall.However, there are no classification exists for intracranial enterogenous cysts, mainly because of their rarity. In current case, we were unable to identify the correct histological type. The atypical pathologic results were major challenge in arriving at a definitive diagnosis. In our case, routine hematoxylin and eosin staining showed that the cyst wall was lined with mono-to-multilayered, non-ciliated, squamous epithelium instead of single or pseudostratified layer of ciliated or non-ciliated, columnar or cuboidal epithelium Fig. a, b. To Base on lumbar CSF examinations, infection was excluded and chemical meningitis was diagnosed, which was caused by cyst leakage intraoperatively (Gauden et al. To our best knowledge, we present the first case of intracranial enterogenous cyst associated with mono-to-multilayered squamous epithelium. Because of the lack of radiographic features and complicated appearance in light microscopy, histopathological examinations play an important role in diagnosis. In our case, during 6-month follow-up, the patient remained neurologically intact without radiographical evidence of cyst recurrence. Nevertheless, long-term follow-up is advised to detect the recurrence as early as possible.


HIAA secretion. To our knowledge, this represents the first reported case of ACTH and serotonin secretion, and likely GHRH or GH cosecretion, from a sacrococcygeal teratoma in an adult.A 60‐year‐old man with a pre‐existing stable sacrococcygeal teratoma developed acromegaly, ectopic Cushing's syndrome, and 5 A 60‐year‐old man presented with clinical features of acromegaly. He had a background of type 1 diabetes, diagnosed aged 28 years, hypertension and a C3–C6 laminoplasty aged 54 years for cervical stenosis. He had continued surveillance following an exploration and partial resection of a sacral dermoid cyst aged 8 years. CT imaging showed a complex, partly cystic, partly solid presacral mass with some erosion of the lower sacrum and coccyx  excess ). The pituitary gland was homogenous on T1‐weighted magnetic resonance imaging (MRI), with no focal mass lesion, although with a convex superior margin. Repeat pituitary imaging with T1‐ and T2‐weighted and T1 dynamic sequences showed no clear focal abnormality, but persistent convexity of the superior aspect of the gland. Due to the absence of a definite surgical target, he was initially treated medically. However, dopamine agonist and somatostatin analogue therapy were poorly tolerated due to unpredictable hypoglycemic events and gastrointestinal side effects and, after a period of time, medical therapy was stopped.11C‐methionine PET‐CT coregistered with volumetric MRI demonstrated diffuse hyperintensity within the pituitary, but no discrete lesion. Somatostatin receptor scintigraphy was reported to show physiological uptake in the bladder and physiological uptake in the pituitary sella. He underwent pituitary exploration and partial transphenoidal hypophysectomy; histology revealed normal pituitary tissue only, but 3 months postoperatively his serum IGF‐1 was just 11 nmol/L (age‐related reference range 5–26 nmol/L) and has remained normal thereafter. Anterior pituitary function was otherwise normal postoperatively.In the weeks preceeding surgery, he developed peripheral edema. Within weeks of surgery, he was admitted with severe fluid retention and abdominal bloating. He had gained 15 kg in weight with persistent hypokalemia despite the use of potassium‐sparing diuretics . There was no proteinuria, and serum albumin was 30 g/L and he had normal cardiac function as determined by MRI. A pulmonary embolus was identified on CT pulmonary angiogram but deemed not large enough to cause cardiac strain, and there was no associated inferior vena cava obstruction to explain his edema. He suffered recurrent infections: bilateral pneumonia, cellulitis, influenza, and norovirus‐associated gastroenteritis. Cortisol excess was suspected and confirmed on a low‐dose dexamethasone suppression test (cortisol postdexamethasone 1070 nmol/L) and an elevated midnight cortisol (>1750 nmol/L). ACTH was elevated ; with all tests performed after resolution of his acute illness. Postoperative cortisol ranged between 800 and 1728 nmol/L.Ectopic ACTH production from the presacral mass was considered, and a core biopsy of this lesion revealed a well‐differentiated neuroendocrine tumor with no mitoses seen and a Ki‐67 of 1% (WHO grade G1 Cushing's syndrome was treated with a combination of subcutaneous octreotide (to reduce ACTH secretion), metyrapone, ketoconazole and, subsequently when eucortisolemia could not be achieved, intravenous etomidate 2.5 mg/h. Once stabilized, he underwent bilateral adrenalectomy with resultant normalization of serum potassium and marked improvement of his edema.Despite adrenalectomy and good recovery, he continued to experience episodes of hypoglycemia, breathlessness, and anxiety with labile blood pressure. 5‐hydroxyindoleacetic acid (5HIAA)  and chromogranin B  were both elevated; chromogranin A was normal. Although the raised 5HIAA was likely responsible for some of these symptoms, they did not completely resolve with low‐dose somatostatin analogue treatment, despite improvement of 5HIAA to 65.3 μmol/24 h, raising the possibility of additional hormonal cosecretion. The most recent ACTH was 47 ng/L (posthydrocortisone), and IGF‐1 has remained normal both prior to, and after, recommencing somatostatin analogue.Sacrococcygeal teratomas usually show fully differentiated tissue derived from the ectoderm, endoderm, and mesoderm. A very small minority have malignant germ cell elements and may secrete alpha‐fetoprotein or beta‐HCG In this case, core biopsy and immunohistochemistry confirmed the diagnosis of a neuroendocrine tumor within a pre‐existing and well‐differentiated sacrococcygeal teratoma that had been stable since childhood, now with pluripotent neuroendocrine secretion. Surgery would be challenging and would be highly likely to lead to an ileostomy and bladder dysfunction and a significant risk of neurological sequelae. Targeted radiotherapy (to the solid component of the teratoma) has been considered, but has been declined by the patient, and at present, the tumor size remains stable.Well‐differentiated neuroendocrine tumors arising in testicular teratomas are well recognized and usually behave in an indolent fashion The cortisol hypersecretion (from ectopic ACTH) was inadequately controlled by medication, necessitating bilateral adrenalectomy. Previous case reports have suggested the existence of ACTH‐dependent Cushing's syndrome in a sacrococcygeal teratoma TB and AMB: involved in the patient's care at the Royal Devon and Exeter Hospital. WMD: involved in the patient's care at St Bartholomew's Hospital. MG: consulted and in charge of methionine PET, EK, and DMB: provided histology reports. TB: prepared the manuscript. All authors contributed to the editing of the manuscript.The authors have nothing to disclose.


