Gastrointestinal stromal tumours （GISTs） are the mostcommon mesenchymal tumour of gastro-intestinaltract. Annual incidence of GIST in United States isapproximately 3000-4000. Clinical presentation ofGIST varies with location and size of tumour but GISTpresenting with palpable abdominal mass is rare. Wereport a case of 38 years old male who presented withlarge abdominal lump. Computed tomography （CT）scan showed a large solid-cystic lesion encasing secondpart of duodenum and distal common bile duct. On CTdifferential diagnosis of Leiomyoma, Leiomyosarcomaand GIST were made. The diagnosis of GIST wasconfirmed by immune-histochemical study of the biopsymaterial. Patient underwent pancreaticodudenectomy.Post-operative course was uneventful. Patient wasstarted on Imatinib therapy post-operatively. Norecurrence noted at six months follow up.
Lin Da Aw
Full Text Available ABSTRACT Main findings: A typical male looking adolescent with a legal female gender assignment presented with haematuria. Investigations led to the diagnosis of Persistent Mullerian Duct Syndrome. The condition is indeed a rare entity that needs a multidisciplinary team management. Case hypothesis: A case of Persistent Mullerian Duct Syndrome undiagnosed at birth because karyotyping was defaulted, thus resulting in a significant impact on the legal gender assignment and psychosocial aspects. Promising future implications: The reporting of this case is important to create awareness due to its rarity coupled with the rare presentation with hematuria as a possible masquerade to menstruation. There were not only medical implications, but also psychosocial and legal connotations requiring a holistic multidisciplinary management.
Ansari, Hossein; Robertson, Carrie E; Lane, John I; Viozzi, Christopher F; Garza, Ivan
Synovial cysts of the temporomandibular joint are rare, and to our knowledge, only 14 cases have been reported. The most common presentation is local pain and swelling. We present a case of a synovial cyst presenting with neuralgia in the distribution of the auriculotemporal nerve, initially misdiagnosed as trigeminal neuralgia.
Spyropoulou, Georgia-Alexandra; Pavlidis, Leonidas; Mylothridis, Panagiotis; Zaraboukas, Thomas; Demiri, Efterpi
Dupuytren disease in children younger than 10 years is rare and only 8 histologically proven cases have been reported. A histologically proven Dupuytren disease in a 10-year-old with an uncommon clinical presentation as a nodule on the radial side of the middle phalanx of the little finger is documented. Dupuytren's disease should be in the differential diagnosis in cases of nodules and contractures in the palm and fingers of children. Copyright Â© 2016 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.
Siderits, Richard; Poblete, Frederick; Saraiya, Biren; Rimmer, Cheryl; Hazra, Anup; Aye, Le
We present a case of angiosarcoma in small bowel, presenting with partial small bowel obstruction in a 79-year-old man with no history of radiation, chemotherapy, toxin exposure, or previous operative intervention. Angiosarcoma of small bowel is a rare entity which may present with nausea, abdominal pain, recurrent bleeding, and usually a history of prior radiation or exposure to specific toxins (polyvinyl chloride). Angiosarcoma of small bowel tends to spread rapidly and has a poor prognosis. We review the surgical and oncologic challenges. We report unique macroscopic findings of raised hyperemic margins, which are suggestive of a vasogenic lesion and the histologic feature of a partially retiform pattern with dense basement membrane material in an otherwise poorly differentiated lesion.
Full Text Available We present a case of angiosarcoma in small bowel, presenting with partial small bowel obstruction in a 79-year-old man with no history of radiation, chemotherapy, toxin exposure, or previous operative intervention. Angiosarcoma of small bowel is a rare entity which may present with nausea, abdominal pain, recurrent bleeding, and usually a history of prior radiation or exposure to specific toxins (polyvinyl chloride. Angiosarcoma of small bowel tends to spread rapidly and has a poor prognosis. We review the surgical and oncologic challenges. We report unique macroscopic findings of raised hyperemic margins, which are suggestive of a vasogenic lesion and the histologic feature of a partially retiform pattern with dense basement membrane material in an otherwise poorly differentiated lesion.
Although brain has been the most common site for toxoplasma infection in acquired immunodeficiency syndrome patients, involvement of spinal cord by toxoplasma has been rarely found. Spinal cord toxoplasmosis can present as acute onset weakness in both lower limbs associated with sensory and bladder dysfunction. A presumptive diagnosis can be made in patients with CD4 count
Chaudhari, Soham; Mobini, Narciss
Linear immunoglobulin A bullous dermatosis is a rare autoimmune mucocutaneous disorder caused by immunoglobulin A autoantibodies produced against several different antigens in the basement membrane zone. Clinically, it is characterized by tense vesicles or bullae, which on histopathological exam demonstrate subepidermal blister with a predominantly neutrophilic infiltrate. A smooth, linear pattern of immunoglobulin A deposition in the basement membrane zone on direct immunofluorescence is considered the gold standard for establishing a diagnosis. Treatment consists of dapsone or sulfapyridine. The authors report a 60-year-old woman who presented with pruritic erythematous patches and plaques on her trunk, back, and legs without blisters, who was diagnosed with eczema for several months with no response to prior treatments. A biopsy was performed, which was consistent with linear immunoglobulin A bullous dermatosis and later confirmed by direct immunofluorescence studies. The authors present this case to increase awareness of this rare disease, which could manifest in a nonclassical, nonblistering fashion.
Sachin R Agrawal
Full Text Available Although brain has been the most common site for toxoplasma infection in acquired immunodeficiency syndrome patients, involvement of spinal cord by toxoplasma has been rarely found. Spinal cord toxoplasmosis can present as acute onset weakness in both lower limbs associated with sensory and bladder dysfunction. A presumptive diagnosis can be made in patients with CD4 count <100/mm 3 based on a positive serum Toxoplasma gondii IgG antibodies, no recent prophylaxis against toxoplasmosis, intramedullary ring enhancing lesion in spinal cord supported by similar lesions in brain parenchyma. Institutions of antitoxoplasma treatment in such patients result in prompt clinical response and therefore avoiding the need of unnecessary invasive diagnostic tests. Here, we report a case of toxoplasmic myelitis in immunocompromised patient presenting as myelopathy who showed significant clinical improvement after starting antitoxoplasma treatment. Hence toxoplasmic myelitis should be considered in toxoplasma seropositive immunocompromised patients presenting as myelopathy and imaging studies showing ring enhancing intramedullary lesion.
Full Text Available Placental site trophoblastic tumour (PSTT is the least common form of gestational trophoblastic neoplasia accounting for only 1-2% of trophoblastic tumors. Approximately 200 cases are reported in English literature. PSTT presenting as a cervical growth is even less common. Differentiation of PSTT from other types of GTN, non-neoplastic gestational trophoblastic disease and non-trophoblastic tumors is important clinically due to differences in their therapeutic approaches.Appreciation of the morphologic features and immunophenotype allows their accurate diagnosis.Although most of the cases of PSTT behave in a benign fashion,the clinical behavior of PSTT can sometimes be variable and several prognostic factors can help to predict the biological behavior of this condition. We report a rare case of placental site trophoblastic tumor, presenting as a cervical mass, in a 38 year old female, and review the literature. [J Interdiscipl Histopathol 2013; 1(5.000: 286-289
Full Text Available Collagenous gastritis is a rare entity of unknown etiology characterized histologically by the presence of a thick subepithelial collagen band associated with an inflammatory infiltrate of gastric mucosa. A 40-year-old male presented with a history of chronic intermittent abdominal pain for about 6 months. Physical examination was unremarkable, and biological tests were within normal range. The patient underwent esophagogastroduodenoscopy and colonoscopy which showed a nodular mucosa of the stomach. Biopsies of the duodenum and colon were unremarkable. However, biopsies of the gastric fundus revealed a mild chronic gastritis characterized by lymphocytic and plasma cell infiltration of deep mucosa, without lymphoid follicle formation or active inflammation. No microorganisms were identified on routine hematoxylin and eosin or Giemsa-stained sections. Subepithelial collagen in the gastric biopsies was thickened and showed entrapped capillaries. Subepithelial collagen was highlighted by Masson's trichrome staining and was negative for amyloid by Congo Red. In the areas containing thickened collagen, there were no intraepithelial lymphocytes. The final pathological diagnosis was collagenous gastritis. Collagenous gastritis is an extremely rare disease, but it is important to recognize its characteristic endoscopic and pathologic findings to make a correct diagnosis. Specific therapy for this rare entity has not yet been established. [J Interdiscipl Histopathol 2015; 3(2.000: 68-70
Pandey, Divya; Sharma, Ritu; Salhan, Sudha
Spontaneous umbilical endometriosis occurring in absence of any previous abdominal or uterine surgery is extremely atypical. Its association with umbilical hernia is very rare and hernia getting spontaneously resolved has not been reported in literature so far. Here we report a case of a patient with spontaneous umbilical endometriosis associated with umbilical hernia which led to spontaneous hernia reduction. This was also associated with multiple uterine fibromyoma and bilateral ovarian endometrioma which were simultaneously treated by total abdominal hysterectomy with bilateral salpingo-oopherectomy along with surgical excision of the endometriotic tissue and repair of the abdominal wall defect. To the best of our knowledge, this is the first described case of spontaneous umbilical hernia reduction due to development of endometriosis.
Palazon, P; Julia, V; Saura, L; de Haro, I; Bejarano, M; Rovira, C; Tarrado, X
Anal canal duplication (ACD) is the rarest of gastrointestinal duplications. Few cases have been reported. Most cases present as an opening in the midline, posterior to the normal anus. The aim of our revision is to contribute with eight new cases, some of them with unusual presentations: five presented as the typical form, one with a perianal nodule, and two presented as two separate orifices (anal canal triplication). Complete excision was performed in all patients with no complications. ACD is the most distal and the least frequent digestive duplication. Its treatment should be surgical excision, to avoid complications such as abscess, fistulization, or malignization. Anal canal triplication has never been described before.
Full Text Available Despite being one of the most common pediatric surgical diseases, some cases of appendicitis are far from straightforward to diagnose. We present here an interesting case of chronic, retrocecal appendicitis in a dancer who presented with isolated back pain.
Full Text Available Osteomyelitis of the maxilla is now a rare event with the advent of antibiotics. The two predominant causes are odontogenic infections and sinusitis. Immunocompromised states such as diabetes, HIV, and malnutrition increase the risk of osteomyelitis. It is important to recognize this early as it is a difficult entity to treat with potentially serious consequences. We report an unusual case of right sided maxillary osteomyelitis in a lady with poorly controlled diabetes in rural Lincolnshire. Biopsy of the right maxillary bone showed features of acute osteomyelitis. This responded well to a prolonged course of oral antibiotics.
Full Text Available Vitamin K deficiency bleeding (VKDB disorder is an uncommon entity, which occurs due to inadequate activity of vitamin K-dependant coagulation factors. An 8-months-old exclusively breast-fed male infant presented with multiple, purpuric and nodular non-collapsible swellings on trunk of 4 days duration. Investigations revealed raised activated partial thromboplastin time and prothrombintime. Fibrinogen level and platelet counts were normal. Late VKDB usually presents as intra-cranial or mucosal hemorrhages.  Though skin and mucosal bleeding may occur in 1/3 rd of infants with VKDB, ′nodular purpura′ is not the common presenting feature. Earlier recognition of VKDB and immediate investigation/treatment helps prevent the potentially fatal outcome of the disease. Very little is mentioned about this entity in dermatology literature.
Full Text Available The cardiofaciocutaneous (CFC syndrome is a condition of sporadic occurrence, with patients showing multiple congenital anomalies and mental retardation and characteristic dysmorphic features. We, thus, report a rare case of this syndrome in a 1-year-old child who presented with typical features of CFC syndrome.
Full Text Available Hepatoblastoma (HB is a rare malignant tumour of the liver and usually occurs in the first three years of life. Hepatoblastoma in adolescents and young adults is extremely rare; nevertheless the prognosis is much worse than in childhood, because these kinds of tumours are usually diagnosed late. Characteristic imaging and histopathological and AFP levels help in the diagnosis of hepatoblastoma. Paraneoplastic features of hepatoblastoma are not uncommon at presentation and include erythrocytosis, thrombocytosis, hypocalcaemia, isosexual precocious puberty, and rarely hypoglycaemia. Even though hypoglycaemia is commonly seen in hepatocellular carcinoma, its association with hepatoblastoma is very rare. We present a case of 15-year-old male patient presenting with complaints of recurrent hypoglycaemic seizures ultimately leading to diagnosis of hepatoblastoma. Managed successfully with neoadjuvant chemotherapy, surgery and adjuvant chemotherapy with adriamycin and cisplatin based regimens. An extensive review of literature in the PubMed and MEDLINE did not reveal much data on paraneoplastic recurrent hypoglycaemic seizures as an initial presentation of hepatoblastomas in adolescents and young adults.
Full Text Available Hamman's syndrome is a rare entity, also known as Macklin's syndrome, a syndrome of spontaneous subcutaneous emphysema and pneumomediastinum. We report an unusual case of a young female patient presenting with breathlessness and chest pain of sudden onset, gradually progressive in nature and with history of bronchial asthma for last 8 years, along with other investigations. Chest x ray and computerized tomography was and later diagnosed as Hamman's syndrome
Hasan, Roumina; Kumar, Sandeep; Rao, Lakshmi
Pelvic localization of synovial sarcoma is a rare phenomenon and to the best of our knowledge its presentation as a large "dumb-bell"-shaped abdomino-pelvic mass showing extension to the thigh has never been reported in the literature. We report a case of a young adult presenting with retention of urine and was found to have a large abdomino-pelvic mass causing bony destruction and compression of pelvic viscera. A biopsy revealed a cellular tumor composed of spindle to oval cells arranged in a hemangiopericytomatous pattern. Histopathology was suggestive of poorly differentiated synovial sarcoma. Immunohistochemistry (IHC) was positive for vimentin, CD 99, Bcl2, Mic2 and focally for EMA and negative for CD 34, CK, desmin, synaptophysin, and WT1. Due to equivocal IHC findings molecular analysis was done which confirmed the diagnosis as synovial sarcoma.
Full Text Available Pelvic localization of synovial sarcoma is a rare phenomenon and to the best of our knowledge its presentation as a large "dumb-bell"-shaped abdomino-pelvic mass showing extension to the thigh has never been reported in the literature. We report a case of a young adult presenting with retention of urine and was found to have a large abdomino-pelvic mass causing bony destruction and compression of pelvic viscera. A biopsy revealed a cellular tumor composed of spindle to oval cells arranged in a hemangiopericytomatous pattern. Histopathology was suggestive of poorly differentiated synovial sarcoma. Immunohistochemistry (IHC was positive for vimentin, CD 99, Bcl2, Mic2 and focally for EMA and negative for CD 34, CK, desmin, synaptophysin, and WT1. Due to equivocal IHC findings molecular analysis was done which confirmed the diagnosis as synovial sarcoma.
Varsha; Kanan; Chanda,, Abhra; Qazi,, S.
TRAP (Twin Reversed Arterial Perfusion) sequence is a rare complication of monozygotic twin pregnancy. It is due to defect in early pregnancy where there is defective arterial anastomosis in placenta. This results in a fetus with an absent heart (acardiac twin). This acardiac twin has a poorly developed upper body and head also. The pump twin (with a normal heart) is also at a risk of heart failure and problems related to preterm birth1.
Full Text Available TRAP (Twin Reversed Arterial Perfusion sequence is a rare complication of monozygotic twin pregnancy. It is due to defect in early pregnancy where there is defective arterial anastomosis in placenta. This results in a fetus with an absent heart (acardiac twin. This acardiac twin has a poorly developed upper body and head also. The pump twin (with a normal heart is also at a risk of heart failure and problems related to preterm birth1.
Full Text Available There are several benign prostatic pathologies that can clinically mimic a prostate adenocarcinoma. Xanthogranulomatous prostatitis is a benign inflammatory condition of the prostate and a rare entity. A 47-year old male, with 3 years of lower urinary tract symptoms, with a palpable hypogastric tumor, digital rectal examination: solid prostate, of approximately 60 g. Initial PSA was 0.90 ng/mL. He underwent surgical excision of the lower abdominal nodule and prostatectomy. Histopathology showed xanthogranulomatous prostatitis, without malignancy. Xanthogranulomatous prostatitis is an extremely rare entity that can simulate prostate adenocarcinoma, therefore having a correct histopathological diagnosis is essential.
Noyola, Alejandro; Gil, José Fernando; Lujano, Heriberto; Piñon, Omar; Muñoz, Gabriel; Michel, José Manuel; Garcia, Jorge; Valdez, Jorge; Morales, Omar
There are several benign prostatic pathologies that can clinically mimic a prostate adenocarcinoma. Xanthogranulomatous prostatitis is a benign inflammatory condition of the prostate and a rare entity. A 47-year old male, with 3 years of lower urinary tract symptoms, with a palpable hypogastric tumor, digital rectal examination: solid prostate, of approximately 60 g. Initial PSA was 0.90 ng/mL. He underwent surgical excision of the lower abdominal nodule and prostatectomy. Histopathology showed xanthogranulomatous prostatitis, without malignancy. Xanthogranulomatous prostatitis is an extremely rare entity that can simulate prostate adenocarcinoma, therefore having a correct histopathological diagnosis is essential.
Hamidi, Hidayatullah; Muhammadi, Marzia; Saberi, Bismillah; Sarwari, Mohammad Arif
Trichobezoar is a rare clinical entity in which a ball of hair amasses within the alimentary tract. It can either be found as isolated mass in the stomach or may extend into the intestine. Trichobezoars mostly occur in young females with psychiatric disorders such as trichophagia and trichotillomania. Authors present a giant trichobezoar in an 18year old female presented with complaints of upper abdominal mass, epigastric area pain, anorexia and weight loss. The patient underwent trans-abdominal ultrasonography (USG), Computed tomography (CT), upper gastrointestinal endoscopy and subsequently laparotomy. USG was inconclusive due to non-specific findings. It revealed a thick echogenic layer with posterior dirty shadowing extending from the left sub-diaphragmatic area to the right sub hepatic region obscuring the adjacent structures. Abdominal CT images revealed a huge, well defined, multi-layered, heterogeneous, solid appearing, non-enhancing mass lesion in the gastric lumen extending from the gastric fundus to the pyloric canal. An endoscopic attempt was performed for removal of this intraluminal mass, but due to its large size, and hard nature, the endoscopic removal was unsuccessful. Finally the large trichobezoar was removed with open laparotomy. Trichobezoars should be suspected in young females with long standing upper abdominal masses; as the possibility of malignancy is not very common in this age group. While USG is inconclusive, trichobezoar can be accurately diagnosed with CT. In patient with huge trichobezoar, laparotomy can be performed firstly because of big size and location of mass, and psychiatric recommendation should be made to prevent relapse of this entity. Copyright © 2016 The Authors. Published by Elsevier Ltd.. All rights reserved.
Full Text Available Hydatid cyst is caused by the parasite Echinococcus granulosus ( L arval form in humans with lesions most frequently encountered in the liver and lungs. It can rarely involve extra - hepatic organs. Primary omental hydatid cyst is rare entity. This report presents the interesting case of a very large primary omental hydatid cyst.
Full Text Available Hybrid tumors are very rare tumors composed of two different tumor entities, each of which conforms to an exactly defined tumor category. A 14-year-old boy was referred for an intraosseous painless lesion with a histopathological feature of multiple odontogenic lesions including calcifying odontogenic cyst, complex odontoma and ameloblastic fibro-odontoma. The final diagnosis considered to be a hybrid odontogenic lesion.
Khurana, Ananta; Singal, Archana; Gupta, Seema
Syphilis is a disease known for centuries, but still continues to be a diagnostic challenge as the myriad manifestations of secondary syphilis can mimic a lot many dermatological disorders. Lichenoid syphilis is an uncommon entity, reported only occasionally in the penicillin era. We present the case of a 32-year-old woman presenting with localized annular lichenoid lesions on the neck.
Gundannavar, Gayatri; Rosh, Radhika M; Chandrasekaran, Shoba; Hussain, Ahad M
This case report presents two female patients whose chief complaint was discoloration of teeth. On careful clinical examination it was found that the patients had features of amelogenesis imperfecta and localised aggressive periodontitis. This article will give an insight of clinical and radiographic features of amelogenesis imperfecta with localised aggressive periodontitis, which is a rare clinical entity.
Full Text Available This case report presents two female patients whose chief complaint was discoloration of teeth. On careful clinical examination it was found that the patients had features of amelogenesis imperfecta and localised aggressive periodontitis. This article will give an insight of clinical and radiographic features of amelogenesis imperfecta with localised aggressive periodontitis, which is a rare clinical entity.
Full Text Available Xanthogranulomatous cystitis (XC is a rare benign disease of unknown etiology. A case of XC in a 30-year-old male is presented due to sparcity of such case report in medical literature. Patient evaluation included clinical, biochemical and radiological studies before treatment. Histological study revealed the rare diagnosis. Patient was asymptomatic at eight weeks follow-up after treatment.
Zarina Abdul Assis
Full Text Available Intraosseous nerve sheath tumors are very rare tumors accounting for lesser than 0.2% of primary bone tumors. We present an 18-year-old female who presented with left facial paresis for the last 1 year. Magnetic resonance imaging (MRI demonstrated expansile, multiseptated, enhancing bony lesion in the left petrous apex. There was also abnormal enhancement of the 7-8 th nerve complex within the internal auditory canal. Tumor was excised by subtemporal extradural approach. The lesion was diagnosed as intraosseous neurothekeoma on histopathology. This is an extremely rare tumor and its MRI appearance in this location is being described for the first time in literature.
Rafay Khan; Waqas Jehangir; Sunil Tulpule; Mohamed Osman; Shilpi Singh; Shuvendu Sen
Urothelial carcinoma in a few cases may result in cardiac metastasis. A rare presentation of this condition is its diagnosis as a result of cardiac tamponade. Tamponade is an unusual entity as a result of urothelial carcinoma and has only been reported in four cases. There have also been only a total of fifteen cases of cardiac metastasis from this form of malignancy. It is through this discussion that we emphasize the importance of early detection and monitoring of cardiac symptoms with the ...
Full Text Available A 56-year-old lady presented with a vesicovaginal fistula (VVF along with past history of abdominal hysterectomy. Biopsy of the fistulous tract showed squamous cell carcinoma (SCC. Patient underwent radical cystourethrectomy, total vaginectomy, and bilateral pelvic lymph node dissection along with ileal conduit. The final histopathology report of the resected specimen showed adenosquamous carcinoma in VVF. As this is a rare entity, we are reporting this case.
Full Text Available Spinal meningiomas are mostly intradural in location although at times these are associated with some extradural extensions. Purely extradural spinal meningiomas (EDSMs are however, extremely rare and when present, may cause diagnostic dilemma preoperatively. Only seven cases of pure EDSM have been reported till date. In this paper, we describe two cases of EDSM affecting the cervical spine and present their clinical profiles, radiological findings, operative management, and follow-up data, along with a review of the literature.
Ponce, José Burgos; Ferreira, Gustavo Zanna; Santos, Paulo Sérgio da Silva; Lara, Vanessa Soares
Lipomas are very common benign slow-growing soft tissue neoplasms composed of mature adipose tissue mostly diagnosed in the fifth decade of life. These tumors rarely present in the oral cavity, representing less than approximately 5% of all benign mouth tumors. They are usually less than 2cm in size and etiology remains unclear. We report a young male patient presenting with a giant lipoma in the buccal mucosa. Histopathology revealed a large area of mature fat cells consistent with conventional lipoma and an area of the mucosal lining of the lesion suggestive of morsicatio buccarum. In the present article, we emphasize the clinicopathological features and differential diagnosis of the disease.
Full Text Available Primary musculoskeletal myoepithelial tumors (METs are distinctly rare tumors and are being increasingly recognized as a result of improved diagnostic criteria and objective confirmation with immunohistochemical markers, including epithelial markers. Recent studies have unraveled distinct molecular mechanisms underlying these tumors. Herein, we present our second diagnosed case of an intraosseous MET that occurred in the tibia of a 37-year-old lady. The case is discussed with regards to current clinicopathological perspectives on these rather uncommon tumors, including our personal experience.
Chaman Lal Gupta
Full Text Available Actinomycosis is considered the most misdiagnosed dis-ease usually involving the cervico fascial region, thorax, abdomen and occasionally also the pelvis, usually in case of the females using intra-uterine devices. The involve-ment of the urinary tract is rare and primary actinomyco-sis of urinary bladder is still rarer. The disease is usually diagnosed by demonstration of the discharged sulphur granules. In our case this was not the usual presentation and the patient was first diagnosed as having appendicu-lar mass and then bladder malignancy. The excised mass demonstrated features suggestive of actinomycosis.
Full Text Available Emphysematous epididymo-orchitis is a rare cause of an acute scrotum and is a surgical emergency. Diagnosis is clinically difficult, and sonography with a high-frequency probe is useful to pick up gas shadows in the scrotal wall or testicular substance. A diabetic patient presented with fever, urinary tract infection, and an acute scrotal swelling. The patient needed orchidectomy and scrotal debridement. As in emphysematous pyelonephritis, this condition occurs in diabetics, and patients may need surgery. There is a need to perform sonography in all diabetic patients with an acutely inflamed scrotum, because detection of gas shadows makes surgical intervention more likely.
Full Text Available Herpes zoster oticus also known as Ramsay Hunt syndrome is a rare complication of herpes zoster in which reactivation of latent varicella zoster virus infection in the geniculate ganglion causes otalgia, auricular vesicles, and peripheral facial paralysis. Ramsay Hunt syndrome is rare in children and affects both sexes equally. Incidence and clinical severity increases when host immunity is compromised. Because these symptoms do not always present at the onset, this syndrome can be misdiagnosed. Although secondary to Bell′s palsy in terms of the cause of acute atraumatic peripheral facial paralysis, Ramsay Hunt syndrome, with incidence ranged from 0.3 to 18%, has a worse prognosis. Herpes zoster oticus accounts for about 12% cases of facial palsy, which is usually unilateral and complete and full recovery occurs in only about 20% of untreated patients. The most advisable method to treat Ramsay Hunt syndrome is the combination therapy with acyclovir and prednisone but still not promising, and several prerequisites are required for better results. We present a case of 32-year-old man suffering from Ramsay Hunt syndrome with grade V facial palsy treated effectively with rehabilitation program, after the termination of the combination therapy of acyclovir and prednisone.
Full Text Available INTRODUCTION: Schwannomas are the benign tumours arising from the Schwann cells. Intraoral and pharyngeal schwannomas are rare and constitute less than 1%. CASE REPORT: We report a case of a 39 year old lady who presented with progressive dysphagia and dysphonia since three months. Intraoral examination showed a well - defined reddish lesion arising from the base of the tongue. She was referred to the department of radiodiagnosis for CT and MRI of the neck to know the extent of the lesion. Differential diagnosis of schwannoma and minor salivary gland tumor was given. Excision biopsy of the lesion was done and the histologic examination showed it as schwannoma. DISCUSSION: Schwannomas of the base of the tongue are rare and should be included in the differential diagnosis based on the imaging features and enhancem ent pattern. CONCLUSION: Imaging features, particularly MRI with contrast helps in differentiating benign from malignant lesions of the base of the tongue. Schwannomas are benign and have good prognosis as they can be excised when compared to the other intraoral malignant lesions.
Full Text Available Primary malignancy of the urachal remnant is a rare neoplasm that accounts for less than 0.01% of all adult cancers, with an estimated annual incidence of 1:5 million. The tumour carries a grave prognosis that attests to its highly aggressive nature. Owing to its extra-peritoneal location, the tumour runs a relatively silent clinical course until late presentation, when most patients display extensive local invasion and metastatic spread. In this report, we highlight a case of primary malignancy of the urachus that on initial clinical evaluation masqueraded as a Sister Mary Joseph’s nodule. Characteristic imaging features, however, proved decisive in establishing the diagnosis of a urachal carcinoma.
Hidayatullah Hamidi, Dr, MD
Conclusion: Trichobezoars should be suspected in young females with long standing upper abdominal masses; as the possibility of malignancy is not very common in this age group. While USG is inconclusive, trichobezoar can be accurately diagnosed with CT. In patient with huge trichobezoar, laparotomy can be performed firstly because of big size and location of mass, and psychiatric recommendation should be made to prevent relapse of this entity.
Full Text Available Splenic abscess is a rare clinical entity with an incidence of 0.2-0.7% in autopsy-based studies. When untreated, splenic abscess is associated with nearly 100% mortality; in treated patients, the mortality rate is 16.6% during the first 90 days. It mostly occurs in patients with neoplasia, immunodeficiency, trauma, diabetes or splenic infarct. The incidence of splenic abscess is thought to be growing because of the increase in the number of immunocompromised patients who are particularly at risk for this disease and also because of the widespread use of diagnostic modalities. However, the optimal treatment for this remains unclear. We present a case of a 42-year-old man diagnosed with multiloculated splenic abscess and was subjected to splenectomy.
Full Text Available The popliteal pterygium syndrome is a congenital malformation that includes orofacial, musculoskeletal and genitourinary anomalies. It is a rare autosomal dominant disorder. We report one family with popliteal pterygium syndrome affecting father and his two daughters, who underwent surgical corrections for multiple congenital malformations.