We are reporting a case of solitary endobronchial papilloma located in posterior segment of the left upper lobe of the lung with malignant transformation and negative human papilloma virus (HPV) strains in a 40-year-old Saudi nonsmoker man. The patient had a concomitant tuberculosis (TB) infection. The patient received appropriate treatment in the form of anti-TB medication and surgical resection of the squamous cell carcinoma followed by chemotherapy. There was no evidence of tumor recurrence, resulting in a complete cure. We are reporting the case as well as a literature review related to the topic. Solitary endobronchial papilloma is a rare papilloma entity which is usually associated with human papilloma virus (HPV) infection and occurring in middle-aged smokers. It is usually classified into three categories: squamous cell papilloma, glandular papilloma, and mixed squamous cell and glandular papilloma. Malignant transformation is rare but has been reported. We are reporting a case of solitary endobronchial papilloma located in the posterior segment of left upper lobe of the lung with malignant transformation and negative for HPV strains.A 42-year-old nonsmoking Saudi man with a history of type 1 diabetes was referred to our institution for investigation of a chronic cough and mild exertional dyspnea. He denied a history of hemoptysis, weight loss, night sweats, or change in appetite. A physical examination revealed a temperature of 37.0°C, respiratory rate of 20 breaths/min, heart rate of 70 beats/min, blood pressure of 110/70 mmHg and oxygen saturation of 96% on room air. A chest examination was entirely normal. There was no lymphadenopathy. The rest of examination was unremarkable. A complete blood count, liver function test, erythrocyte sedimentation rate and C-reactive protein were within normal limits. Mycobacterium tuberculosis culture was also positive. Multiple biopsies were taken from the endobronchial lesion and histopathology was consistent with the diagnosis of squamous papilloma (The chest-X-ray was unremarkable. The Computed Tomography (CT) scan revealed a lobulated round nodule in the left upper lobe adjacent to the bronchus, measuring 1.8 × 1.6 cm with an absence of significant lymph nodes . Flexiblapilloma . There wapilloma . Repeateapilloma . The patWe are reporting a case of solitary endobronchial papilloma with malignant transformation and concomitant TB infection in a middle-aged, nonsmoking Saudi man. Our case is worth reporting considering the rarity of the solitary endobronchial papilloma. What is remarkable is the uncommon malignant transformation of this rare condition which is usually associated with smoking and positive HPV. In addition, the patient had a concomitant TB infection which we think has no relation with the endobronchial papilloma and considered as an accidental finding as the patient has no symptoms suggestive of TB infection. Up to our knowledge, there is no data to describe the case of endobronchial papilloma and concomitant TB infection. It is, however, worth further investigation. Our patient received appropriate treatment in the form of anti-TB medication and surgical resection of the squamous cell carcinoma followed by chemotherapy with no evidence of tumor recurrence resulting eventually in a complete cure.Solitary papilloma is commonly found in genitalia or oral cavity but it is very unusual to be found in the airways as in our patient. Solitary endobronchial papilloma is a rare papilloma representing only 0.5% of all types of papilloma and 0.3% of all lung tumors .Because of the rarity of solitary endobronchial papilloma, little is known about the epidemiology in terms of gender, age distribution, and the relationship with environmental factors. A review of 41 papilloma cases found 76% to occur in men . AdditioSolitary endobronchial papilloma is usually classified into 3 types: squamous papilloma (in smokers and associated with HPV), glandular papilloma, and mixed type. Two multiple papillomas of the tracheobronchial tree with malignant transformation have been first reported by DiMarco et al. . MalignaIn our case, there is a possibility of the existence of squamous carcinoma at the initial presentation of the patient. However, multiple biopsies were taken from the endobronchial lesion and histopathology was consistent with squamous papilloma. There was no evidence of malignancy or granuloma in all biopsies taken from the endobronchial lesion.Solitary endobronchial papilloma has a variety of clinical presentations. It can be without symptoms and discovered incidentally. If symptoms occurred, it includes cough, dyspnea, hemoptysis, wheezing or asthma-like symptoms, recurrent pneumonia, lobar collapse, atelectasis, air trapping, postobstructive infections, and bronchiectasis as a complication of bronchial obstruction . PaganinRadiological features are variable and not diagnostic for endobronchial papilloma. Features may include a shadow, infiltrates, mass, pulmonary nodule or postobstructive atelectasis, and bronchiectasis.In conclusion, solitary endobronchial papilloma is a rare tumor. Clinical and radiological presentations are not diagnostic and cannot differentiate the papilloma from other tumors. A biopsy is required to make the diagnosis and malignant transformation, though it is rare, is a potential complication which should be considered during the management of the patient.


An enterogenous cyst is a rare entity categorized as an intestinal cyst. In most cases, enterogenous cysts are seen in the mediastinum, peritoneal cavity, spinal canal, subarachnoid space, and cerebral ventricle.A 14-year-old Asian (Japanese) boy reported feeling pain in his left groin, and a mass was found. We did not perform orchiectomy because intraoperative frozen sections showed no malignant findings. On histological examination the resected specimens contained columnar epithelium surrounded by smooth muscle. Based on these findings, an enterogenous cyst was diagnosed. Few cases of enterogenous cysts of the testis have been described.We encountered a case of an enterogenous cyst of a pediatric testis. An enterogenous cyst is a rare entity categorized as an intestinal cyst. On histological examination, enterogenous cysts have intestinal epithelium and smooth muscle –3. In moA 14-year-old Asian (Japanese) boy was referred to our department for further examination of a left testicular tumor, which had been detected after he presented with sudden pain in his groin 5 days earlier. His left testis showed no swelling and no rubefaction. Four days after the initial visit, he underwent a surgical operation on his left testicular tumor. He had no remarkable medical history including past medical, social, family, and environmental history, except for mild mental retardation.Ultrasonography (US) showed irregular contrast in his testicular tumor. The laboratory data were nearly within normal limits, including alpha-fetoprotein (AFP) and human chorionic gonadotropin (hCG): 1.0 ng/mL and <0.1 mIU/mL, respectively. His lactate dehydrogenase (LDH) was slightly elevated at 226 U/L. His laboratory data for complete blood cell counts and serum examination were as follows: white blood cell (WBC) 5590/μL, red blood cell (RBC) 483/μL, platelets (Plt) 28.8/μL, aspartate aminotransferase (AST) 17 IU/L, alanine aminotransferase (ALT) 9 IU/L, and creatinine 0.43 mg/dL. A urine analysis showed no remarkable findings. Physical and neurological examinations showed normal findings, and his blood pressure was 110/62 mmHg at the time of admission. Because of these findings, we could not rule out teratoma or other testicular malignancies, so a rapid pathological examination during surgery was performed. He was found to be free from recurrence 12 months after surgery using US.We first performed US to detect the location of the tumor and cut the testicular tunica, after which an additional 4-cm incision was made on his central scrotum. Although the tumor margin was not detected, we extracted the testicular tumor with the surrounding normal testis. The tumor had multiple capsuled cysts containing liquid. An intraoperative rapid pathological diagnosis showed no malignant findings, so we did not perform orchiectomy. On histological examination, the tumor showed spermatogenesis and consisted of intestinal tissue.The resected specimen was 12×11×10 mm in size, including the tumor and marginal tissue Fig. .Fig. 1ReAn enterogenous cyst is an intestinal cyst with muscle layer cells and cysts covered by intestinal cysts. Several hypotheses regarding the development of this tumor have been proposed. One hypothesis suggests that the spine and endoderm were differentiated insufficiently during the embryonal period, because enterogenous cysts are usually seen in the central nervous system or as spinal abnormalities . Anotheret al. in 2006 [On histological examination, enterogenous cysts contain intestinal epithelium surrounded by smooth muscle, mimicking intestinal mucosa –3. In mo in 2006 . Since tThe differential diagnoses of enterogenous cyst include teratoma, metastasis from a mucinous tumor, and mucinous cystadenoma. Most pediatric testicular tumors are mature teratomas, which contain three elements of germ layers such as epithelia, cartilage, muscle, nerve, as well as other types of tissue. A teratoma is defined as a benign tumor and is usually nucleated; the testis can be preserved in some cases, depending on the intraoperative pathological diagnosis .Metastatic mucinous cyst of the testis is more frequently observed than mucinous tumors with a testicular origin . The colet al. also reported a case of enterogenous cyst removal with successful preservation of the testes and no recurrence for 1 year [This pediatric enterogenous cyst arose solely from the testis with cylindrical epithelium and a smooth muscle layer. Because we observed no findings suggestive of malignancy, the tumor was successfully removed and the testes preserved. Mondaini Here we described a case of an enterogenous cyst of the testis in a pediatric patient with successful preservation of the testis.