Pichierri, Angelo; Ruggeri, Andrea; Morselli, Carlotta; Delfini, Roberto
Fourth ventricle meningiomas (FVMs) are rare, often misdiagnosed, lesions. To the best of our knowledge, 47 cases have been reported in the literature: we describe our series of three cases treated at our Institution, focusing on some diagnostic tips and intraoperative features of these tumours. Our three patients have a history of headache. Gait disturbances, vomiting and/or diplopia complicated the clinical picture before the referral at our Department. The operations were uneventful, and the patients fully recovered from neurological symptoms. They are free of recurrence at a median follow-up of 19 years. FVMs are rare lesions, which are difficult to differentiate preoperatively from the much more common ependymomas. A preoperative distinction would be extremely advantageous: indeed, although both tumours share similar radiological and clinical patterns, they clearly differ as to surgical difficulty and outcome. In fact, meningiomas are comparatively easier to remove, granting better clinical results.
Majdoub, Senda; Zemni, Houcem; Zaghouani, Houneida; Ben Salem, Halima Houda; Amara, Habib; Bakir, Dajla; Kraeim, Chakib
Dermatomyositis is a connective characterized by inflammation of skeletal muscle with cutaneous manifestations. Their etiologies, still unknown, associate environmental and genetic factors. Among lung complications described, interstitial pneumopathies are common complications. Other complications are rarely reported as pneumomediastinum. We report a case of pneumomediastinum with aeric in massive skin dissection occurred in a patient with dermatomyositis. We will discuss the frequency, causes and pathophysiology of the disease according to the literature.
Sousa, Jamira; Cabezuelo, Lourdes; Rodrigues, Adriano; Costa, Nascimento; Cipriano, Maria Augusta
Heterotopic gastric mucosa is an extremely rare condition; in the anorectal region only a very few cases were described up to now. The authors report a case of a 46-year-old woman, asymptomatic, to whom a massive recess was discovered in the rectum, at 12 cm of the anal verge; a bottom flat lesion with 1.5 cm of diameter revealed a focus of normal appearing fundic-type gastric mucosa at histology.
Zaeem Ismail Ebrahim
Full Text Available Pseudomyxoma peritonei (PMP is a rare complication of mucinous tumours of appendiceal or ovarian origin that results in peritoneal and omental implants. Clinical morbidity and mortality arise from the fact that copious amounts of extracellular and peritoneal mucin result in distortion and loss of function of visceral organs. Therapeutic paracentesis is not possible because of the nature of the mucin. Currently, new techniques are being used to attempt to debulk the mucin volume; none, however, has lead to superior outcome.
Sunil N Mishra
Full Text Available Trigeminal trophic syndrome is a rare condition resulting from self-manipulation of the skin after a peripheral or central injury to the trigeminal system. The syndrome consists of a classic triad of anaesthesia, paraesthesia, and a secondary persistent or recurrent facial ulceration. We describe a 60 year-old woman who developed this syndrome as a sequel to the gasserian ganglion block for trigeminal neuralgia. She had also developed melasma within 1 year. A remarkable benefit was achieved by proper patient education and topical antibiotics which led to the healing of all ulcerations within 4 weeks. In the case reported here, the diagnosis of the trigeminal trophic syndrome was made primarily as a result of the physician′s experience with the syndrome previously.
Full Text Available Pineal tumors comprise 0.4-1.0% of intracranial space occupying lesions in adults. Papillary tumor of pineal region (PTPR is a very rare entity. It has been newly described in WHO 2007 classification of brain tumors. Only a few case reports are available in the literature. We report a case of a 60-year-old female presenting with headache, giddiness and reduced vision. Imaging studies showed a pineal mass with areas of hemorrhage. All ventricles were normal. There was a past history of a pineal gland tumor excised 2 years ago. This case is being reported for its rarity and aggressiveness in the form of recurrence. Limited/available immunohistochemistry workup has been done.
Azurara, Laura; Marçal, Mónica; Vieira, Filipa; Tuna, Madalena Lopo
Pituitary gland duplication is a particularly rare finding. Different theories have been proposed to explain its pathogenesis, however, this phenomenon is not yet totally understood. Recently, duplication of the pituitary gland (DPG)-plus syndrome has been described, associating DPG with other blastogenic defects. We present the clinical and imaging findings of a newborn girl with DPG, associated with multiple other midline anomalies, including a nasopharyngeal teratoma, palate cleft deformity, bifid nasal bridge, tongue and uvula, hypoplasia of the basis pontis and corpus callosum, duplication of the basilar artery and hypothalamic hamartoma. We describe our patient's multidisciplinary team approach and emphasise the importance of reporting upcoming cases, in order to give more insight into the understanding of this complex entity.
Rohan S Valsangkar
Full Text Available Granulomatous inflammation of the prostate is a rare type of inflammation of the prostate. It is of various types, with the non-specific type of granulomatous inflammation being the most common. Xanthogranulomatous prostatitis is a rare type of granulomatous prostatitis of which very few cases have been reported. Histologically it is characterized by the presence of pale-looking foamy macrophages. It can be an incidental finding after transurethral resection of the prostate (TURP, although it may mimic prostatic malignancy clinically, biochemically, and rarely histologically. We report a rare case of xanthogranulomatous prostatitis which presented as a prostatic abscess, a presentation never reported in literature so far. The patient was managed with TURP.
Rupareliya, Chintan; Hejazi, Seyedali
Pure alexia refers to an acquired disorder associated with the damage to medial occipitotemporal gyrus in the dominant hemisphere, which is also known as visual word form area (VWFA). VWFA is involved in rapid word recognition and fluent reading. Alexia without agraphia is a disconnection syndrome that occurs when the splenium is also damaged with the occipital lobe on a dominant side. We report a case of a 72-year-old right-handed male who presented with alexia without agraphia accompanied by right homonymous hemianopia resulting from acute infarct of the left occipital lobe, the splenium of the corpus callosum and posterior thalamus that probably occurred on the previous day. During the evaluation, he exhibited marked impairment in the ability to read with the vision being grossly normal. Magnetic resonant imaging (MRI) revealed an acute infarct of the left occipital lobe, the splenium of the corpus callosum and posterior thalamus. A computerized tomography angiogram (CTA) revealed left posterior cerebral artery (PCA) territory infarct without any evidence of hemorrhagic conversion. Infarction of the occipital lobe on the dominant side (left) in a right-handed individual may cause a disruption in the visual word form area and is manifested by an inability to read with no abnormalities in visual acuity. PMID:28690938
Villalón-López, José Sebastián; Souto-del Bosque, Rosalía; Montañez-Lugo, Juan Ignacio; Chávez-González, Bruno
Splenic metastases from solid tumors are a rare event with an incidence of only 2.9% to 9%. Splenic metastases from cervical cancer are a rare entity. Only a few cases have been reported of isolated spleen metastases from cervical cancer. We present the case of a 76-year-old woman with moderately differentiated endocervical adenocarcinoma stromal and endocervical invasion. Clinical stage was Ib1 and Ca-125 values of 150 U. She was managed with hysterectomy and pelvic lymphadenectomy. She received pelvic radiotherapy (45 Gy) followed 24 Gy of brachytherapy. Two years later she presented with abdominal pain. Abdominal computed tomography showed two splenic parenchymal lesions without disease in the remainder of the abdominal cavity and chest with a Ca-125 of 2,733 U. The patient is submitted to splenectomy. Histopathology demonstrates splenic metastases of well-differentiated adenocarcinoma from the endocervix. Immunohistochemical stain showed positivity from carcinoembryonic antigen; estrogen and progesterone receptors are negative. Ca-125 level 8 weeks after surgery was 16 U/ml. The patient received six cycles of adjuvant chemotherapy with paclitaxel and cisplatin. At 12 months follow-up the patient is alive and without evidence of tumor activity. The spleen is an uncommon site of metastasis. Splenectomy is considered the appropriate treatment in order to avoid complications such as splenic rupture and splenic vein thrombosis as well as to improve pain control from splenomegaly. Twelve months after surgery our patient is alive and without evidence of tumor activity.
Managutti, Anil; Managutti, Sunita; Patel, Harsh; Menat, Shailesh
Orthokeratinized odontogenic cyst (OOC) is a rare developmental jaw cyst, considered as distinct entity from odontogenic keratocysts as it exhibits a less aggressive behaviour and a very low rate of recurrence. Most commonly occurs in mandibular molar and ramus region, rarely seen in maxilla and mandibular premolar area. But till now never reported in condyle, this article describes a case of OOC involving the mandibular condylar head in a 41-year-old male. The initial clinical diagnosis was given as TMD based on the clinical features, but radiographic evaluation revealed osteolytic lesion in right side condylar head. Condylotomy was performed and the specimen was sent for histopathological examination. A definite diagnosis of OOC was made by histopathological examination of the biopsy specimen. We report a rare entity in this article with its management.
Full Text Available Hydatid cyst is caused by the parasite Echinococcus granulosus commonly seen in temperate regions. Primary omental hydatid cyst is rare entity. Diagnosis can be achieved with contrast-enhanced computed tomography of abdomen and pelvis along with serology. Eosinophilia is a strong pointer to hydatid cyst as a differential diagnosis. Open or laparoscopic excision of the cyst along with medical therapy remains the treatment of choice.
Ahuja, Arvind; Sharma, Mehar Chand; Suri, Vaishali; Sarkar, Chitra; Sharma, B S; Garg, Ajay
Pineal anlage tumour is a rare tumour of the pineal gland that is not listed in the 2007 World Health Organization classification of tumours of the central nervous system. Pineal anlage has been defined as a primary pineal tumour with both neuroepithelial and ectomesenchymal differentiation but without endodermal differentiation. We report a pineal anlage tumour in a 4-month-old boy, the youngest patient reported with this rare tumour, with a brief review of the literature. Clinicians and neuropathologists should be aware of this entity as it is likely to be misdiagnosed as a teratoma or a melanocytic tumour of the central nervous system.
Tudisco, Jean-Blaise; Fumeaux, Christophe; Petignat, Pierre-Auguste
Eosinophilic meningitis is a rare entity, which is a complication of an underlying disease. Its diagnosis and treatment is always a challenge for the hospital practitioner. The aim of this case report and review is to identify the most important aetiologies, and show the diagnostic and therapeutic modalities of Eosinophilic meningitis. The most frequent causes of Eosinophilic meningitis are parasitic and fungal infections. In Europe Eosinophilic meningitis is essentially seen in travellers returning from endemic areas for these agents. The treatment is directed against the underlying disease and can differ depending on the aetiology and severity of the clinical manifestations.
Full Text Available Upper gastrointestinal bleeding can be a life threatening complication. Hookworms infestation usually causes chronic blood loss resulting in anemia. A 52 year old male presented to us with massive gastrointestinal blood loss and hemodynamic instability. On examination he had anemia, which on evaluation was iron deficient microcytic hypochromic anemia with eosinophilia. On evaluation of small bowel mucosa showed multiple hookworms moving on the surface. He improved with anthelmintics, blood transfusion and supportive management. The repeat evaluation showed eradication of worms. Hookworms presenting as a massive gastrointestinal bleeding is a rare entity. Enteroscopy plays an important role in diagnosing hookworm infestation.
Rather, Ajaz; Salati, Sajad Ahmad
Ascariasis is the most common helminthic infection in the world. We present a very rare manifestation of ascariasis in which the worms came out through the T-tube tract of a 36 years old patient who had undergone cholecystectomy with choledocholithotomy.
Full Text Available In this case report, we have brought out a very rare presentation of hypothyroidism in the form of cataplexy and this case is of significance because there have been no similar case reports of hypothyroidism presenting as cataplexy so far. The other highlight of the case is that treatment of hypothyroidism alone resulted in complete freedom from cataplexy without the need for agrypnotic drugs. [Int J Res Med Sci 2014; 2(1.000: 328-329
Full Text Available Malignant sinonasal carcinomas are a rare entity comprising less than 1% of all cancers and around 3% of all head and neck malignancies seen in humans. Among these 15-20% are transitional cell carcinoma also known as non keratinizing carcinoma of sinonasal tract. We are reporting the case of a 45 years female with history of nasal obstruction and epistaxis. A contrast enhanced computed tomography (CECT was done which showed mucosal thickening in the right nasal cavity. Endoscopy assisted biopsy was taken which revealed non keratinizing carcinoma (transitional type. Very few reported cases of this type of malignancy was found. A possible reason could be multiple synonyms like cylindrical cell carcinoma, Schneiderian carcinoma and transitional cell carcinoma.
Almodovar-Real, Ana; Aneiros-Fernández, Jose; Molina-Leyva, Alejandro; Fernandez-Pugnaire, Ma Antonia
Palmoplantar lichen planus is a localized and rare subtype of lichen planus (LP) often underdiagnosed. Several morphological types of palmoplantar lesions have been defined in LP. We present an unusual case of the palmoplantar kyperkeratotic variant of LP. Histopathology examination confirmed our diagnosis. We emphasize the importance of this rare entity in the differential diagnosis of palmoplantar dermatoses.
Full Text Available Hemophilia A is a X-linked hereditary condition that lead to decreased factor VIII activity, occurs mainly in males. Decreased factor VIII activity leads to increased risk of bleeding events. During neonatal period, diagnosis is made after post-partum bleeding complication or unexpected bleeding after medical procedures. Subgaleal hemorrhage during neonatal period is a rare, severe extracranial bleeding with high mortality and usually related to traumatic labor or coagulation disorders. Subgaleal hemorrhage complications result from massive bleeding. We present a neonate with unremarkable family history and uneventful pregnancy with a vaginal delivery with no instrumentation, presenting with severe subgaleal bleeding at 52 hours of life. Aggressive support measures were implemented and bleeding managed. The unexpected bleeding lead to a coagulation study and the diagnosis of severe hemophilia A. There were no known sequelae. This case shows a rare hemophilia presentation reflecting the importance of coagulation studies when faced with unexplained severe bleeding.
Full Text Available Airplane travel headache is rare and has recently been described as a new form of headache associated with a specific situation. Of the 1,208 patients with primary headaches attending a tertiary care neurology hospital, two (0.16% patients satisfied the criteria for headache related to airplane travel. Both the patients fulfilled the proposed diagnostic criteria for airplane travel headache. This unique headache had a mean duration of 24 minutes, localized to the medial supraorbital region described as having an intense jabbing or stabbing character that occurred exclusively and maximally during aircraft landing or take-off, following which pain intensity subsided . This rare headache felt on aircraft descent is probably due to the squeeze effect on the frontal sinus wall, when air trapped inside it contracts producing a negative pressure leading to mucosal edema, transudation and intense pain. Use of nasal decongestants either alone or in combination with naproxen sodium prior to ascent and descent abated the headache episodes. Awareness about this unique entity is essential to provide proper treatment and avoid patient suffering.