Clozapine is known to cause fecal impaction and ileus with resultant colonic necrosis due to compression of colonic mucosa. There are rare reports of clozapine causing necrosis of other portions of the gastrointestinal tract unrelated to constipation. We describe a case of acute necrosis of the upper gastrointestinal tract and small bowel to due to clozapine and quetiapine.A 66-year-old white man with a past medical history of schizophrenia, maintained on clozapine and quetiapine, presented with hypoxic respiratory failure caused by aspiration of feculent emesis due to impacted stool throughout his colon. His constipation resolved with discontinuation of clozapine and quetiapine, and his clinical condition improved. These medicines were restarted after 2 weeks, resulting in acute gastrointestinal necrosis from the mid esophagus through his entire small bowel. He died due to septic shock with Gram-negative rod bacteremia.Clozapine may cause acute gastrointestinal necrosis. Constipation is a common side effect of antipsychotic medicines, and is attributed to their anticholinergic properties . ConstipEscherichia coli and Klebsiella oxytoca. Surgical consultation was obtained, but no surgical intervention was possible given the widespread gastrointestinal involvement and his moribund state. He died within 24 hours of developing symptoms. An autopsy was declined.A 66-year-old white man with a history of schizophrenia presented to our emergency department with acute altered mental status and feculent emesis. His abdomen was distended but not tender. He was found to be in severe respiratory distress requiring endotracheal intubation, and shock requiring vasopressor infusion. Computed tomography (CT) imaging revealed acute respiratory distress syndrome (ARDS) due to aspiration as well as impacted stool throughout his colon. Home antipsychotic medicines, clozapine and quetiapine, were held, and after manual disimpaction and enemas he had copious bowel movements and resolution of abdominal distension. His shock and ARDS gradually improved. Vasopressors were discontinued on day 3, and he remained normotensive. In-patient medicines were carbamazepine, lansoprazole, phenytoin, polyethylene glycol, senna, and subcutaneous heparin prophylaxis. Clozapine 100 mg every 12 hours was restarted on day 11, and a single dose of quetiapine 400 mg was given on day 16, both at home doses. Six hours after quetiapine was restarted, he was noted to have recurrence of abdominal distension and new onset of copious foul-smelling hematemesis. An abdominal X-ray showed diffuse small bowel dilation Fig. , with coOur patient’s initial presentation with aspiration of feculent emesis due to impacted stool throughout the colon is within the known spectrum of life-threatening clozapine-induced colonic hypomotility. Fecal impaction resolved with discontinuation of clozapine and quetiapine.Acute gastrointestinal necrosis developed 5 days after restarting clozapine and 6 hours after restarting quetiapine. EGD and abdominal X-ray were consistent with necrosis from the mid esophagus through the entire small bowel. His new onset foul smelling hematemesis, abdominal distension, and polymicrobial Gram-negative rod bacteremia clinically matched imaging findings, and he died within 24 hours of developing symptoms. Given the temporal association with his clinical deterioration, we believe his acute gastrointestinal necrosis was caused by re-initiating clozapine and quetiapine.Other causes of gastrointestinal necrosis were considered. It is unlikely that proximal splanchnic arterial occlusion from embolic or other source occurred, as necrosis was present in multiple vascular territories. Our patient had remained off vasopressors with normal blood pressure for 13 days, making a shock state an unlikely cause. His other medications did not include vasoactive drugs known to cause mesenteric ischemia such as alpha agonists, digoxin, or ergotamines .et al. in 2013 reported a case of a 47-year-old man who developed acute necrosis of small bowel and colon 6 days after initiating clozapine [There are few reports of clozapine causing acute necrosis of portions of the gastrointestinal tract other than the colon. Yu 3 (5-HT3) serotonin receptor [3 inhibiting drug used to treat irritable bowel syndrome with diarrhea, was withdrawn from the market due to causing ischemic colitis unrelated to constipation, and was subsequently reintroduced with a black box warning [A possible mechanism of clozapine-induced acute gastrointestinal necrosis is antiserotonergic effect. Clozapine is the only antipsychotic medicine known to be a competitive inhibitor of the 5-hydroxytryptaminereceptor . Alosetr warning , 10.Clozapine is known to cause fecal impaction and ileus, compressing the colonic mucosa and leading to colonic necrosis. Two recent cases have described clozapine-induced acute necrosis of the small bowel and upper gastrointestinal tract, without constipation. We present a third such case. Further monitoring is needed to confirm this suspected life-threatening side effect of clozapine.


A 19-year-old female presented to ENT OPD of Dhulikhel Hospital, Kathmandu University Hospital, with history of foreign body sensation in throat for 2 weeks and mass in left tonsil for 1 week. There is no history of difficulty swallowing or recurrent throat infection. Physical examination revealed a pedunculated mass arising from upper pole of left tonsil. Bilateral tonsillectomy was done under general anaesthesia. Grossly, 2.5 × 1.5 × 1 cm polypoidal mass, soft in consistency, was found to be attached to left tonsil. Histopathology report was consistent with lymphangiomatous polyp of tonsil. Postoperative period was uneventful and she was normal during her follow-up at 1 month with bilateral healthy tonsillar fossa. Around 90% of all lymphatic malformations arise in head and neck , 2. MostBenign tumours or tumour-like lesions of palatine tonsil are more common than malignant ones. Squamous papilloma accounts for majority of benign lesions, whereas vascular tumours are rarely reported . TonsillA 19-year-old female presented with history of foreign body sensation in throat for 2 weeks and mass in the left tonsillar region for 1 week. There is no history of sore throat or difficulty swallowing. There was no history of any chronic illness or similar illness in her family members.Examination of oropharynx revealed a pedunculated mass arising from upper pole of left palatine tonsil. There was no cervical lymphadenopathy. Blood parameters were within normal level.Bilateral tonsillectomy was done under general anaesthesia. Grossly, a 2.5 × 1.5 × 1 cm, soft to firm, polypoidal mass was found attached to the upper pole of left tonsil with a slender stalk .The cut surface showed reddish areas. Histologically, the tissue was lined by hyperkeratotic, parakeratotic stratified squamous epithelium with underlying parenchyma showing numerous lymphatic channels lined by endothelium, some with luminal eosinophilic secretions. Intervening stroma was densely infiltrated by lymphocytes and plasma cells Figures  and 3.Postoperative period has been uneventful for 1 month of follow-up.Benign tumours are more common than malignant in case of palatine tonsils. Squamous papilloma accounts for the majority of benign lesions, whereas vascular tumours are rarely reported . The tonThe true incidence of these types of tumour in the general population is currently unknown but many researchers suggest that it is most likely higher than expected , 7. ThisPathogenesis of tonsillar lymphangiomatous polyp remains unclear. Chronic inflammation and associated obstruction of lymphatic channels were previously suggested as a possible mechanism, but this is unlikely given that chronic tonsillitis occurs much more commonly than lymphangiomatous polyp , 7.Other mechanism like isolated hamartomatous proliferation is most likely responsible for the aetiology of these tumours. This is further supported by the haphazard proliferation of elements that are normally found in the tonsillar tissue , 8.Lymphangiomatous polyps of the palatine tonsils have not been linked to other lymphatic lesions in the head and neck region nor do they seem to be part of generalized lymphatic malformation, which has been reported in the lower gastrointestinal tract , 8.Mostly the management is tonsillectomy but some literatures have shown comparable result with excisional biopsy .Lymphangiomatous polyps are uncommon benign lesion appearing in palatine tonsils. Often these are mistaken to be malignant growth causing unnecessary stress to the patient and their family. Tonsillectomy is the curative treatment in these conditions with no reported recurrence.


An 18-year-old male presented to the emergency department of our hospital with complaints of bilateral leg numbness and weakness since about a month. Magnetic resonance imaging of the spine revealed an extramedullary extradural mass at the T9-T11 level causing marked spinal cord compression. Emergent surgery was performed. An epidural mass was seen after laminectomy and partially removed. Microscopic examination showed a diffuse infiltration of small- to medium-sized lymphoid cells with irregular nuclei, dispersed nuclear chromatin, prominent nucleoli, and scant cytoplasm in adipose tissue . ImmunohThe spinal cord is an extremely rare initial site of involvement for B-cell lymphoblastic lymphoma. To our knowledge, there are only 3 reported cases in the English literature  1,2,3].,2,3.1,2,Lymphoblastic lymphoma should be included in the differential diagnosis of spinal masses.