Garg, Ravinder; Kukar, Neetu; Bajwa, Sukhminder Jit Singh; Kaur, Shaminder
Cold agglutinin disease (CAgD) is a type of autoimmune hemolytic anemia which generally occurs in adults and is characterized by the presence of IgM antibodies directed against polysaccharide antigens on red blood cell surface. A 16-year-old male, having clinical picture of sepsis and anemia, presented to the Emergency Department of our Institute in an Hemodynamically unstable condition. Investigation profile revealed hemolysis due to CAgD, which responded to corticosteroids, antibiotics and supportive treatment. This case highlights the importance of recognizing this entity in such type of cases presenting with sepsis and anemia. PMID:26229347
Saurabh R Jindal
Full Text Available A patient presenting with fever and purpura often presents a diagnostic challenge for the dermatologist. Acute hemorrhagic edema of infancy (AHEI is a rare acute cutaneous leukocytoclastic vasculitic syndrome of infants leading to this presentation. We present an 18-month-old girl with AHEI who presented with fever, edema, and purpuric lesions involving the face, ears and extremities with uneventful complete recovery.
Carr, S. P.; MacNamara, F. T.; Muhammed, K. M.; Boyle, E; S. M. McHugh; Naughton, P.; Leahy, A
Introduction. Gallstone ileus (GSI) of the colon is an extremely rare entity with potentially serious complications including perforation. Case Presentation. An 88-year-old man presented to the emergency department with abdominal pain and distension. Clinical exam revealed signs of peritonism. Computed tomography (CT) revealed GSI of the transverse colon with a closed-loop large bowel obstruction (LBO) and caecal perforation. The patient underwent emergency laparotomy. A right hemicolectomy w...
Full Text Available Eccrine syringofibroadenoma (ESFA is a rare, benign tumor of eccrine sweat gland origin that usually presents as a nodule on the extremities of an elderly person. It can also present as an ulcerative plaque, verrucous lesion, papular or nodular lesion or as palmoplantar keratoderma. Although the clinical features are variable, histology is characteristic in the form of anastomosing strands, cords and columns of epithelial cells embedded in a fibrovascular stroma. We report the case of a 62-year-old male with a nodular lesion on the extremity that caused secondary involvement of the nail in the form of complete nail dystrophy. The histology showed features consistent with ESFA. Nail involvement by ESFA is a rare presentation and is rarely described in the literature.
Fiorani, Cristina; Scaramuzzo, Rosa; Lazzaro, Alessandra; Biancone, Livia; Palmieri, Giampiero; Gaspari, Achille L; Sica, Giuseppe
Duplications of the alimentary tract (ATD) are rare congenital anomalies often found early in life. They may occur anywhere in the intestinal tract but the ileum is the most frequently affected site. Clinical presentation of ATD in adults is variable and because these lesions occur so infrequently they are rarely suspected. In the present report we describe a case of ileal duplication in a 61-year-old patient with Crohn’s disease. Despite various radiological investigations and medical consultations, the diagnosis was only made on the surgical specimen. PMID:22007281
Jiten P Kothadia
Full Text Available The increased deposition of iron in gastric mucosa is known as gastric siderosis. It is believed that the only regulated step of the iron metabolism cycle occurs during absorption in the small intestine. Once this system becomes overwhelmed due to either local or widespread iron levels, then iron can be absorbed very quickly by a passive concentration-dependent mechanism. This excess iron is initially stored in the liver but later can be found in the pancreas, heart and joints. Excess iron is not expected to deposit in the gastric mucosa. This gastric deposition has been found in association with hemochromatosis, oral iron medications, alcohol abuse, blood transfusions, hepatic cirrhosis and spontaneous portacaval shunt with esophageal varices. The precise mechanism of this iron deposition in gastric epithelial and stromal cells is still not well understood; thus, identification of iron in gastric mucosa raises many questions. On histology, the pattern of deposition is variable, and recognition of the pattern is often useful to choose the appropriate workup for the patient and to diagnose and possibly treat the cause of iron overload. In this article, we have described a well-referenced review of this rare clinical entity with different histological patterns, diagnostic tests and the clinical significance of the different patterns of iron deposition.
Ball, William Robert; Elshaieb, Mohamed; Hershman, Michael Jeremy
An elderly woman presented with abdominal pain and vomiting, was known to have gallstones. A CT scan was arranged identifying gallstone ileus and cholecystitis. Ensuing sepsis precipitated fast atrial fibrillation delaying the planned laparotomy. Her symptoms subsequently resolved with conservative management. Ten days following admission her abdomen became distended. A repeat CT scan showed large bowel dilation with intramural air suggestive of obstruction and bowel ischaemia. Emergency laparotomy was performed identifying a large 23 mm gallstone impacted at the rectosigmoid junction (gallstone coleus). The stone was milked back to the transverse colon where it was retrieved and a transverse loop colostomy was formed. Gallstone ileus is rare; gallstone coleus is even rarer. On review of the published literature both entities have not been seen in the same patient during the same admission or indeed caused by the same gallstone.
Vandana U Grampurohit; Hephzibah Rani; S S Parinitha; U S Dinesh
Neoplastic cells with myoepithelial differentiation are often present in both benign and malignant salivary gland neoplasms. Potential diagnostic problems may arise due to morphologic heterogeneity of myoepithelial cell-rich lesions in fine-needle aspiration cytology (FNAC). Myoepithelial carcinoma is a malignant salivary gland tumor composed exclusively of cells with myoepithelial differentiation. Histopathologically, it is a well-established entity but its cytological features have rarely b...
Full Text Available Abstract Background Intestinal tuberculosis is a common problem in endemic areas, causing considerable morbidity and mortality. An isolated primary caecal perforation of tubercular origin is exceptionally uncommon. Case presentation We report the case of a 39 year old male who presented with features of perforation peritonitis, which on laparotomy revealed a caecal perforation with a dusky appendix. A standard right hemicolectomy with ileostomy and peritoneal toileting was done. Histopathology revealed multiple transmural caseating granulomas with Langerhans-type giant cells and acid-fast bacilli, consistent with tuberculosis, present only in the caecum. Conclusions We report this extremely rare presentation of primary caecal tuberculosis to sensitize the medical fraternity to its rare occurrence, which will be of paramount importance owing to the increasing incidence of tuberculosis all over the world, especially among the developing countries.
Full Text Available Persistent Mullerian duct syndrome (PMDS is a rare form of Disorder of sex development in which Mullerian duct derivatives (fallopian tubes, uterus and the proximal vagina are present in an otherwise normally differentiated 46 XY male. In the majority of cases, PMDS is a surprise finding either during orchidopexy or during inguinal hernia repair. We report a case of 4 year child with female type (Type III PMDS. We are discussing the presentation, management and review of the literature.
Eduardo SM Fernandes; Raquel L Bernardo; Moacir M Fernandes; Rogério MC Araújo; Renato Sebbe; Asterio Monte; José F Coelho; Antonio AP Souza; Joaquim Ribeiro Filho
BACKGROUND: Douglas described choledochal cysts in 1852 and Todani proposed an anatomy-based classiifcation in 1977. The classiifcation is the most extensively used, but does not include some rare variations. We present a case of hepatic duct diverticulum, one of the variations, and discuss its diagnosis and treatment. METHODS: A 57-year-old woman presented with upper abdominal pain and discomfort associated with nausea and vomiting. She was ifnally diagnosed with cholelithiasis and right hepatic duct diverticulum. RESULT: The patient underwent resection of the hepatic duct diverticulum and cholecystectomy, and was asymptomatic 26 months after surgery. CONCLUSIONS: Hepatic duct diverticulum is a rare form of choledochal cyst, not included in Todani's classiifcation. Todani's classiifcation including this and other uncommon variations of choledochal cysts must be reviewed. The best diagnostic imaging methods and treatment for choledochal cysts must be deifned.
Shaik Afshan Jabeen
Full Text Available Leigh syndrome (LS is a heterogeneous familial or sporadic neurodegenerative disorder. It is typically seen in infancy or childhood, although rare cases of adult onset have been described. The authors describe a 37-year-old woman who presented with protracted gastrointestinal symptoms followed by acute brain stem syndrome with severe metabolic acidosis and who subsequently showed dramatic clinical and neuroradiological improvement.
Full Text Available Double elevator palsy (DEP currently known as Monocular Elevation Deficit is a rare condition characterized by restricted elevation of one eye in all positions of up gaze . Clinically it presents as a unilateral restriction of superior rectus (SR and inferior oblique (IO. We present a case report of DEP in 3 year old boy who presented with sudden onset of DEP in right eye in our OPD. Very little information on acquired causes of DEP is available. Diagnosis , differential diagnosis and m anagement are discussed and then the literature is reviewed.
Kharel Sitaula, R; Batta, S; Shrestha, G B; Shrestha, J K
Kissing nevus is a congenital nevus in adjacent parts of the eyelids. Malignant transformation of kissing or divided nevi of the eyelids is rarely described. To report a very rare case of malignant transformation of kissing nevus with ocular and extraocular spread. A 57- year- old man with 6/6 visual acuity in both eyes presented with a kissing nevus present since birth in right upper and lower eyelids which had a slow growth phase. The upper lid in the area of the nevus was thickened with a 20x12x15 mm black pigmented crusted hemorrhagic nodular lesions. The lower lid had a 6 mm black pigmented ulcerated lesion over the pre-existing nevus in the lateral third of the lid with full thickness infiltration. Another 5x4 mm pigmented lesion over the lower medial lid margin with a thickness of about 3 mm extended to the conjunctival side of the lower lid. Right sided pre-auricular and sub-maxillary nodes were palpable. A biopsy of tissue samples from the eyelid and pre-auricular nodes were consistent with malignant melanoma. Malignant transformation of kissing nevus is rare. It can spread to the conjunctiva,pre-auricular and sub-mandibular lymphnodes. © NEPjOPH.
Gómez Sánchez, María Encarnación; de Manueles Marcos, Fernando; Martínez Martínez, Maria Luisa; Vera Berón, Roberto; Azaña Défez, Jose Manuel
Acral persistent papular mucinosis (APPM) is a rare subtype of localized lichen myxedematosus. It consists of small papules localized exclusively on the back of the hands, wrists and extensor aspects of distal forearms with no other clinical or laboratory manifestations. The lesions tend to persist and may increase slowly in number. Histologically, hematoxylin-eosin and Alcian blue staining demonstrate mucin accumulation in the upper reticular dermis with separation of collagen fibers as a result of hyaluronic acid deposition. Treatment is rarely necessary due to the absence of symptoms. We present a 27-year-old healthy woman with asymptomatic papules on her upper extremities, which adequately meet clinical and pathological criteria of acral papular mucinosis.
Full Text Available Ellis-van Creveld (EVC syndrome is a genetic disorder with autosomal recessive transmission, which may clinically present as small stature, short limbs, fine sparse hair, hypoplastic fingernails, multiple musculofibrous frenula, conical teeth, hypoplasia of the enamel, hypodontia, and malocclusion. Heart defects, especially abnormalities of atrial septation, have been found in about 60% of cases. The mutation in EVC and EVC2 gene is responsible for this syndrome. The presence of multiple orodental findings makes this syndrome important for dentists. The aim of this article is to present a rare case of EVC syndrome in a 10-year-old girl along with the review of literature.
Kamal, Reet; Dahiya, Parveen; Kaur, Simerpreet; Bhardwaj, Rohit; Chaudhary, Karun
Ellis-van Creveld (EVC) syndrome is a genetic disorder with autosomal recessive transmission, which may clinically present as small stature, short limbs, fine sparse hair, hypoplastic fingernails, multiple musculofibrous frenula, conical teeth, hypoplasia of the enamel, hypodontia, and malocclusion. Heart defects, especially abnormalities of atrial septation, have been found in about 60% of cases. The mutation in EVC and EVC2 gene is responsible for this syndrome. The presence of multiple orodental findings makes this syndrome important for dentists. The aim of this article is to present a rare case of EVC syndrome in a 10-year-old girl along with the review of literature.
Gasco, Jaime; Franklin, Brodus; Rangel-Castilla, Leonardo; Campbell, Gerald A; Eltorky, Mahmoud; Salinas, Paul
Angioleiomyomas are benign neoplasms most often located in the subcutaneous tissue of middle-aged individuals and usually confined to the subcuticular and deep dermal layers of the lower extremities. An intracranial site for this tumor is exceedingly rare, with very few reports documenting locations in the neuraxis. To the authors' knowledge the present case represents the first reported instance of an infratentorial angioleiomyoma. The authors conducted a review of selected English-language papers published since 1960 describing well-documented cases of intracranial vascular leiomyomas, with detailed information on the clinical presentation, radiology, pathology, and particulars of surgical management in each case.
Full Text Available Although metastases to bones from solid tumors are very common, involvement of small bones of the hands is extremely rare. We report the first case of acrometastasis in vaginal carcinoma. We present a 65-year-old multiparous woman with FIGO Stage II vaginal carcinoma. The patient received treatment with external beam radiotherapy followed by brachytherapy. She had complete response to above treatment. Eleven months later, she presented with swelling on dorsum of hand. Investigations revealed metastasis to 4th metacarpal bone with lung metastasis. Hence, we present a patient with metastasis of carcinoma vagina to the 4 th metacarpal to draw the attention for the potential of such lesions to be developed in this region. It should be remembered that bone metastasis at unusual sites might be seen in vaginal carcinoma. We present this case because of its rarity and for documentation and discussion.
Full Text Available Tuberculosis of the musculoskeletal system is generally confined to bones and joints. The surrounding soft tissue is secondarily infected. Tuberculous bursitis, tenosynovitis and primary pyomyositis are rarer manifestations of the disease. Of these, primary tuberculouspyomyositis is probably the rarest entity. We report a case of tubercular myositis of infraspinatus in an 8 year-old female who presented with pain, low grade fever, weight loss, anorexia, progressively increasing pain in the scapular region and restriction of movements. There was no history of trauma, diabetes, immunosuppression, corticosteroid usage, or renal failure. History of contact was present. Tenderness was present along the medial border of scapula and movements of upper extremity requiring movement of the scapula were painful and grossly restricted. MRI of the scapulothoracic region and shoulder revealed small amount of fluid along medial border of scapula with T2 hyperintensity of infraspinatus. Histopathology showed caseous necrosis, inflammatory cells and granulomatous cells suggestive of tuberculosis. Polymerase Chain Reaction for Mycobacterium tuberculosis was found to be positive. Patient was started on four-drug antitubercular treatment and regular dressings. The patient's general condition improved and at 4 weeks post starting ATT, there was no pain and the patient was able to perform complete range of movement. This is probably the first reported case of tubercular myositis of infraspinatus in an immunocompetent patient without any identifiable focus elsewhere in the body. Rarity of the condition, presence of characteristic findings on MRI and histopathology make the case illustrative for young Orthopaedics surgeons. [Int J Res Med Sci 2016; 4(8.000: 3619-3621
Zahedi, Farah Dayana; Gendeh, Balwant Singh; Husain, Salina; Kumar, Ramesh; Kew, Thean Yean
Esthesioneuroblastoma is a rare malignant neoplasm of oflactory neuroepithelium and usually located at the olfactory cleft at superior nasal cavity. Ectopic localization of esthesioneuroblastoma is even rarer and usually posed with a diagnostic dilemma and delay in the diagnosis and management, We report a rare case of ectopic esthesioneuroblastoma of the sphenoclivus with the presentation of intermittent unilateral epistaxis, intermittent intractable headache without anosmia. Nasal endoscopy findings showed a pulsatile mass at the anterior face of the sphenoid sinus with extension posteriorly towards the clivus region and occupying the floor of the sphenoid sinus. Endonasal transclival endoscopic excision of tumour was performed which involved otorhinolaryngology surgeon and neurosurgeon with intraoperative navigation imaging and frozen section. The histopathological findings was esthesioneuroblastoma. Due to its rarity and unusual presentation, the diagnosis of ectopic esthesioneuroblastoma is difficult and can be misdiagnosed with the other type of malignancy. Therefore, the histopathological result is important in confirming the type of tumour and can lead to the next step of management.
Sushil G. Kachewar
Full Text Available Macrodystrophia lipomatosa is a rare congenital nonhereditary mesenchymal hamartomatous malformation resulting in localized gigantism of parts of extremities that manifests clinically as macrodactyly or megalodactyly. Radiological and Pathological hallmark is the disproportionate fibroadipose tissue proliferation in subcutaneous tissue, nerve sheaths, and periosteum that leads to soft tissue and bony enlargement. As imaging is the key to early diagnosis, findings on various modalities like plain radiographs, CT scan and MRI are highlighted here.
Full Text Available Objective: Lymphangiomas are rare congenital malformations commonly seen in children. Its occurrence in adults is uncommon with very scarce reports in the literature. It mainly occurs in infants or children younger than two years of age. We report these cases to emphasize the need to consider cystic lymphangiomas in differential diagnosis of neck masses in adults.Case Reports: We describe two cases in adults, one a 27-year-old man and the other a 59-year-old male. The former presented with recurrent neck mass for 5 years, and the latter presented with asymptomatic neck mass of a short duration. Both were not associated with any pain, discoloration, discharge or sudden increase in size.Conclusion: The awareness of occurrence of cystic lymphangioma in adults is important for its proper management which includes complete surgical removal, to prevent recurrence.
Wong, Chee S.; Dupley, Leanne; Varia, Haren N.; Golka, Darek; Linn, Thu
Meckel's diverticulum is the most common congenital abnormality of the small intestine that results from incomplete closure of the vitelline (omphalo-mesenteric) duct. This true diverticulum, ~2 ft from the ileocecal valve commonly found on the anti-mesenteric border of the ileum, is benign and majority asymptomatic. Diagnosis challenges arise when it became inflamed or presented in following ways, for example, haemorrhage (caused by ectopic pepsin—and hydrochloric acid—secreting gastric mucosa), intestinal obstruction (secondary to intussusception or volvulus) or the presence of diverticulum in the hernia sac (Littre's hernia). We report a case of a 59-year-old male who was admitted under the surgical service at Blackpool Victoria Hospital with suspected appendicitis that turned out to be a Meckel's diverticulitis, a rare presentation of an acute abdomen. We discuss the issues involved in his investigation and management as well as perform a literature review comparing different surgical approaches. PMID:28064243
Full Text Available Supernumerary breast or polymastia is a well-documented anomaly of the breast and commonly presents along the embryonic milk line extending between the axilla and groin. Reported incidence of accessory breast is 0.4-6% in females. During 2 years period, we encountered only two cases out of twenty cases of axillary lumps. We present one case of fibroadenoma in ectopic breast tissue (EBT in axilla. Ectopic breast denotes breast tissue at more than two pectoral regions, which is mostly benign but at times can be malignant. EBT is at a greater risk of malignancy. Fibroadenoma of ectopic axillary breast tissue (EBT is quiet rare, but should always be kept in mind for differential diagnosis of an axillary mass.
Nv, Dravid; As, Gadre; Rp, Damle; Ps, Bagale; Kh, Suryawanshi
Papillary poroid hidradenoma is a very rare entity which belongs to poroid neoplasms, which represents 10% of sudoriferous tumours. It can be easily misdiagnosed as a malignant neoplasm. A 34-years-old male presented with an asymptomatic mass over the right chest wall, below the nipple, of 18 months duration. Clinical examination revealed tense cystic swelling which revealed hypoechoic and solid components on imaging. Clinically, a malignancy was suspected and a surgical excision was done. Histology and immunoreactivity to pan cytokeratin, epithelial membrane antigen and carcino- embryonic antigen confirmed the diagnosis. We are reporting this case because of the rarity of its presentation.
Entity – Case Report and Brief Review of Literature. Rashmi Patnayak ... weight. She gave a history of on and off fever 2 months back, which was ... remains unknown. This is an additional .... origin of pure SCC of the stomach. They include.
Walke, Vaishali Atmaram; Shende, Nitin Y; Kumbhalkar, D T
Clear Cell Sarcoma of Kidney (CCSK) is known for its morphologic diversity, aggressive behaviour, tendency to recur and metastasis to bone. Amongst the various morphologic subtypes, anaplastic CCSK is associated with worse prognosis. Here, we report a case of this rare variant of CCSK. A five-year-old boy presented with history of lump and pain in abdomen since one week. The Computed Tomography (CT) scan revealed a large mass occupying the middle and inferior pole of right kidney. The clinical impression was Wilms tumour. Nephrectomy specimen was received and the diagnosis of CCSK anaplastic variant was offered only after excluding the differentials and after performing ancillary tests such as Immunohistochemistry (IHC). Thus, this case emphasizes the diagnostic challenges on morphology and the essential role of IHC in arriving at a definitive diagnosis, because failure to do so may deprive the child from optimal treatment.
Yashavantha Kumar C
Full Text Available Introduction: Bilateral shoulder dislocation are most commonly posterior type. These are most commonly due to seizure disorder and electrocution. Anterior shoulder dislocations occurring bilaterally without any predisposing factors are very rare. These types of injuries are due to trauma with a unique mechanism of injury. To best of our knowledge there are only few cases of similar kind are reported in literature. We hereby report a interesting case of posttraumatic, bilateral anterior dislocation of shoulder without associated fracture in a 45 old women without any predisposing pathoanatomy.Case Report: A 45-year-old women presented to casualty with sudden onset of pain and restriction of movement in both shoulders fallowing trauma. Immediately post trauma she had severe pain and restriction of both shoulders. On examination arms were abducted and externally rotated. Bilateral shoulder movements were painful and restricted . There was loss of round contour of shoulder with increased vertical diameter of axilla anteriorly. Radiological examination revealed bilateral anterior dislocation of the shoulders without any associated fractures. Closed reduction done by Milch technique after intraraticular lignocaine injection. MRI of bilateral shoulder showed no pathological lesion. Both shoulders were immobilized with a shoulder immobilizer for three weeks.Conclusion: Most of the bilateral shoulder dislocations are posterior type seen in seizure disorders. Bilateral traumatic anterior shoulder dislocations are rare and are seen as a result of unique mechanism of injury. In our case patient had a fall on her elbows causing forced extension. If diagnosed and treated promptly completely normal function of the shoulders can be restored.
Chandrashekhar Anil Sohoni
Full Text Available Extra-adrenal myelolipoma is a well-described entity in adult population, however it is extremely rare in paediatric age group. An unusual case of intra-peritoneal extra-adrenal myelolipoma in an 8-year-old child is presented here. The lesion was incidentally detected while evaluating the patient for spasmodic abdominal pain. Ultrasonography followed by CT scan and MRI imaging suggested the diagnosis which was confirmed by histopathology. A non-surgical approach was adopted and there was no progression of the lesion on follow-up imaging.
Mümtaz Taner Torun
Full Text Available Background. Cysts in the larynx are rare and generally asymptomatic. However, large cysts in adults can be symptomatic. If they are symptomatic, they typically present with respiratory and feeding difficulties. They are usually benign in terms of pathology. Several surgical techniques may be used for treatment. Case Report. A 56-year-old man presented to our clinic with hoarseness. Routine laryngeal examination revealed a giant mass and the larynx could not be visualized. At magnetic resonance imaging (MRI, a cystic mass originating from the vallecula was detected. There was no pathology at the glottic level. We planned tracheotomy for the airway and endoscopic surgery for excision. The mass was excised using CO2 laser and was reported as benign. Conclusion. An asymptomatic vallecular cyst may cause difficult intubation in any operation. It may also cause respiratory or other complications. Airway management should be led by an ear, nose, and throat surgeon, since tracheotomy may be required. Endoscopic excision with CO2 laser is a good choice for treatment in elective cases. In this report, we discuss the diagnosis and treatment of a patient with an asymptomatic giant vallecular cyst.
Torun, Mümtaz Taner; Seçkin, Ender; Tuncel, Ümit; Kılıç, Caner; Özkan, Özalkan
Background. Cysts in the larynx are rare and generally asymptomatic. However, large cysts in adults can be symptomatic. If they are symptomatic, they typically present with respiratory and feeding difficulties. They are usually benign in terms of pathology. Several surgical techniques may be used for treatment. Case Report. A 56-year-old man presented to our clinic with hoarseness. Routine laryngeal examination revealed a giant mass and the larynx could not be visualized. At magnetic resonance imaging (MRI), a cystic mass originating from the vallecula was detected. There was no pathology at the glottic level. We planned tracheotomy for the airway and endoscopic surgery for excision. The mass was excised using CO2 laser and was reported as benign. Conclusion. An asymptomatic vallecular cyst may cause difficult intubation in any operation. It may also cause respiratory or other complications. Airway management should be led by an ear, nose, and throat surgeon, since tracheotomy may be required. Endoscopic excision with CO2 laser is a good choice for treatment in elective cases. In this report, we discuss the diagnosis and treatment of a patient with an asymptomatic giant vallecular cyst. PMID:26688767
Full Text Available We report of an 18-year-old male who presented with an epileptiform disorder, features of hypocalcemia, and an extrapyramidal symptom in the form of choreoathetosis. On evaluation he had idiopathic hypoparathyroidism with extensive calcifications in the extrapyramidal system of the brain; basal ganglion, as well as in the cerebral cortex and cerebellum, which is a rare entity. We report the rare presentation of a common disorder, which requires to be considered in evaluating hypoparathyroidism.
Kataria, Sant Prakash; Sharma, Jyoti; Singh, Gajender; Kumar, Sanjay; Malik, Shivani; Kumar, Vinod
Mucormycosis is the common name given to several different diseases caused by fungi in the order of mucorales. The clinical hallmark of these opportunistic pathogens in invasive mucormycosis is tissue necrosis resulting from angioinvasion and subsequent thrombosis. Rarely the disease may affect immunocompetent individuals. In addition, the breast involvement in this disease is very rare. Herein, we describe a case of primary breast mucormycosis diagnosed on fine needle aspiration cytology (FNAC), in an immunocompetent young female, which is extremely rare. Diagn. Cytopathol. 2016;44:761-763. © 2016 Wiley Periodicals, Inc.
Lazaraki, Georgia; Nakos, Andreas; Katodritou, Eirini; Pilpilidis, Ioannis; Tarpagos, Anestis; Katsos, Ioannis
Pseudoachalasia is a rare clinical entity with clinical, radiographic, and manometric features often indistinguishable from achalasia. Primary adenocarcinomas arising at the gastroesophageal junction or a tumor of the distal esophagus are the most frequent causes of pseudoachalasia. Rarely, processes other than esophagogastric cancers including chronic idiopathic intestinal pseudo-obstruction, amyloidosis, sarcoidosis, Chagas' disease, vagotomy, antireflux surgery, pancreatic pseudocysts, von Recklinghausen's neuroinomatosis, gastrointestinal stromal tumor, and other malignancies and rare genetic syndromes, may lead to the development of pseudoachalasia. Secondary achalasia is extremely rare, with less than 100 cases reported in the literature so far. Gastrointestinal manifestations in primary or secondary amyloidosis include abdominal pain, diarrhea, constipation, malabsorption, obstruction, motility disturbance, intestinal infarction, perforation, and hemorrhage; however, gastrointestinal tract involvement is asymptomatic in most instances. We present here a rare case of multiple myeloma initially presenting with dysphagia because of esophageal amyloidosis and manometric findings typical of achalasia.
PM van Schaik; MF Ernst; HA Meijer; K Bosscha
A 41-year-old man presented with a 6-mo history of changed defecation and rectal bleeding.A 3-cm polypoid tumor of the lower rectum was found at rectosigmoidoscopy,which proved to be a leiomyosarcoma upon biopsy.Dissemination studies did not show any metastases.He was underwent to an abdomino-perineal resection(APR).Histopathology of the specimen showed a melanoma (S-100 stain positive).Two years after the resection,metastases in the abdomen and right lung were found.He died one and half years later.Primary anorectal melanoma is a rare and very aggressive disorder.According to current data,one should always perform a S-100 stain when anorectal sarcoma is suspected.A positive S-100 stain suggests the turnout to be most likely a melanoma.Subsequently,thorough dissemination studies need to be performed.Depending on the outcome of the dissemination studies,a surgical resection has to be performed.Nowadays,a sphincter-saving local excision combined with adjuvant loco-regional radiotherapy should be preferred in case of small tumors.The same loco-regional control is achieved with less "loss of function" compared to non-sphincter roving surgery.Only in the case of large and obstructing tumors an abdomino-perineal resection is the treatment of choice.
Alejandra; Gordillo; Hernández; Eduardo; Dominguez-Adame; Lanuza; Auxiliadora; Cano; Matias; Rosario; Perez; Huertas; Katherine; Maria; Gallardo; Rodriguez; Purificacion; Gallinato; Perez; Fernando; Oliva; Mompean
Gangliocytic paragangliomas are rare tumors that almost exclusively occur within the second portion of the duodenum. Although these tumors generally have a benign clinical course, they have the potential to recur or metastasize to regional lymph nodes. The case report presented here describes a 57-year-old female patient with melena, progressive asthenia, anemia, and a mass in the second-third portion of the duodenum that was treated by local excision. The patient was diagnosed with a friable bleeding tumor. The histologic analysis showed that the tumor was a 4 cm gangliocytic paraganglioma without a malignant cell pattern. In the absence of local invasion or distant metastasis, endoscopic resection represents a feasible, curative therapy. Although endoscopic polypectomy is currently considered the treatment of choice, it is not recommended if the size of the tumor is > 3 cm and/or there is active or recent bleeding. Patients diagnosed with a gangliocytic paraganglioma should be closely followed-up for possible local recurrence.