Synovial chondromatosis (SCh) can undergo malignant transformation. Pathologic diagnosis of secondary synovial chondrosarcoma (SChS) is challenging and misdiagnosis may result in over- or undertreatment.A systematic review revealed 48 cases of SChS published in 27 reports since 1957. Data was collected to identify findings indicative of SChS and outcomes of treatment.At median follow-up of 18 months, patients were reported as alive (10%), alive without disease (22%), alive with disease (15%), dead of disease (19%), dead of pulmonary embolism (4%), and unknown (29%). Initial diagnosis of SChS  was after biopsy in 58%, local resection in 29%, and amputation in 13%. Seventy-four percent of patients underwent 1.8 (mean) resections. Patients treated prior to 1992 were managed with amputation in 79% of cases compared to 48% after 1992. Symptoms were present for 72 mos prior to diagnosis of SChS.Synovial chondrosarcoma demonstrated symptom progression over several months (82%), rapid recurrence after complete resection (30%), and medullary canal invasion (43%). The SChS tumor dimensions were seldom quantified.Malignant degeneration of synovial chondromatosis is rare but can necessitate morbid surgery or result in death. Pathognomonic signs for SChS including intramedullary infiltration are present in the minority of cases. Progression of symptoms, quick local recurrence, and muscle infiltration are more suggestive of SChS. Periarticular cortical erosion, extra-capsular extension, and metaplastic chondroid features are non-specific. Although poorly documented for SChS, tumor size is a strong indicator of malignancy.Biopsy and partial resection are prone to diagnostic error. Surgical decisions are frequently based on size and clinical appearance and may be in conflict with pathologic diagnosis. Synovial chondromatosis (SCh) is a metaplastic chondroid proliferation within articular joints and, less commonly, in bursa or tendon sheath -3. The vIt is well recognized that most cartilaginous conditions such as SCh and multiple enchondromatosis represent low-grade or benign cartilage tumors on histologic analysis. Patients with SChS are often underdiagnosed and undergo multiple resections before SChS is considered as a diagnosis and adequate treatment is performed. The histologic challenges of diagnosing chondrosarcoma are well-recognized for low grade cartilage tumors .Malignant transformation of SCh has previously been reported in the literature through numerous case reports and series. The purpose of this systematic review was to 1) identify clinical, radiographic and histopathologic findings indicative of SChS in the setting of SCh, 2) determine the outcomes of SChS, and 3) formulate a treatment approach for SChS.st, 2014 (Table 1). The search strategy consisted of terms synovial, chondromatosis, osteochondromatosis, malignant, chondrosarcoma combined with the Boolean operator AND Fig. (1). Case series of benign SCh were excluded. All cases of malignant transformation of SCh were included. Three non-English language articles were translated and reviewed as well. Forty-eight patients in 28 level IV evidence articles, as defined by Sackett [vs. intramedullary), primary site of disease, presenting clinical symptoms, prior treatment for SCh, episodes of local recurrence, pathologic/histologic grade, clinical signs of transformation, method of SChS diagnosis, size of SChS lesion, treatment for SChS, metastatic spread, disease status and length of follow-up. Increasing pain over 6-12 weeks was interpreted as clinical progression. Early recurrence of SCh was defined as recurrence less than 12 months after resection.A literature search on malignant transformation of SCh was performed for all studies indexed on PubMed and for cited references in aforementioned studies up to June 1 Sackett , from 19 Sackett , 14-32 .1).An Excel worksheet  was created to compile and juxtapose all relevant data for comparison. The Fisher’s exact test was used to compare rates of amputation. Characteristics specific for neither SCh nor SChS were also noted , hip (33%),, ankle (6%), shoulder (4%), and other/unknown (6%). At latest follow-up , outcomes were reported as alive (10%), alive without disease (22%), alive with disease (15%), dead of disease (19%), dead of pulmonary embolism (4%), and unknown (29%). Further specification regarding death from disease or pulmonary embolism was not available. The rate of local recurrence for SCh that underwent malignant transformation was very high. Seventy-four percent of patients underwent multiple resections with a mean of 1.8 resections  and 61% eventually required amputation. Prior to 1992, the incidence of amputation was 79% (15 of 19) and after 1992, it was 48% (13 of 27) (p = 0.0017). Symptoms of pain and swelling were present for a mean of 72 months (3 to 462) prior to diagnosis of SChS. Initial diagnosis of SChS  was after biopsy in 58%, local resection in 29%, and amputation in 13%.The following attributes were documented by the reporting authors in the following proportion of cases for SChS: rapid progression of pain (82%), invasion of medullary bone on imaging or histology (43%), rapid recurrence (<12 mos) after complete resection of SCh (30%), or infiltration of adjacent muscle (12%).A soft tissue mass, joint effusion, joint space destruction, osseous erosion, local recurrence, and pathologic features of low-grade chondrosarcoma were universally reported as not specific for either SCh or SChS , 26, 31.2). Concerning features include rapid clinical progression of pain, local recurrence within 12 mos after resection, soft tissue infiltration of tumor, and large size [3). Similar to the diagnostic and grading challenges of other chondroid malignancies, accurate assessment of SChS requires consideration of clinical, radiographic and histologic findings [Differentiating SChS from benign SCh is a significant histologic and clinical challenge at presentation and following resection. Based on this systematic review of reported cases, objective diagnostic findings for SChS include medullary bone invasion on imaging or histology and pathologic findings consistent with grade II or III chondrosarcoma , 33, 34 findings .Distinguishing benign from malignant chondroid tumors is notoriously difficult on pathology alone . A benig2), making surgical decisions about the extent of resection can be difficult.Benign cartilaginous conditions such as Ollier’s disease and SCh are recognized to have a more aggressive histologic appearance than intraosseous cartilage lesions or enchondromas , 26. ForLimitations of this study include the availability of only Level IV evidence and multiple studies where tumor size is not quantified and long-term patient survival is not documented. Because of the rarity of this condition, extrapolations need to be made from the existing literature that may not be supported by large cohort or controlled studies. The treatment for SChS has evolved to include more limb preservation options rather than amputation, but because sample size is relatively limited and the variability between characteristics of each case, it is difficult to draw broad conclusions or recommendations regarding limb preservation.The standard initial treatment for symptomatic SCh is joint-preserving arthroscopic or open resection , 29. Inc Depending on the extent of disease, total joint arthroplasty may be necessary. For patients with large tumors >5-10 cm) and local invasion, open biopsy and incomplete resection are likely to result in a benign or low-grade cartilaginous neoplasm diagnosis  cm and l, 17, 30.If there is local recurrence of a low-grade SChS or the presence of intermediate- or high-grade SChS on pathologic evaluation, wide resection is necessary and in many cases, amputation will be required to achieve adequate margins. Surgical decisions regarding large or recurrent tumors should be made on the basis of tumor size and imaging appearance, even with an apparent histologic diagnosis of “low-grade” chondrosarcoma.


Even though they are quite uncommon, puerperal genital haematomas can be associated with serious maternal morbidity. Key findings are significant perineal pain and, depending on the location, visible swelling. However, attention can be drawn to its progression by the rare occurrence of persistent painful “bearing down” efforts, even after the successful delivery of the baby. The final size of this haematoma and the rare presentation make it truly uncommon. The primary goals of treatment include the prevention of further blood loss, minimizing tissue damage, relieving pain, and reducing the risk of infection. Management is generally conservative for small collections, but surgery is indispensable when they acutely expand in size or are large with worsening symptoms.  Vulvovaginal haematoma (VVH) occurring postpartum is a relatively uncommon complication of labour but can be a cause of serious morbidity and even maternal death . This isClinically significant haematoma requiring surgical intervention occurs in about 1 in 1000 vaginal deliveries, while estimates from case series report incidences of 1 : 500 to 1 : 12,500 vaginal births .Vulvovaginal haematomas classically present a few hours after delivery. The patient is usually restless, complaining of pelvic pain, inability to pass urine or rectal tenesmus. The rare presentation with the urge to “bear down” may confuse the accoucheur who is not conversant with this pathology leading to the possibility of extensive tissue damage and postpartum collapse from hypovolemic shock before an accurate diagnosis is made .We therefore present the case of a nullipara with puerperal VVH that mimicked the “bearing down” efforts of the second stage of labour.A 26-year-old now Para 1 was referred from a peripheral hospital to the Teaching Hospital because of persistent “bearing down” efforts after a spontaneous vaginal delivery of a live male neonate with birth weight of 3400 gm six (6) hours prior to presentation. The labour had lasted about 9 hours. The birth attendant, having examined the patient again and excluded the possibility of an undiagnosed twin, decided to refer her for expert management.Examination on admission revealed a young lady who was pale, dehydrated, and intermittently straining in response to “the irresistible urge to push.” Her pulse rate was 102 beats/min, regular and of moderate volume with a blood pressure of 120/70 mmHg. The uterus was about 20-week pregnancy size and was well contracted. Pelvic examination revealed a blood-smeared vulva with a tender swelling involving the right labia minus and majus measuring about 4 cm by 4 cm.A diagnosis of puerperal genital haematoma was made. The plan was to institute conservative management with vaginal pack and analgesics. Intravenous access was established and blood sample was obtained for packed cell volume (PCV) (which was 22%) and grouping and cross-matching of three units of whole blood. The limits of the swelling were ink-marked and observed for one hour to confirm if there would be an enlargement. Progressive expansion of the VVH occurred, necessitating surgical intervention. The intraoperative findings included a huge 12 cm by 10 cm right vulvovaginal haematoma completely occluding the vaginal cavity and stretching the overlying perineal skin (Figures There was no significant bleeding from the vagina. Incision and evacuation of the haematoma and exploration of the cavity were done and 1500 mls of clotted blood was evacuated from the cavity. Haemostasis was achieved with “figure-of-eight” sutures. The space was obliterated with deep sutures and the overlying skin approximated carefully, avoiding injury to the urethra. The vagina was packed with gauze to further assist with haemostasis . She had after vaginal delivery [Puerperal VVH are rare and have not been extensively reported. They are usually unilateral and in most cases develop insidiously, attention being drawn to them when the woman collapses in shock, groans in pain, or complains of “bearing down” paindelivery . This isdelivery , which cdelivery . Since tdelivery . The casManagement options are either conservative or surgical depending on the size and rate of progression of the haematoma. Conservative management was initially instituted in the patient presented because the swelling was less than 5 cm. Other conservative measures employed when the haematoma is <5 cm and static/not rapidly increasing in size include ice packs, pressure dressing, bladder drainage, and analgesics . The visFollowing evidence of progression or in large haematomas, surgical management should be instituted with the incision made as medially as possible to minimize visible scarring . The preContinuing bleeding should be excluded by close monitoring of the operation site, evaluation of the vital signs, and urinary output. When it occurs, the site should be reexplored and identified bleeders ligated. Sometimes, ligation of the internal iliac artery or even hysterectomy may be necessary  or use oTo prevent infection, broad-spectrum antibiotics should be given. Good analgesia and close observation are important postoperatively. Prompt resolution of haematoma will improve outcome and result in reduced scarring, postpartum pain, and dyspareunia .