Full Text Available Synovial cyst occurs secondary to traumatic, degenerative, or inflammatory conditions. Synovial cysts represent abnormal distension of bursae, which communicate with the joint. Giant synovial cysts are typically due to rheumatoid arthritis, other causes being trauma and synovial pseudoarthrosis. A 33-year-old male presented to an outpatient clinic with a massive swelling on his posterolateral aspect of right thigh extending from upper one-third to the knee joint which had been increasing in size over the past six months. This was associated with dull aching pain. All laboratory investigations were within normal parameters. Even FNAC was inconclusive. With time, swelling was increasing in size. Ultrasound revealed the cystic nature of swelling. MRI showed large cystic lesion 24 × 10 × 12 cm in posterolateral aspect of thigh extending up to knee joint. Following the MRI, an excision was planned. After excision, histological examination confirmed the synovial nature of the cyst, which had a collagenous wall and dense chronic inflammatory cells. As the disease is extremely rare and asymptomatic, precise diagnosis is difficult and often delayed. We consider that open surgical excision should be reserved for cases of large synovial cysts because it can provide a complete resection of the lesion and minimize the risk of recurrence.
Kavanagh, Crystal; Kaoutzanis, Christodoulos; Spoor, Kristen; Friedman, Paul F
Jejunal diverticulosis is a rare entity with a reported clinical incidence of 0.5%. However, symptoms relating to its presence are non-specific, which does not only delay diagnosis, but also increases the risk of serious complications approaching 15%. We report a case of perforated jejunal diverticulum presented with a 6-month history of significant weight loss and acute abdominal pain. We discuss clinical presentation in both simple and complex cases, diagnostic pitfalls and management strategies.
Pradhan, Sultan A.; Khannan, Rajan; Hazarika, Biswajyoti; Desai, Meena
Epithelial-myoepithelial carcinoma is a rare salivary gland tumor. It comprises less than 1% of all salivary gland tumors. It generally arises from the parotid gland. Unusual sites of occurrence include sinonasal tract, lung, trachea, lacrimal gland and breast. Histopathologically epithelial-myoepithelial carcinoma comprises a dual population of ductal and myoepithelial cells. We report an extremely rare case of epithelial-myoepithelial carcinoma occurring in the sinonasal tract of young man.
Full Text Available Introduction: Histoplasmosis is a rare fungal disease caused by dimorphic fungi Histoplasma capsulatum. The causative fungus is present in soil, infects through inhalation and manifests in three main types-acute primary, chronic cavitary and progressive disseminated Histoplasmosis. Disseminated Histoplasmosis (DH is defined as a clinical condition where fungus is present in more than one location. Among the forms of histoplasmosis, DH is the rarest and generally found in immune-compromised individual. Here we are presenting our experiences of the series of cases of Disseminated Histoplasmosis in immune-competent individuals who have been diagnosed in our institute in last 5 years. Materials and methods: This is a single centre retrospective observational study, from May 2009 to April 2014. Only cases with Disseminated Histoplasmosis in otherwise healthy immune-competent individuals were included in the study. The Histoplasmosis is confirmed by either presence of Histoplasma in biopsy specimen from extra-pulmonary organ or by positive growth in fungal culture Result: Total seven patients met the inclusion criteria. Five out of 7 patients were male. The mean age was 35 years. Five of the 7 patients presented with fever for long duration. Six patients complained of significant weight loss before diagnosis. On examination, one patient had skin nodules, five patients had hepato-splenomegaly, and two patients had lymphadenopathy. The laboratory investigation revealed anaemia in six out of 7 patients, and pancytopenia in 3 patients. Two patients had features of hemophagocytic syndrome in the bone marrow. All of the patient had undergone treatment with conventional amphotericine B deoxy-cholate and azole antifungal. One patient with adrenal involvement died in hospital. The patient with skin nodule had recurrent relapses. The other patients had resolution of symptoms and clinically cured. Conclusion: Disseminated Histoplasmosis is not an uncommon
Full Text Available Pleuroparenchymal fibroelastosis (PPFE is a rare entity that has been recently included in the official American Thoracic Society/European Respiratory Society (ATS/ERS statement in 2013 as a group of rare idiopathic interstitial pneumonias (IIPs. PPFE is characterized by pleural and subpleural parenchymal thickening due to elastic fiber proliferation, mainly in the upper lobes. The etiology of the disease is unclear, although some cases have been associated as a complication after bone marrow transplantation, lung transplantation (LT, chemotherapy, and recurrent respiratory infections. The patients usually report progressive dyspnea and dry cough and are predisposed to develop spontaneous or iatrogenic pneumothoraces after surgical lung biopsy (SLB for its diagnosis. That is why better awareness with the clinical and radiologic features can help optimal management by the multidisciplinary team. Novel invasive techniques such as cryobiopsy may become useful tools in these patients as it could spare SLB. We present the first reported cases in Spain.
Guasch, Ignasi; Becker, Caroline; Andreo, Felipe; Fernández-Figueras, Maria Teresa; Ramirez Ruz, José; Martinez-Barenys, Carlos; García-Reina, Samuel; Lopez de Castro, Pedro; Sansano, Irene; Villar, Ana; Ruiz-Manzano, Juan
Pleuroparenchymal fibroelastosis (PPFE) is a rare entity that has been recently included in the official American Thoracic Society/European Respiratory Society (ATS/ERS) statement in 2013 as a group of rare idiopathic interstitial pneumonias (IIPs). PPFE is characterized by pleural and subpleural parenchymal thickening due to elastic fiber proliferation, mainly in the upper lobes. The etiology of the disease is unclear, although some cases have been associated as a complication after bone marrow transplantation, lung transplantation (LT), chemotherapy, and recurrent respiratory infections. The patients usually report progressive dyspnea and dry cough and are predisposed to develop spontaneous or iatrogenic pneumothoraces after surgical lung biopsy (SLB) for its diagnosis. That is why better awareness with the clinical and radiologic features can help optimal management by the multidisciplinary team. Novel invasive techniques such as cryobiopsy may become useful tools in these patients as it could spare SLB. We present the first reported cases in Spain. PMID:26380141
Dorwal, Pranav; Sachdev, Ritesh; Pande, Amit; Jain, Dharmendra; Jha, Bhawna; Raina, Vimarsh
Hepatosplenic T-cell lymphoma is a rare haematopoietic malignancy that comprises less than 1% of Non-Hodgkin lymphomas. We are reporting a case of a 26-year-old female, who presented with pallor, weight loss, jaundice, pancytopenia and hepatosplenomegaly. The bone marrow examination showed infiltration by lymphoid cells. These cells on flow cytometric evaluation showed the phenotype of hepatosplenic T cell lymphoma. The cells were positive for CD3, CD8, CD56 and TCR γδ and negative for CD5, CD4, CD8, CD16, CD57, TCRαβ along with B cell markers. This case is reported for being a rare clinical entity and its presence in an immunocompetent female making it rarer.
Jha, Tulika; Bardhan, Jayati; Das, Bibekananda; Patra, Kajal Kumar; Dhali, Badal; Seth, Shelley
Meckel-Gruber syndrome is an inherited genetic disorder of unknown aetiology. It is an autosomal recessive condition and its incidence is as rare as 1:13,250 to 1:140,000. Some population show an increased incidence of this condition eg, Finnish and Gujarati Indians. Since the time it was first reported by Meckel in 1822 and subsequently by Gruber in 1934, only 200 cases have been reported. Here the case was diagnosed antenatally by an ultrasound and termination of the pregnancy at an early stage was done as per the wishes of the parents. This interesting and rare case of Meckel-Gruber syndrome is reported here.
Full Text Available Pityriasis versicolor is a superficial fungal infection of the skin caused by the yeast of the genus Malassezia and presents as hypo or hyper pigmented scaly macules. The most commonly affected sites include upper trunk, upper arms, neck and the abdomen. Lesions confined to the acral parts like hands and feet have rarely been reported. In this article the author reports a 40 year old male who presented with multiple hypo pigmented scaly macules confined to the acral parts (hands and wrist. The acral variant of pityriasis versicolor is considered to be a very rare clinical entity which prompted the author to report this case.
Full Text Available Cherubism is a non-neoplastic, rare, hereditary childhood disease of bone characterized by bilateral enlargement of the jaws caused by bone degradation and replacement by fibrous tissue. The affected child is reminiscent of the cherub portrayed in Renaissance art. We report the clinicopathological and radiographic features of cherubism and review of literature.
Full Text Available Reticulate acropigmentation of Kitamura (RAK is a rare, autosomal dominant disorder first described in Japan characterised by a reticulate pattern of slightly atrophic, angulated, hyperpigmented macules affecting the acral areas of the body. We hereby report a case of RAK in a young Indian male with adermatoglyphia that has not been previously reported in the literature.
Full Text Available Myxofibrosarcoma is one of the most common sarcomas in the extremities of elderly patients that rarely affect the oral cavity. In this article we describe a case of primary myxofibrosarcoma of maxilla in a 19-year-old boy. Microscopically the tumor showed loosely arranged spindle-shaped tumor cells with mild pleomorphic spindle-shaped nuclei, some with minute nucleoli in a fibromyxoid stroma. The cells were strongly immunoreactive for vimentin and negative for CD-68, S-100 protein and desmin. Based on clinical, histological and immunohistochemical findings, a final diagnosis of myxofibrosarcoma was established. It is an unusual aggressive variant that frequently recurs and metastasizes, reinforcing the importance of its correct diagnosis. We report a rare case of myxofibrosarcoma in maxilla, with emphasis on immunohistochemical findings.
Ranganathan, Kannan; Mathew, Seema Alice; Sreena, Nellimad Sreedharan; Lavanya, Nagarajan
Pleomorphic lipoma is a rare, benign, soft tissue neoplasm that characteristically occurs as a subcutaneous mass in the posterior neck or upper back and rarely in the tonsillar fossa and oral cavity. Histologically, pleomorphic lipoma contains varying amounts of mature fat, areas of spindle and pleomorphic cells, floret giant cells and thick rope - like collagen in a myxoid stroma. Pleomorphic lipoma with scanty fatty elements is called the fat free variant of pleomorphic lipoma. The combination of meagre amount of fat and presence of pleomorphic elements gives a pseudosarcomatous picture under the microscope leading to misdiagnosis and over treatment. Here, we report a case of fat free pleomorphic lipoma, first of its kind in the oral cavity and discuss the diagnostic features and differential diagnosis.
Full Text Available Cystic adrenal neoplasms are uncommon and may be incidentally discovered during radiologic procedure not directly performed for the evaluation of adrenal disease. Cystic pheochromocytomas are uncommon neoplasms that originate from the adrenal medulla, clinically differing from the more frequent solid pheochromocytomas. Here, we report a case of 72 year-old woman affected by asymptomatic pseudocystic pheochromocytoma (70x50mm, incidentally discovered, with a false negative 123I-monoiodobenzylguanidine (123I-MIBG scintigraphy. Histopathological examination led to diagnosis of pseudocystic pheochromocytoma with typical architecture. The screening for germline mutation of succinate dehydrogenase complex subunits (SDHB, SDHC, Von Hipple-Lindau syndrome (VHL and multiple endocrine neoplasia type 2 (RET genes were negative, finding only some polymorphisms for RET and SDHB. We emphasize the relevance of an accurate pre-operative diagnosis, especially in rare diseases, and the requirement of experience and multidisciplinary management in these rare pathologies.
Natashya Hilda Sima
Full Text Available Ramsay Hunt syndrome is a rare cause of facial nerve paralysis in children, caused due to reactivation of latent Varicella–Zoster virus within the geniculate ganglion. In addition to the facial nerve, Ramsay Hunt syndrome may also affect the vestibulocochlear nerve leading to inner ear dysfunction and in severe case may also involve other cranial nerves. We report a case of Ramsay Hunt syndrome in a 15-year-old child.
Full Text Available Cystic epithelial-stromal tumors of the prostate are rare, with 82 cases reported in literature. These cases have been published under a variety of diagnoses, including phyllodes tumor and prostatic stromal proliferation of uncertain malignant potential as well as a malignant tumor called “prostatic stromal sarcoma”. We report a case of a 60-year-old man with the histological diagnosis of phyllodes tumor of the prostate in transurethral resection specimen.
Andreas Bannowsky; Andreas Probst; Helmut Dunker; Tillmann Loch
Cystic epithelial-stromal tumors of the prostate are rare, with 82 cases reported in literature. These cases have been published under a variety of diagnoses, including phyllodes tumor and prostatic stromal proliferation of uncertain malignant potential as well as a malignant tumor called “prostatic stromal sarcoma”. We report a case of a 60-year-old man with the histological diagnosis of phyllodes tumor of the prostate in transurethral resection specimen.
Weidner, Anna-Sophie; Molina, David; DeSimone, Robert A; Cohen, Marc A; Giorgadze, Tamar; Scognamiglio, Theresa; Hoda, Rana S
Riedel thyroiditis is a rare fibrosing disorder characterized by extension of the fibroinflammatory process beyond the thyroid capsule. Due to the nature of this lesion, fine-needle aspiration often yields scant material and may be interpreted as non-diagnostic. In this report, we describe cytologic features that allow the cytopathologist to favor a diagnosis of Riedel thyroiditis, thereby guiding appropriate further work-up and management.
Full Text Available One of the rare anatomical variations that can be of significant importance for the dentist is the bifid mandibular canal. Many complications can occur from this condition such as failure of anesthesia when performing inferior alveolar nerve block, difficulties during the surgical extraction of the third mandibular molar, and during implants placement. Therefore, good knowledge of this condition is essential. In this report, we describe the radiographic finding of a unilateral bifid mandibular canal.
Full Text Available Congenital granular cell tumor is a rare benign neoplastic growth affecting the gingival mucosa of neonates. Prenatal ultrasound diagnosis has recently come to focus and in spite of several reports on immune-histochemical and other advanced marker studies, the cause and origin of the lesion remains debatable till date. Review of literature on prenatal diagnosis and histopathology along with immunohistochemistry is discussed.
Full Text Available Celiac disease is a gluten sensitive enteropathy that involves an abnormal immunological response to glutens in wheat, rye etc. It predominantly involves the small intestinal mucosa, though, extra luminal manifestations can also occur. One rare extraluminal manifestation is cavitatory mesenteric lymph node syndrome. It occurs in refractory celiac disease and is associated with poor prognosis due to various complications. The diagnosis is often made on imaging when cystic mesenteric lymph nodes with fat-fluid levels are seen and this can then be confirmed by histopathological examination. We recently had a typical case where we were able to make this diagnosis.
K P Bharath
Full Text Available Trauma to the deciduous teeth may have severe consequences. Pulp necrosis is the commonly observed sequel in traumatized primary teeth and is one of the possible etiologic factors for the development of dentigerous cyst. Dentigerous cyst is more commonly seen in mandibular third molar and maxillary canine region and is rarely associated with other teeth. This article addresses a case of dentigerous cyst associated with the germ of upper permanent central incisor, an uncommon site of occurrence, which was impacted, dislodged from its natural site of eruption after trauma to the predecessor incisor.