Salivary gland tumors (SGT) constitute a small proportion of primary respiratory system neoplasms. Benign SGT comprises pleomorphic adenoma and is exceedingly rare in the lungs. We hereby present a rare case of a benign primary pleomorphic adenoma of the lung. The histological pattern of the tumor was indistinguishable from the head and neck SGT counterparts and showed biphasic morphology. Malignant features were not observed and a metastatic SGT was ruled out. Although little is known about the clinical progression of these rare tumors, surgical resection and interval surveillance remains the treatment of choice. Pleomorphic adenomas (PA) are the most common type of salivary gland tumors. Histologically, they are characterized by mixed epithelial, myoepithelial and stromal cell components. PA can occasionally undergo a malignant transformation to a carcinoma ex-pleomorphic adenoma and give rise to metastasis. Rarely, they can arise in the tracheobronchial system as primary neoplasm . PulmonaA 63-year-old African American female was admitted to our hospital with nausea, vomiting and abdominal pain of few days duration. She was diagnosed with diabetic ketoacidosis and was started on an insulin drip. Her continuous abdominal pain warranted a computed tomography (CT) of the abdomen and pelvis which revealed an incidental finding of a 3.3 x 2.7cm left lower lobe pulmonary mass, potentially representing a malignant neoplasm. In retrospect, our patient denied any a cough, shortness of breath, hemoptysis or weight loss. A subsequent chest CT scan showed a soft tissue attenuating mass in the medial left lower lobe measuring 3.5 x 4.1 x 3.1cm  classification of lung tumors includes three malignant salivary gland-type tumors: adenoid cystic carcinoma, mucoepidermoid carcinoma, and epithelial-myoepithelial carcinoma. The two benign salivary gland-type tumors are the mucous gland adenomas and pleomorphic adenomas . The latMacroscopically, PAs are polypoid tumours; they have a rubbery consistency, are often encapsulated and can have a myxoid or cartilaginous appearance. Histologically, they are described as mixed tumors and are characterized by biphasic cellular glandular components, mainly epithelial ducts and/or tubules which are embedded in a chondromyxoid stroma .Most PAs are benign and rarely undergo malignant transformation. Features of malignant variants include cellular atypia, frequent mitotic figures, and necrosis. Our patient’s PA did not have any malignant features. A physical examination of the salivary glands was conducted in our patient to rule out a metastatic origin of the lung PA.The clinical presentation of PAs varies depending on the location of the tumour. Endobronchial tumours which arise from the epithelium of sub-mucosal bronchial glands are usually associated with cough, hemoptysis and shortness of breath. Peripheral tumours can sometimes present with fever, weight loss and pleural effusions. Nonetheless, the majority of cases are asymptomatic . In factLittle is known about the best treatment options for these exceedingly rare lung neoplasms; however surgical resection remains the most common approach as these tumours have the potential of malignant transformation. Our patient is scheduled for a follow-up visit to discuss surgical resection and interval surveillance. In conclusion, primary benign pleomorphic adenomas of the lung are rare tumors of which the majority are asymptomatic and are incidentally discovered. In evaluating patients with pulmonary PAs, metastatic lesions from mixed tumors of the salivary glands and other biphasic pulmonary neoplasms such as blastoma, hamartochondroma, and carcinosarcoma should be considered in the differential diagnosis. Surgical resection is the treatment of choice for PA. Long-term surveillance is warranted due to the concerns for malignant transformation and recurrence of the disease.


Lipoma is a very common soft tissue neoplasm, but only infrequently found in the oral region. Intramuscular lipoma (IML) is a relatively common variant of lipoma. The most common site for IML is the large muscles of the extremities, and it is quite rare in the oral cavity. A case of IML affecting the floor of the mouth/tongue of a 42-year-old female is described. The patient presented with a 4 cm diameter yellow mass in the right side of the sublingual area. Axial and coronal magnetic resonance imaging demonstrated its infiltrating nature that can be distinguished from the ordinary well-encapsulated lesion. The lesion was excised with adequate surgical margins. Histopathologically, the lesion was composed of mature adipose tissue that infiltrated the muscle in a diffuse manner. No lipoblasts, atypical cells, or high mitotic index were found. There was no evidence of recurrence two years postoperatively. Review of the literature yielded that IML occurring in the sublingual region is extremely rare. Lipomas are benign neoplasm composed of mature adipocytes. They are usually sharply circumscribed and possess some peripheral fibrous demarcation (pseudocapsule).They are the most common tumor of the trunk and extremities. About 15–20% of cases involve the head and neck regions, while 1–4% affect the oral cavity [Oral lipomas (OLs) occur most commonly in the parotid region followed by the buccal mucosa, tongue, floor of the mouth, and palate. Histologically, lipomas are classified as classic lipoma, fibrolipoma, intramuscular lipoma (IML), angiolipoma, and spindle cell lipoma. OL usually presents as a slow-growing painless mass that rarely exceeds 20 mm in diameter . The exaA 42-year-old female, generally healthy except for gastroesophageal reflux treated with lansoprazole 30 mg/day, presented with a 4.0 cm painless swelling of the right sublingual area that was growing slowly over three years. Clinical examination revealed a soft tissue mass, covered by the normal-appearing mucosa, with no ulceration . No cervUnder general anesthesia, via an incision in the right floor of the mouth, the tumor was removed, together with a thin margin of normal muscle tissue, in the inferior aspect and some normal oral mucosa in the superior aspect.The surgical specimen consisted of a soft yellow-white mass with smooth external surfaces and a uniform solid yellow cut surface.Microscopic examination revealed mature adipose tissue with striated muscle fibers interspersed within . There wThe postoperative course was uneventful. Two years after surgery, the patient has no evidence of recurrence .Oral lipomas (OLs) commonly present as a soft, mobile, lobulated, yellow mass. It is usually observed among adults, between the age of 40 and 60 years, with no gender preference. The most common site for OL is the buccal mucosa, a region that is normally rich with adipose tissue, followed by the tongue, lips, floor of the mouth, palate, and gingiva .OL consists of mature adipocytes organized into lobules that are separated by septa of fibrous tissue. Although the OL is morphologically indistinguishable from normal fat, it differs from normal body fat, in that lipids contained in the OL are metabolically inactive .Oral IML seems to be larger in size and displays an infiltrative pattern of growth, as compared with the classic OL. It is characterized by a much deeper localization, slow-growing, painless mass that often can cause swelling and deformity. Its consistency is often rubbery. In the rare occasion, when the infiltration is very extensive, it may cause local muscle dysfunction or sensory disturbance, due to local pressure on a branch of the trigeminal nerve .Oral IMLs are very rare and are described most commonly in the tongue , 7, 8.Local recurrence rate for the IML is reported as high as 63% . This hiDue to the infiltrative-like pattern of growth of IML, it may be difficult to distinguish IML from a well-differentiated liposarcoma, just by means of diagnostic imaging modalities (ultrasound (US), computerized tomography (CT), or MRI). Hence, the only way to obtain a diagnosis is by an incisional or excisional biopsy. In this case, the mass was completely resected without a biopsy. The authors felt that the lesion could be completely excised. In spite of the tumor appearing to invade the muscles, the degree of invasion based on palpation was limited, and the authors felt that the lesion involved some muscle fibers that could be completely excised without compromising the overall structure or function of the involved muscles. Alternatively obtaining an incisional or trucut biopsy before complete resection could be considered reasonable as well.The mass of IML may present high-signal intensity with some strands of low-signal intensity, which may refer to the remnants of the lingual muscle fibers. CT appearance of a well-differentiated liposarcoma may be very similar to the remnants of infiltrated soft tissue . DespiteOne more infiltrative lipoma to consider is the spindle cell lipoma which may occur occasionally in the tongue. Although it rarely infiltrates adjacent tissue, there are variants with predominance of the adipocyte component and unusual forms of multifocal disease  which maOral lipoma is quite rare. The variant of intramuscular (infiltrating) lipoma should be kept in mind and should be differentiated microscopically from liposarcoma. Complete surgical excision with minimal morbidity is recommended.


Cardiogenic shock can occur due to compression of the four pulmonary veins and the left atrium by a mediastinal tumor. Steroid infusion can be a temporary alternative therapy before obtaining a definite diagnosis and performing an intervention with stents to dilate the pulmonary veins. An 81‐year‐old man who had undergone left upper lobectomy due to squamous cell carcinoma 7 years ago was referred to our emergency department because of severe dyspnea. Physical examination showed signs of cardiogenic shock and a chest X‐ray revealed lung congestion. An echocardiographic evaluation revealed decreased left atrial cavity size due to a compressive tumor, and ECG‐gated cardiac computed tomography (CT) was subsequently performed that revealed a large tumor (40 mm minor axis diameter) in the posterior mediastinum and compression of the left atrium and pulmonary veins (PVs) (Fig. Single PV stenosis due to a compressive or invasive tumor was previously reported in a few case reports KY and FF: equally contributed to the conception of the work. KY: wrote the first draft of the manuscript. All authors: revised it critically and approved the final version to be published.None declared.


We present a case of a 50-year-old woman with acute dacryocystitis that was complicated by posterior rupture of the lacrimal sac causing an orbital cellulitis with subsequent visual acuity of no light perception. Upon presentation, she was immediately started on broad-spectrum antibiotics and underwent surgical incision and drainage of the lacrimal sac abscess but never regained vision. There are 4 cases in the literature of permanent severe vision loss from acute dacryocystitis. Prompt diagnosis and close monitoring of acute dacryocystitis are therefore essential to prevent extension into the orbit and possible optic nerve compromise.  Dacryocystitis is an infection of the lacrimal sac secondary to obstruction of the nasolacrimal duct. Acute dacryocystitis typically causes preseptal cellulitis but is rarely associated with orbital cellulitis. Typically orbital cellulitis responds to systemic antibiotic therapy and surgical drainage without permanent optic nerve compromise.A 50-year-old Hispanic woman with no significant past medical history presented to our emergency department with a 1-week history of left eye pain and 1-day history of decreased vision in her left eye. She had visited another hospital 2 days prior to presentation, where she was diagnosed with acute dacryocystitis and preseptal cellulitis, based on her clinical exam and orbital imaging . She wasOn evaluation, visual acuity was 20/25 OD and no light perception OS. The left pupil was 4 mm and nonreactive with a left relative afferent pupillary defect. She had left proptosis with complete ophthalmoplegia. She also had left upper and lower eyelid edema and erythema and significant resistance to retropulsion of the left globe . IntraocComputed tomography (CT) of the orbits with contrast showed an enlarged left lacrimal sac, a large complex fluid collection in the inferomedial left orbit, and severe tenting of the posterior globe . There wThe patient was started promptly on intravenous vancomycin and piperacillin-tazobactam and taken emergently to the operating room. Significant purulent material was drained from the lacrimal sac, which was noted to have ruptured posteriorly into the orbit. After drainage of the lacrimal sac and medial aspect of the orbit, no additional purulence was noted. A lateral canthotomy and inferior cantholysis was performed.Postoperatively, the patient had significant reduction in proptosis, resistance to retropulsion, eyelid edema, and erythema. As there was no improvement in motility after admission day 4, intravenous methylprednisolone was administered with subsequent significant improvement in motility. Unfortunately, her visual acuity remained at no light perception. Staphylococcus epidermidis. She was discharged on postoperative day 6 with a peripherally inserted central catheter and a 2-week course of intravenous ceftaroline and oral metronidazole. Three months after discharge, she underwent uncomplicated left external dacryocystorhinostomy and lateral canthoplasty. Crawford tubes were removed 12 weeks postoperatively. At 6 months of follow-up, she was without tearing.Intraoperative cultures were positive forAcute dacryocystitis can commonly cause preseptal cellulitis but is rarely associated with orbital cellulitis. In such cases, associated orbital cellulitis typically does not cause optic nerve compromise, and patients do well after systemic antibiotics and surgical treatment –5. Staphylococcus aureus [ Streptococcus viridans, and Streptococcus intermedius. The fourth case was a 65-year-old woman with a history of recurrent dacryocystitis and nasolacrimal duct obstruction who presented with visual acuity of no light perception and a posteromedial orbital abscess [There are only 4 cases of acute dacryocystitis leading to severe, permanent vision loss reported in the literature , 6, 7. I abscess . She undA prior episode of dacryocystitis is accepted as risk factor for orbital spread of the infection. When examining previously reported cases, Kikkawa et al. found an incidence of prior dacryocystitis in 40% of patients presenting with an acute episode and posterior extension . AlthougKikkawa et al. emphasize the strong anatomical barriers to posterior infectious spread from the lacrimal sac. Although the deep heads of the preseptal and pretarsal orbicularis muscles function as the primary barrier, the insertions of the orbital septum and medial canthal ligaments at the lacrimal crest provide an additional impediment to posterior extension. They note, however, that a breach of these structures provides direct access into the intraconal space, making abscess formation immediately sight-threatening and requiring urgent intervention .Our case underscores the need for prompt diagnosis and close monitoring of acute dacryocystitis. If an abscess is present, we recommend incision and drainage in an attempt to prevent rupture of the lacrimal sac and posterior extension into the orbit, with dacryocystorhinostomy providing the definitive treatment. If orbital involvement does develop, optic nerve compromise may progress quickly and be irreversible. As seen in our case, the patient had no light perception from severe orbital infection with acute dacryocystitis. Despite aggressive treatment at the time of presentation to our institution, she did not recover vision in the affected eye.


Duodenal atresia is rarely associated with situs inversus abdominus. We report a case of duodenal atresia associated with small bowel atresia of apple peel type and situs inversus abdominus.  A 3-kg baby boy was born by spontaneous vaginal delivery at 34 weeks of gestation to a 37-year-old mother, G6P4A2, whose pregnancy was complicated by diabetes mellitus and toxemia, treated respectively by insulin and anti-hypertensive drugs prior to delivery. Prenatal ultrasonography reports were normal. At birth, the baby was admitted to the neonatal intensive care unit for respiratory distress related to an early neonatal infection. On the second day of life, bilious vomiting and meconium emission delay were noticed. An abdominal X-ray revealed double bubble sign with no air distally and the stomach was localized in the right side of the abdomen. An upper contrast study confirmed the diagnosis of complete duodenal obstruction and right sided stomach . Abdominal ultrasonography revealed liver located in the left upper quadrant of the abdomen and a spleen in the right one. Surgical exploration showed atresia of the distal portion of duodenum and of the proximal jejunum, multiple atresia of the remaining small bowel wrapping around its blood supply in a spiral manner resembling an apple peel, mesenteric defect, agenesis of the superior mesenteric artery and abdominal situs inversus . Nothing could be done peroperatively. The baby was kept perfused on a central venous catheter and died after 32 days. Duodenal atresia is a relatively common anomaly with an estimated incidence of 1 in 3000 to 1 in 5000 live births [1]. It is also common for duodenal atresia to be associated with other anomalies mainly congenital heart disease and Down's syndrome [1]. Our case had duodenal atresia, multiple atresias including apple peel type, and situs inversus abdominus. The association of these two types of intestinal atresia is extremely rare and only five cases were reported before [2]. Our case is unique as it has additional association with situs inversus abdominus which is not reported earlier. The management of these patients is complex. The difficulty lies in the treatment of apple peel atresias that continues to have a serious prognosis with significant morbidity and mortality [3]. Our baby was considered inoperable because of the extent of multiple intestinal atresias that make the attempt of multiple primary anastomoses difficult. Rich et al [3] reported a successfully treated apple peel syndrome with a total of six primary small bowel anastomoses. Other procedures were described to treat multiple intestinal atresias: silicone stenting as a method to preserve bowel length and enterostomy [4]. In our case, the decision was taken not to intervene further in consultation with parents based on poor prognosis.Source of Support: NilConflict of Interest: Nil