Kaliki Hymavathi Reddy
Full Text Available Ovarian cancer is the third most common neoplasm of the female genital tract. Based on the cell type of origin, primary ovarian malignancies are classified into surface epithelium, germ cell, and sex cord tumors. Sex cord tumors account for 1% to 2% of ovarian malignancies. They may contain granulosa cells, theca cells, sertoli cells, or fibroblasts of gonadal stromal origin. Granulosa Cell Tumours (GCTs account for approximately 2-5% of all ovarian tumors and can be divided into adult (95% and juvenile (5% types based on histologic findings. GCTs secrete estrogen thus resulting in menstrual irregularities in the affected individual. More serious estrogen effects can occur in various end organs such as uterus resulting in endometrial hyperplasia, endometrial adenocarcinomas and increased risk of breast cancers. Androgen production is also reported but rare and produces virilization in the affected women. Juvenile Granulosa Cell Tumours (JGCTs are clinically and histopathologically distinct from the GCTs. They are rarely encountered but mostly in youngsters. Surgery is the primary modality of treatment with chemotherapy being reserved for advanced or recurrent disease states. We herewith report an interesting case of JGCT in a young teenage girl. [Int J Reprod Contracept Obstet Gynecol 2014; 3(4.000: 1150-1154
Nafiseh Sadat Nabavi
Full Text Available Xanthelasmoid or pseudoxanthomatous mastocytosis is an extremely rare variant of diffuse cutaneous mastocytosis. Herein, we describe an adult male with cutaneous mastocytosis showing multiple widespread yellowish ovoid papules like eruptive xanthoma. A 60-year-old male visited our outpatient clinic with a 1-year history of generalized yellowish, ovoid, and skin color papular eruption located on the trunk, groin, extremities, with the modest pruritus. Vital signs were stable, and Darier′s sign was negative. No other subjective and objective signs were detected during the examination. No abnormality was detected in his diagnostic laboratory tests. Skin biopsy was taken, and histopathologic examination revealed proliferation of mast cells with ovoid and spindle nuclei with distinct cytoplasm borders around the capillaries, which was compatible with mastocytosis. Antihistamine was prescribed for pruritus control which was successful, but eruptions were persistent, and even 1-year phototherapy was not useful.
Mohammad Asif Kiresur
Full Text Available The keratocystic odontogenic tumor (KCOT is a frequently encountered developmental cyst of the jaws. The occurrence of KCOT in the maxillary sinus is rare. The mucosa of the maxillary sinus is susceptible to infections, allergic diseases, and neoplasm. The anatomic position of maxillary premolar and molar teeth is in close contact with the sinus predispose to spreading of pulp and periodontal infection, odontogenic cyst, and tumors to the sinus. Diagnosis and treating KCOT in maxillary sinus is challenging as treatment has to be rendered for sinusitis because of pathology in the sinus and for KCOT. We report a case of 35-year-old female with KCOT involving the lining of the maxillary sinus and put forward hypotheses for the origin of KCOT in the maxillary sinus.
Lim, Sue Zann; Ong, Kong Wee; Tan, Benita Kiat Tee; Selvarajan, Sathiyamoorthy; Tan, Puay Hoon
Breast sarcoma is a rare condition. It consists of a heterogeneous group of non-epithelial tumours arising from the mesenchymal tissue of the breast. It has a distinctly different natural history, treatment response and prognosis as compared with carcinoma of the breast. A different diagnostic approach and treatment strategy have to be defined for this group of tumours. Due to its rarity, the current understanding on breast sarcoma is limited and is mostly based on small retrospective case series or case reports. Hence, the management generally follows the algorithms derived from randomised control trials of soft tissue sarcomas in the extremities and chest wall. Through this review, we discuss the results of major retrospective studies on breast sarcomas including data on epidemiology, aetiology, diagnostic approach, treatment strategies and outcomes of this challenging and potentially aggressive condition.
Cavalli, Tiziana; Giudici, Francesco; Nesi, Gabriella; Amorosi, Andrea; Santi, Raffaella; Brandi, Maria Luisa; Tonelli, Francesco
Approximately 300 cases of sporadic parathyroid cyst (PCs) have been reported to date. Only two cases have been described in MEN1 so far. Detection by imaging could be challenging, especially in multiglandular primary hyperparathyroidism (HPT) and clinical outcome could be different. During the period 1990-2014, 71 MEN1 patients were operated for primary hyperparathyroidism in our centre. We report three cases of PCs in MEN1 patients affected by HPT, who underwent a total or subtotal parathyroidectomy with transcervical thymectomy. In our series, all three patients had an unsatisfactory postoperative course, at variance with the high percentage (over 90 %) of long-term success in MEN1 patients operated at our centre. One patient affected by cystic degeneration of all the four parathyroid glands reported persistent hypoparathyroidism, despite autografts of parathyroid tissue. For the other two cases, surgery failed to cure hyperparathyroidism, perhaps because of the presence of undetected ectopic parathyroid tissue. In the context of a multiglandular disease such as MEN1 syndrome, PCs seem rare but our experience shows about a 4 % incidence. Furthermore their presence, even in expert hands, could affect the preoperative identification of the parathyroid glands due to the difficult differential diagnosis between PC and other cystic lesions of the neck, and intraoperative detection of the glands as well as the postoperative outcome.
Khanduri, Sachin; Chhabra, Saakshi; Raja, Anshul; Bhagat, Saurav
Twin reversed arterial perfusion (TRAP) sequence is an extremely rare complication of monochorionic multi-fetal pregnancy, occurring once in 35,000 births. This condition is characterized by a malformed fetus without a cardiac pump being perfused by a structurally normal (pump) twin via an artery-to-artery anastomosis in a reverse direction. We report a case of a primigravida, who came for routine antenatal checkup to our hospital at 31 weeks gestational age. Ultrasound imaging and magnetic resonance imaging revealed twin monochorionic intrauterine pregnancy with a viable, normal-appearing first twin and amorphous structured second twin connected by umbilical vessels. The patient was monitored with weekly ultrasonography, echocardiography, and Doppler ultrasound examination to ascertain the well-being of the pump twin. She delivered successfully at term a normal live baby and an acardius acephalus fetus. Plain X-ray of the acardius acephalus fetus confirmed the absence of cephalic structures. The perinatal mortality of the pump twin ranges from 35 to 55%; hence, it is essential to diagnose the presence of a pump twin at an early gestational age through improved imaging techniques, so that intervention can be planned early in the pregnancy for a better outcome of the pump twin. PMID:25861543
Priya Shirish Joshi
Full Text Available Ameloblastomas are one of the common odontogenic tumors of the oral cavity. They usually present with unilocular or multilocular radiolucency associated with or without impacted tooth. Among the variants of ameloblastomas, desmoplastic ameloblastoma (DA casts a mixed radiolucent/radiopaque shadow on X-ray and radiograph seldom suggests a diagnosis of ameloblastoma. DA is a rare entity that exhibits important differences in anatomic distribution, histologic appearance, radiographic findings when compared to other variants of ameloblastoma. This fact has been considered by World Health Organization (WHO and now it has been included as a separate entity in WHO classification (2005 of odontogenic tumors. About 145 cases of DA have been reported worldwide, but cases reported in India are very few. We report a case of a hybrid variety of DA in a female patient in the anterior maxillary region between 12 and 13 as painless hard swelling, showing mixed radiolucent/radiopaque shadow on radiographic examination. Histopathology revealed odontogenic epithelium in the form of follicles, typical of solid/multicystic ameloblastoma, with cystic degeneration and squamous metaplasia at places and elsewhere there were odontogenic islands compressed by dense fibrocellular stroma suggestive of desmoplasia. Also seen was osseous metaplasia. We have also done a detailed review of literature concerning the presentation of DA.
Full Text Available Abstract We present the case of a 20-year-old male who underwent successful surgical correction of pectus excavatum with the Highly Modified Ravitch Repair (HMRR. At 29 months the attempted operative removal of the Ravitch bar was unsuccessful despite the impression of adequate bar location on chest x-ray. Subsequent imaging with computed tomography was unclear in determining whether the bar was supra or infra-diaphragmatic due to the tissue distortion subsequent to initial surgery. Video assisted thoracoscopic surgery (VATS successfully retrieved the bar and revealed that it was not in the thorax, but had migrated to the intra-abdominal bare area of the liver, with no evidence of associated diaphragmatic defect or hernia. Intra-abdominal pectus bar migration is a rare clinical entity, and safe removal can be facilitated by the use of the VATS technique.
Full Text Available Eruptive collagenomas are non familial connective tissue nevi of unknown etiology presented with an abrupt onset. While most cases are reported in young adults, there is a paucity of literature in children. We report a case of a 4-year-old girl, who presented with multiple asymptomatic, papules, plaques and nodules on the face, trunk and upper extremities with no systemic involvement. Histopathologically, the lesion showed thickened homogenized collagen fibres highlighted by Masson′s trichrome stain and paucity in elastic fibres by Verhoeff-van Gieson stain, confirming the diagnosis of eruptive collagenoma.
Ghulam Rehman Mohyuddin
Full Text Available A 70-year-old female presented with a 4-week history of dry cough and wheezing. Chest radiograph showed a 10.5 cm mass-like density in the anterior mediastinum which had not been previously visualized. Computed tomography scan (CT of the chest showed a right hilar mass encasing and narrowing right upper lobe bronchus and right mainstem bronchus and secondary atelectatic changes. Biopsy was consistent with a diagnosis of lymphomatoid granulomatosis Grade 3. She responded well clinically and radiologically to therapy. Lymphomatoid granulomatosis is a rare EBV-associated disorder which is considered a lymphoproliferative disease. The most common radiographic feature is multiple lung nodules. An isolated hilar mass is an exceptionally rare presentation of this rare disease.
Cho Ee Ng
Conclusion: There should be high clinical suspicion of bowel metastasis in patients presenting with positive faecal occult blood with or without bowel symptoms even if the incidence is less <1% of metastases, particularly in cases where the initial breast tumour was large, with positive axillary nodes.
Kordzadeh, Ali; Lorenzi, Bruno; Kalyan, Jiten P.; Hanif, Muhammad A.; Charalabopoulos, Alexandros
Paraumbilical hernia sac usually contains omentum, bowel loop and rarely appendicular epiploicae, metastatic deposits and vermiform appendix. Presentation of acute appendicitis in a paraumbilical hernia is rare and limited to few case reports in the literature. Herein, we would like to report a case of a successfully treated acute appendicitis presenting in a paraumbilical hernia in an 84-year-old lady with 6-month follow-up. PMID:28096326
Ryan, Russell J H; Akin, Cem; Castells, Mariana; Wills, Marcia; Selig, Martin K; Nielsen, G Petur; Ferry, Judith A; Hornick, Jason L
Mast cell sarcoma is a rare, aggressive neoplasm composed of cytologically malignant mast cells presenting as a solitary mass. Previous descriptions of mast cell sarcoma have been limited to single case reports, and the pathologic features of this entity are not well known. Here, we report three new cases of mast cell sarcoma and review previously reported cases. Mast cell sarcoma has a characteristic morphology of medium-sized to large epithelioid cells, including bizarre multinucleated cells, and does not closely resemble either normal mast cells or the spindle cells of systemic mastocytosis. One of our three cases arose in a patient with a remote history of infantile cutaneous mastocytosis, an association also noted in one previous case report. None of our three cases were correctly diagnosed as mast cell neoplasms on initial pathological evaluation, suggesting that this entity may be under-recognized. Molecular testing of mast cell sarcoma has not thus far detected the imatinib-resistant KIT D816V mutation, suggesting that recognition of these cases may facilitate specific targeted therapy.
Kaustubh B. Harshey
Full Text Available A 31-year-old Indian male presented with floaters and diminution of vision in the right eye. Ocular examination showed features of old anterior uveitis with posterior subcapsular cataract and fine, refractile crystals in the vitreous cavity and on the retinal surface. A thorough workup for all known causes of crystalline retinopathy was inconclusive. Unilateral crystalline retinopathy has been sparingly reported. This is the first report of unilateral, crystalline vitreoretinopathy in the absence of any demonstrable and known cause for intraocular crystals.
Full Text Available First rib fractures are scarce due to their well-protected anatomic locations. Bilateral first rib fractures accompanying bilateral scapular fractures are very rare, although they may be together with scapular and clavicular fractures. According to our knowledge, no case of bilateral first rib fractures accompanying bilateral scapular fractures has been reported, so we herein discussed the diagnosis, treatment, and complications of bone fractures due to thoracic trauma in bias of this rare entity.
Arnaoutakis, Demetri; Naseri, Iman
Objectives Oncogenic osteomalacia is a paraneoplastic syndrome in which the tumor secretes a peptide-like hormone, fibroblast growth factor, resulting in urinary loss of phosphates. Methods We present the case of a 50-year-old woman with a benign phosphaturic mesenchymal tumor (PMT) involving the ethmoid sinus with obstruction of the ostiomeatal complex causing unilateral nasal airway obstruction. Results The tumor was initially thought to be an esthesioneuroblastoma based on primary pathology interpretation and on clinical and radiographic appearance. However, a benign PMT was later confirmed by further testing. Conclusion The tumor was removed entirely by the endoscopic transnasal approach, leading to a full resolution of symptoms. PMID:26623233
Maheshwari, Anshul; Tiwari, Siddhi; Mathur, Deepak K; Bhargava, Puneet
Lupus vulgaris is the most common presentation of cutaneous tuberculosis in India and can present as papular, nodular, plaque, ulcerative, vegetating, and tumid forms. Unusual variants include the frambesiform, gangrenous, ulcerovegetating, lichen simplex chronicus, myxomatous, and sporotrichoid types. We describe a rare sporotrichoid presentation of lupus vulgaris on the leg of a 28-year-old female of 12 years duration.
Damato, Angela; Pusceddu, Sara; Milione, Massimo; Mazzaferro, Vincenzo; Magli, Michelle; Seregni, Ettore; De Braud, Filippo; Buzzoni, Roberto
The incidence of neuroendocrine tumors is rising, and this rise is explained by more than just better diagnostic procedures. About 85% of these neoplasms arise in gastrointestinal or pulmonary sites, but cases where the location is more unusual also occur in clinical practice. The tailgut cyst is a rare entity well described in the medical literature, but a neuroendocrine tumor within such a cyst is a very rare event, with about 30 cases described in the literature to date. In this report we present the case of a young woman with this unusual diagnosis. The characteristics of the case differ from most previous case reports in a few respects: the patient was a young rather than middle-aged female; she had a presacral mass with a significant solid component; at diagnosis, there was evidence of a lytic lesion in the coccyx. Despite this particular medical presentation, radical surgery was accomplished. In this disease the greatest risk is local relapse, but adjuvant radiotherapy may compromise the patient's fertility. We therefore opted for strict control only, but this decision might be debatable.