Dear Editor,A 29-day-old infant was evaluated at our center for a hepatic mass found duringgestation. Ultrasound revealed a heterogeneous lesion which comprised three anechoicareas with hypoechoic debris and a predominantly hyperechoic central region with signsof vascularization on Doppler imaging .ComputeAfter the definitive diagnosis was made, the patient underwent chemotherapy withcisplatin and doxorubicin. Upon completion of the second cycle of chemotherapy, it wasdecided that the patient should undergo liver transplantation, which occurred at 8months and 3 days of age. The surgical specimen showed large masses of an emerald colorwith components of cystic degeneration and central necrosis with adjacent pseudocysts.The histopathological study confirmed a mixed mesenchymal/epithelial hepatoblastomathroughout the neoplastic tissue, with formation of pseudocysts.-4. Hepatoblastoma is themost common primary hepatic malignancy in children, accounting for nearly 80% of allmalignant liver tumors.Hepatoblastoma usually presents as an incidental finding of an asymptomatic abdominalmass in children under 5 years of age. On ultrasound, hepatoblastomas appear aspredominantly solid masses that are hyperechoic relative to the adjacent liver, althoughhypoechoic fibrotic septa can also be seen. Epithelial hepatoblastomas may appearhomogeneous, whereas mixed epithelial/mesenchymal tumors are heterogeneous  and frequently contain echogeniccalcifications with acoustic shadowing and anechoic foci representing hemorrhage ornecrosis. The appearance ofhepatoblastoma on computed tomography is that of a well-defined mass with regularborders that is hypoattenuating in comparison with the adjacent hepatic parenchyma. Thetumor commonly displays diffuse heterogeneous contrast enhancement. Approximately halfof all hepatoblastomas appear lobulated or septated, especially on contrast-enhancedimages,7.Liver tumors are not uncommon in adults,9. Our conclusion is that we should be aware of this rare mostly cysticpresentation of hepatoblastoma, should we encounter cystic hepatic lesions in childrenunder 3 years of age with elevated alpha-fetoprotein levels.In the case presented here, the imaging findings indicated a different entity. Thepredominantly cystic appearance of the tumor was consistent with a cystic liver tumor,namely mesenchymal hamartoma. Mesenchymal hamartomas, which typically occur in childrenunder 2 years of age, present as a large solitary neoplasm with variable amounts ofsolid and cystic components on ultrasound or computed tomography


Primary vascular tumors of the bone are rare; however, EHE is one of the most common primary malignant vascular tumors to occur in bone. A review of the literature found few cases that involved the calcaneus; those cases found that involved the calcaneus were either part of a multifocal or metastatic disease process. Our case This case has clinical follow-up greater than 2 years post-operatively and could be a guide for treatment of a rare disorder with a substantial paucity of literature. Benign primary bone entities are more common than their malignant counterparts. It was estimated that the age adjusted incidence rate for all bone and joint malignancies is 0.9 per 100,000 persons/year [osarcoma –6. The losarcoma .EHE was given its name by Weiss and Enzinger in 1982 to reflect that it is neither completely benign nor fully malignant . More reTo date, there have been no reports of solitary EHE in the calcaneus. There are a few cases that show the involvement of the calcaneus, all of which occurred in a multifocal or metastatic setting , 14–17. and CAMTA1  ruled out proximal metastasis; however, bilateral hilar and mediastinal adenopathy were reported along with increased signal in the stomach and bilateral parotid glands.The patient was taken to the operating room for excision and curettage with argon beam followed by cement filling of the lesion and percutaneous pinning. A biopsy taken during surgery showed the tumor to be histologically the same as the previous biopsy, but now involving the surrounding bone. A thorough oncology and pulmonary workup for the mediastinal adenopathy ruled out metastatic disease, as a biopsy showed a benign anthracotic lymph node with marked histiocytosis. The patient returned to weight bearing activity at 3 months and work after 6 months. CT scan and radiographs of the hindfoot at his 6 month and 3 year follow-up showed well fixed hardware without recurrence Fig. . PET scaThe majority of EHE cases of the bone occur in the lower extremities, followed by the spine . The mosThe second case, reported by Liu et al. , describA case report of a 17-year-old male illustrated another multicentric involvement of the lower extremity . This yoThe final case showed involvement of the calcaneus in a 28-year-old woman who presented with a 3-month history of left heel pain made worse by walking . Radiogrvariable nature of the malignancy. Clinically, these patients presented with an indolent clinical course; however, the rapid progression over a few months from negative radiographic identification to prominent lytic lesions suggests a rapid cellular development of the malignancy.As illustrated in our case and the above case reports, the most frequent clinical presentation of EHE of the bone is local pain –9. SwellMicroscopically, these lesions arise from vessels by expanding the vessel in a centrifugal pattern from the lumen extending outward into soft tissue , 7. It hOften the histological features can deviate from what is typical and show a solid growth pattern, epithelioid change, or spindle cell morphology. These changes can complicate the diagnosis. In 2012, Errani et al.  reportedThere have been no predisposing factors identified . DespiteComplete and wide local excision is the proposed method of treatment –6, 9, 13


The brown tumor or osteitis fibrosacystica is a benign bone lesion that caused by hyperparathyroidism. This complication has been decreased by diagnosis and successful treatment of hyperparathyroidism. The brown tumor of hyperparathyroidism results from a metabolic disorder that affects long bones, ribs, and pelvis. The maxillary involvement is rare. Parathyroidectomyis a good choice for patients with high parathyroid hormone (PTH) levels and diffuse brown tumor leading to a gradual decrease in the maxillary tumors. We report the case of a 50-year-old woman with chronicrenal failure presented with one-year history of slowly enlarging mass of the left cheek after 17 years of maintenance hemodialysis. The physical examination revealed a mass of the left jaw and the infraorbital region associated with a left exophtalmia. The mass was soft in consistency and tender on palpation. Her serum PTH concentration was 4830 pg/ml and her calcemia was normal. The computed tomography (CT) of the facial skeleton showed multiple osteolytic lesions of the maxillary sinuses and mandibular bone suggestive of brown tumors. The expansive lesion of the left maxillary bone found an extensive involvement of the left maxilla and the maxillary sinus. The patient underwent a subtotal parathyroidectomy. The PTH concentration taken 1 hour after the surgery confirmed a level within the normal range. At 12-month follow-up, there was an improvement of the exophtalmia and partial regression of the tumors.


The canal of Nuck is an embryological vestige of the processus vaginalis, and presents a potential site for endometriosis seeding. Hydroceles in this region are a rare cause of inguinal swelling in females. In addition, endometriosis localized to the canal of Nuck is exceedingly rare.A 44-year-old Japanese woman presented with a painful mass overlying her right pubis. She underwent surgery to completely excise the mass. During surgery, division of the external oblique aponeurosis revealed a cyst that occupied the inguinal canal and it adhered to the transverse fascia, inguinal ligament, and pubic bone. The cyst was dissected from the round ligament, and the defect in the internal inguinal ring was repaired and reinforced with mesh. On macroscopic examination, the cyst had a heterogeneous fibrous aspect with dark brown inclusions. Microscopic examination revealed that the cyst was tortuous, lined by mesothelial-like cells, and accompanied by partial subcapsular hemorrhage. Endometrium-like tissue was observed in the cystic wall. Immunohistochemical staining for podoplanin confirmed the mesothelial origin of the cyst-lining cells. The epithelial cells and stromal cells were positive for estrogen receptors.In this case of an endometriosis-associated hydrocele of the canal of Nuck, the mesothelial origin of the cyst-lining cells and endometriosis were confirmed by positive immunohistochemical staining for podoplanin and estrogen receptors, respectively. We determined that hydrocele resection and reinforcement of the anterior inguinal canal wall (if necessary) are appropriate treatments for this condition. A hydrocele of the canal of Nuck in females is analogous to the encysted hydrocele of the spermatic cord in men . InguinaEndometriosis is a relatively common condition, although the estimated frequency varies by population. A previous study has reported that endometriosis affects 6 to 10% of reproductive-aged females, 50 to 60% of females (including teenage girls) with pelvic pain, and up to 50% of females with infertility  and hydrA 44-year-old premenopausal Japanese woman was referred to our department for a painful mass overlying her right pubis. She was a homemaker, and had first noticed the mass when she lifted several heavy books. She reported feeling the same symptom before, but did not remember when it had occurred. A review of her medical history showed that she had undergone laparoscopic surgery for intrapelvic endometriosis 10 years ago, but was not given any medication after being discharged. Her menstrual cycle had been irregular, but she denied fluctuation of the mass size in response to her menstrual cycle. She had a normal vaginal delivery 5 years before the present admission. She was a social drinker and a non-smoker of tobacco. Her family history revealed that her father had chronic renal failure and hypertension.A physical examination found a 3-cm subcutaneous thickening to the right of the midline over her right pubis, and the site was painful on palpation. A laboratory examination showed normal values in standard tests, except for a slightly elevated total bilirubin level of 1.4 mg/dL. Through computed tomography of her abdomen and pelvis, we identified a subcutaneous, well-defined cystic nodule measuring 2.7 × 2.2 cm in the inguinal canal  represents the best option among current treatment choices because it allows for precise histological examination of the excised cyst and reduces the risk of relapse.


Dear Editor,Here, we report the case of a 9-year-old girl with hyperparathyroidism. Ultrasound showedrenal cysts and increased echogenicity of the parenchyma in both kidneys. The diagnostichypothesis was hyperparathyroidism secondary to chronic/polycystic kidney disease. Thepatient presented with gradually worsening kidney function and hypertension, and newimaging scans were requested. The ultrasound showed anechoic, multiloculated images inthe pyelocaliceal region of both kidneys, and perirenal, subcapsular cysts. A computedtomography (CT) scan was acquired, although no contrast agent was used, which precludedan accurate characterization. Nevertheless, the CT scan revealed changes similar tothose observed on ultrasound. We also performed magnetic resonance imaging (MRI), whichshowed pyelocaliceal, perirenal cysts, with altered intensity of the signal of the renalparenchyma and loss of corticomedullary differentiation , confirm. As a result, there is accumulation oflymph in the renal lymph ducts, making them ectatic and forming simple ormultiloculated, typically asymmetric and bilateral, collections in the pyelocaliceal,perinephric, or parenchymal regions, although, in some cases, only a part of one kidneyis affected  the adjacent structures. Those formations can compressthe kidney cortex, expand the sinus and distort the calyceal system. In some cases,small, predominantly peripheral, hypodense collections can be seen, with attenuationvalues of 0-15 HU. There may bethickening of the renal fasciae and retroperitoneal collections crossing the midline atthe level of the renal hilum. After the administration of iodine contrast, there is noenhancement of the collections or of the walls of the cystic formations. In MRI, the cysts exhibit a low signalon T1-weighted sequences-although the signal strength can be increased if there isbleeding-and a more intensesignal on T2-weighted sequences, without enhancement. In addition, RL can be diagnosedon MRI scans by identifying perirenal lymphatic collections with inversion of thecorticomedullary signal intensity,4, as depicted in RL is a rare benign disease that occurs because of miscommunication between the renallymphatic drainage system and the retroperitoneal lymphatic systemaffected -B,C. The


Sacrococcygeal pilonidal disease is a chronic, well-recognized entity, characterized by the recurrent formation of an abscess or draining sinus over the sacrococcygeal area. It is one of the most common surgical problems. Rarely, chronic inflammation and recurrent disease leads to malignant transformation, most commonly to squamous cell carcinoma (SCC).We describe an extremely unusual case of SCC developing in a 60-year-old patient with a chronic pilonidal sinus complicated by an anal fistula. After wide surgical excision of the pilonidal sinus and fistulas and because of the poor healing process 6 months later, colonoscopy and a percutaneous fistulography were performed, revealing an anal canal-pilonidal fistula. Patient was treated with a more radical surgical resection with a prophylactic loop colostomy, but healing was not accelerated. Multiple biopsies were then taken from the surgical site at the time, which revealed the development of SCC. CT and MRI imaging techniques revealed SCC partial invasion of the coccyx and sacrum. As a result, aggressive surgical approach was decided. Histological examination revealed moderately to poorly differentiated SCC, and the patient was treated with adjuvant radiation therapy postoperatively. Nine months later, recurrence was found in the sacrum and para-aorta lymph nodes and the patient died shortly after. We discuss the clinical features, pathogenesis, treatment options, and prognosis of this rare malignant transformation.The development of SCC in chronic pilonidal disease is a rare but serious complication. Symptoms are usually attributed to the sacrococcygeal pilonidal disease (SPD), and diagnosis is often made late by histological examination of biopsies. Malignant transformation should be suspected in chronic SPD with recurrent episodes of inflammation, repeated purulent discharge, poor healing, and chronic complex fistulas. Pilonidal disease is a chronic disorder that primarily affects Caucasian boys and men between the ages of 15 and 40 years old, but rarely those over 50 years old . Cases oA 60-year-old man with chronic and complex sacrococcygeal pilonidal disease (SPD) was referred to our outpatient department for evaluation and management. The SPD had manifested 7 years prior to this admission. The patient reported several episodes of pilonidal abscess formation, which had resolved with either spontaneous drainage and discharge or topical incision. His past medical history was unremarkable. The patient was morbidly obese (body mass index (BMI) 49), but his nutritional status was good. Clinical examination revealed chronic SPD with complex fistulas that had formed through chronic inflammation. He underwent wide surgical excision of the pilonidal sinus, including all fistulas, and histopathological examination of the specimen revealed chronic inflammation. The surgical trauma was left to heal by secondary intention, but even 6 months later, the healing process was poor, with seropurulent discharge detected Fig. . SubsequA new colonoscopy was scheduled, and biopsies of the anal canal were obtained. These confirmed the development of SCC. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed SCC partial invasion of the coccyx and sacrum. The inguinal lymph nodes were clinically impalpable, and imaging confirmed that they were normal. We decided that an aggressive surgical approach was warranted and performed abdominoperineal resection, followed by resection of the invaded traumatic area, complete removal of the coccyx, and partial resection of the sacrum. Perforator-based flaps were used to reconstruct the defected area. The localization of perforators around the sacrum was established preoperatively by a handheld Doppler ultrasound scanner. The pedicle was secured; the rest of the flap was raised; and once the flap perfusion was satisfactory, it was carefully lifted from the donor bed and rotated around this pedicle into the recipient defect. The donor defect was closed primarily.Histological examination revealed moderately to poorly differentiated SCC. The cells were medium to large, with moderately pleomorphic nuclei. There was focal keratinization, and the tumor exhibited a predominantly solid and focally trabecular pattern of growth. The neoplastic cells infiltrated the large bowel wall, dermis, bone, and skeletal muscle Fig. –d.Fig. 2The patient was treated with adjuvant radiation therapy postoperatively. No adjuvant chemotherapy was offered. Despite the aggressive surgical intervention, the prognosis was poor. Recurrence was found in the sacrum and para-aorta lymph nodes 9 months after the radical surgery, and the patient died of a respiratory infection soon after this.invader, which is the loose hair; the force, which causes the in-growth; and the vulnerability of the skin to the in-growth of the hair at the depth of the natal cleft [SPD frequently occurs as a chronic skin infection in the region of the buttock crease or natal cleft , 10. It al cleft . A singlal cleft . Additioal cleft . Pilonidal cleft , 10. In Malignant transformation is a rare but well-known complication of long-standing SPD, occurring in approximately 0.1% of patients with recurrent and complex SPD , 15, 16.Pilonidal carcinoma can be diagnosed by gross inspection, revealing an ulcer with a friable, rapidly growing, bleeding, and fungating margin, in the pilonidal cyst or sinus . MultiplThe most important consideration in the differential diagnosis of pilonidal carcinoma is pseudocarcinomatous hyperplasia of the squamous epithelium , 22. In When there is no evidence of inguinal adenopathy, wide surgical excision is indicated, using strict oncologic techniques of en bloc resection and minimizing violation of the tumor margins and the lesion . PresacrIn conclusion, the development of SCC in chronic pilonidal disease is a rare but serious complication. Symptoms are usually attributed to the SPD, and diagnosis is often made late by histological examination of biopsies. Malignant transformation should be suspected in chronic SPD with recurrent episodes of inflammation, repeated purulent discharge, poor healing, and chronic complex fistulas. All specimens of pilonidal disease should be sent routinely for histopathological evaluation. Histopathological studies of multiple biopsies allow us to make an accurate diagnosis of SCC and initiate aggressive surgical excision, with or without adjuvant radiotherapy, as the appropriate treatment.