Carr, S P; MacNamara, F T; Muhammed, K M; Boyle, E; McHugh, S M; Naughton, P; Leahy, A
Introduction. Gallstone ileus (GSI) of the colon is an extremely rare entity with potentially serious complications including perforation. Case Presentation. An 88-year-old man presented to the emergency department with abdominal pain and distension. Clinical exam revealed signs of peritonism. Computed tomography (CT) revealed GSI of the transverse colon with a closed-loop large bowel obstruction (LBO) and caecal perforation. The patient underwent emergency laparotomy. A right hemicolectomy was performed, the gallstone was removed, and a primary bowel anastomosis was undertaken. A Foley catheter was sutured into the residual gallbladder bed to create a controlled biliary fistula. The patient recovered well postoperatively with no complications. He was discharged home with the Foley catheter in situ. Discussion. Gallstone ileus is a difficult diagnosis both clinically and radiologically with only 50% of cases being diagnosed preoperatively. Most commonly it is associated with impaction at the ileocaecal valve and small bowel obstruction. Gallstone ileus should also be considered as a rare but potential cause of LBO. This is the first reported case of caecal perforation secondary to gallstone ileus of the transverse colon. Successful operative management consisted of a one-stage procedure with right hemicolectomy and formation of a controlled biliary fistula.
S. P. Carr
Full Text Available Introduction. Gallstone ileus (GSI of the colon is an extremely rare entity with potentially serious complications including perforation. Case Presentation. An 88-year-old man presented to the emergency department with abdominal pain and distension. Clinical exam revealed signs of peritonism. Computed tomography (CT revealed GSI of the transverse colon with a closed-loop large bowel obstruction (LBO and caecal perforation. The patient underwent emergency laparotomy. A right hemicolectomy was performed, the gallstone was removed, and a primary bowel anastomosis was undertaken. A Foley catheter was sutured into the residual gallbladder bed to create a controlled biliary fistula. The patient recovered well postoperatively with no complications. He was discharged home with the Foley catheter in situ. Discussion. Gallstone ileus is a difficult diagnosis both clinically and radiologically with only 50% of cases being diagnosed preoperatively. Most commonly it is associated with impaction at the ileocaecal valve and small bowel obstruction. Gallstone ileus should also be considered as a rare but potential cause of LBO. This is the first reported case of caecal perforation secondary to gallstone ileus of the transverse colon. Successful operative management consisted of a one-stage procedure with right hemicolectomy and formation of a controlled biliary fistula.
Full Text Available Abstract Introduction Schwannoma is a rare tumor among pancreatic neoplasms. Schwannomas vary in size, and most of them are cystic, mimicking pancreatic cystic lesions. Generally, a definitive diagnosis is made at the time of histological analysis. The mainstay treatment is surgical resection. Case presentation We report an unusual presentation of pancreatic schwannoma with abdominal pain and several episodes of cholangitis in a 54-year-old Caucasian (Iranian man. The condition was not diagnosed pre-operatively and Whipple's procedure was performed. Conclusion Pancreatic schwannoma is an important clinical entity to include in the differential diagnosis of pancreatic lesions. Pre-operative diagnosis is difficult but computed tomographic findings may be helpful. The tumor may also have atypical and rare presentations, such as cholangitis and weight loss. For benign tumors, simple enucleation is usually adequate, whereas malignant tumors require standard oncological resection.
Full Text Available The odontoameloblastoma (OA, also known as ameloblastic odontoma, is a very rare odontogenic tumor that is similar to ameloblastoma in its locally aggressive behavior. OA includes odontogenic ectomesenchyme in addition to odontogenic epithelium that resembles an ameloblastoma both in structure and in behavior. Its clinical presentation, however, often mimics the more innocuous odontoma, and hence, the recognition of its aggressive nature is commonly only ascertained after its histopathologic diagnosis following enucleation. This paper presents a rare case of OA with unusual clinical and radiological features.
Full Text Available DiGeorge syndrome is a congenital genetic disorder that affects the endocrine system, mainly the thymus and parathyroid glands. The syndrome produces different symptoms, which vary in severity and character between patients. It manifests with craniofacial dysmorphism and defects in the heart, parathyroid, and thymus. Patients can present with a palatal deformity and nasal speech. This rare entity is caused mainly due to deletion of chromosome 22q11.2. Radiographic evaluation of DiGeorge syndrome is necessary to define aberrant anatomy, evaluate central nervous system, craniofacial abnormalities, musculoskeletal system, and cardiothoracic contents. It also helps in planning surgical procedures and surgical reconstructions. We report a case of DiGeorge syndrome in a 4-month-old neonate and discuss the clinical, imaging, and cytogenetic findings that helped in the diagnosis of this rare entity.
Full Text Available Pneumothorax due to mycetoma is extremely rare and has been described only in patients undergoing intensive cytotoxic therapy for hematologic malignancies. A non-immunocompromised subject presenting with pneumothorax due to rupture of the mycetoma into the pleural cavity is being described here.
Full Text Available Congenital anomalies of the urogenital tract are the most common anomalies found in the foetus, neonates and infants, but anterior urethral valves and diverticula are rare. Here, we present a case with congenital anterior urethral diverticulum associated with patent ductus arteriosus and polydactyly.
Full Text Available A dilated odontoma is an extremely rare developmental anomaly represented as a dilatation of the crown and root as a consequence of a deep, enamel-lined invagination and is considered a severe variant of dens invaginatus. An oval shape of the tooth lacking morphological characteristics of a crown or root implies that the invagination happened in the initial stages of morphodifferentiation. Spontaneous eruption of an odontoma is a rare occurrence and the occurrence of a dilated odontoma in a supernumerary tooth is even rarer with only a few case reports documented in the English literature. We present an extremely rare case of erupted dilated odontoma occurring in the supernumerary tooth in anterior maxillary region in an 18-year-old male, which, to the best of our knowledge, is the first ever case reported in English literature.
Sharma, Gaurav; Nagra, Amritpreet; Singh, Gurkeerat; Nagpal, Archna; Soin, Atul; Bhardwaj, Vishal
A dilated odontoma is an extremely rare developmental anomaly represented as a dilatation of the crown and root as a consequence of a deep, enamel-lined invagination and is considered a severe variant of dens invaginatus. An oval shape of the tooth lacking morphological characteristics of a crown or root implies that the invagination happened in the initial stages of morphodifferentiation. Spontaneous eruption of an odontoma is a rare occurrence and the occurrence of a dilated odontoma in a supernumerary tooth is even rarer with only a few case reports documented in the English literature. We present an extremely rare case of erupted dilated odontoma occurring in the supernumerary tooth in anterior maxillary region in an 18-year-old male, which, to the best of our knowledge, is the first ever case reported in English literature. PMID:26989523
Sharma, Gaurav; Nagra, Amritpreet; Singh, Gurkeerat; Nagpal, Archna; Soin, Atul; Bhardwaj, Vishal
A dilated odontoma is an extremely rare developmental anomaly represented as a dilatation of the crown and root as a consequence of a deep, enamel-lined invagination and is considered a severe variant of dens invaginatus. An oval shape of the tooth lacking morphological characteristics of a crown or root implies that the invagination happened in the initial stages of morphodifferentiation. Spontaneous eruption of an odontoma is a rare occurrence and the occurrence of a dilated odontoma in a supernumerary tooth is even rarer with only a few case reports documented in the English literature. We present an extremely rare case of erupted dilated odontoma occurring in the supernumerary tooth in anterior maxillary region in an 18-year-old male, which, to the best of our knowledge, is the first ever case reported in English literature.
Saxena, Chitrapriya; Aggarwal, Pooja; Wadhwan, Vijay; Bansal, Vishal
Squamous cell carcinoma (SCC) arising from the wall of an odontogenic cyst (also known as primary intraosseous carcinoma) is a rare tumor which occurs only in jaw bones. This tumor was first described by Loos in 1913 as a central epidermoid carcinoma of the jaw. Primary intraosseous carcinomas (PIOC) may theoretically arise from the lining of an odontogenic cyst or de novo from presumed odontogenic cell rests. According to the new histological classification of tumors of the World Health Organization, odontogenic keratocyst is nowadays considered a specific odontogenic tumor and the PIOC derived from it is considered as a specific entity which is different from other PIOCs derived from the odontogenic cysts. The following report describes a case of such extremely rare entity that is primary intraosseous SCC of the mandible derived from an OKC in a 60-year-old male patient with brief review of literature.
Singh Sneh; Jain Promil; Aggarwal Garima; Dhiman Pratibha; Singh Sunita; Sen Rajeev
Hepatic tuberculosis particularly in the absence of military tuberculosis is rare. It can occur as a primary case or due to reactivation of an old tubercular focus. We report case of a 24 year old married female who died of primary hepatic tuberculosis. She had no evidence of tuberculosis elsewhere. Appropriate treatment initiated early can result in marked recovery whereas failure to recognize this entity can prove to be fatal.
Subramanian Ilanchezhian, MBBS, MD
Full Text Available Ovarian hyperstimulation syndrome is a disorder associated with ovulation induction and is rarely seen in pregnant women. Very few cases of spontaneous ovarian hyperstimulation syndrome have been reported in a woman who is neither pregnant nor undergoing ovulation therapy. This case report describes how spontaneous ovarian hyperstimulation syndrome is associated with primary hypothyroidism in a 25-year-old, nonpregnant woman who is not on ovulation induction therapy. Imaging and laboratory findings confirmed the diagnosis of this rare entity, which aided the clinicians in providing prompt management and in preventing further complications.
Full Text Available Small cell carcinoma of the bladder is a rare, aggressive, poorly differentiated neuroendocrine neoplasm accounting for only 0.3-0.7% of all bladder tumors. Since the tumor is very rare, pathogenesis is uncertain. Small cell carcinomas of the urinary bladder are mixed with classic urothelial carcinomas or adenocarcinomas of the bladder in 68% cases, making pure primary small cell carcinoma even a rarer entity. The unknown etiology and natural history of small cell carcinoma of the urinary bladder represent a challenge both to the pathologist and urologists for its diagnosis and treatment, respectively.
Shoib, Sheikh; Dar, Mohamand Maqbool; Arif, Tasleem; Bashir, Haamid; Bhat, Mohammad Hayat; Ahmed, Javid
Sheehan's syndrome (SS) refers to the occurrence of varying degree of hypopituitarism after parturition (1). It is a rare cause of hypopituitarism in developed countries owing to advances in obstetric care and its frequency is decreasing worldwide. However, it is still frequent in underdeveloped and developing countries. Sheehan's syndrome is often diagnosed late as it evolves slowly (2,3). Reports of psychoses in patients with Sheehan's syndrome are rare. Herein, a case report of psychosis in a 31 year old woman who developed Sheehan's syndrome preceded by postpartum haemorrhage is presented. Treatment with thyroxine and glucocorticoids resulted in complete remission after attaining euthyroid and eucortisolemic state.
Sriram Bhat M
Full Text Available Free peritoneal perforation is a rare complication of Crohn's disease with a report of only 100 cases in the literature. It needs an emergency exploration and an unaware general surgeon is confounded in intraoperative decision-making. We present our experience when this rarity struck us in a district hospital and briefly review the guidelines of optimal management of this complication of Crohn's disease.
Singh Sunita; Chhabra Sonia; Aggarwal Garima; Kalra Rajnish; Duhan Amrita; Sen Rajeev
Cysticercosis is a condition that occurs when man is infected with larvae ofTaenia solium. Oral cysticercosis is a rare event, and it represents difficulty in clinical diagnosis. A case of oral cysticercosis in 11 year old girl is presented which complained of painless swelling for 6 months. A FNAC was performed which revealed bluish pink fibrillary material and interspersed nuclei and fragments of wall of larvae. Patient was treated with antihelminthic.
Subramani, Vijayanirmala; Narasimhan, Malathi; Ramalingam, Suganya; Anandan, Soumya; Ranganathan, Subhashini
Cementoblastoma is a rare benign odontogenic neoplasm which is characterized by the proliferation of cellular cementum. Diagnosis of cementoblastoma is challenging because of its protracted clinical, radiographic features, and bland histological appearance; most often cementoblastoma is often confused with other cementum and bone originated lesions. The aim of this article is to overview/revisit, approach the diagnosis of cementoblastoma, and also present a unique radiographic appearance of a cementoblastoma lesion associated with an impacted tooth.
Full Text Available Cementoblastoma is a rare benign odontogenic neoplasm which is characterized by the proliferation of cellular cementum. Diagnosis of cementoblastoma is challenging because of its protracted clinical, radiographic features, and bland histological appearance; most often cementoblastoma is often confused with other cementum and bone originated lesions. The aim of this article is to overview/revisit, approach the diagnosis of cementoblastoma, and also present a unique radiographic appearance of a cementoblastoma lesion associated with an impacted tooth.
Ali Murat Ceyhan
Full Text Available PFAPA syndrome is a recently identified clinical entity of unknown etiology characterized by recurrent episodes of abrupt onset of high fever, aphthous stomatitis, pharyngitis and cervical lymphadenopathy. The associated symptoms do not regress with antibiotic therapy. The dramatic response of symptoms to single dose corticosteroids is hallmark feature of this syndrome. PFAPA syndrome usually begins under 5 years of age and in most cases resolves spontaneously until ten years old. PFAPA syndrome is extremely rare in adults, and to our knowledge, only about 20 cases have been described to date. Although PFAPA syndrome is increasingly reported in the pediatric literature, and is a known condition for the pediatricians, most dermatologists are unfamiliar with this entity. In this report, we describe a late onset of PFAPA syndrome in a 25-year-old boy who presented to our clinic for his recurrent aphthous stomatitis and fever.
Full Text Available Blastomycosis is a chronic granulomatous and suppurative mycosis, caused by Blastomyces dermatitidis, which in the great majority of cases presents as a primary pulmonary disease. Primary cutaneous blastomycosis is very rare. We present a 57-year-old female patient with a solitary, slowly progressive nodule over upper lip of 2½ months duration. Initially, differential diagnosis of cutaneous leishmaniasis, pyoderma and deep mycoses were entertained. Slit smear preparation was suspicious of deep mycotic infection which was subsequently confirmed by biopsy and culture.
Full Text Available Omental cysts are rare entity with a prevalence of 1: 1,000,000 in adults and in 1: 20, 000 in children. We are reporting a case of a 30 year female patient with abdominal lump over epigastrium and left hypochondrium for 6 months; diagnosed on laparotomy as uniloculated omental cyst originating from lesser omentum. Omental cyst is a challenging diagnostic entity with varied presentations and a wide range of differential diagnosis has to be kept in mind.
Chaudhry, Ikram Ulhaq; Cheema, Ahsan Iqbal; AlShamasi, Zahra; Mutairi, Hadi
Primary posterior mediastinal ectopic goitre is an extremely rare entity; we report a case of a 28-year-old man who presented with dysphagia, respiratory distress and hoarseness of voice, gradually worsening over a period of 3 months. CT scan of the thorax revealed a giant posterior mediastinal ectopic goitre. The mass was removed through a right posterolateral thoracotomy. The patient's symptoms, respiratory distress and dysphagia disappeared immediately after surgery while his voice gradually returned to normal after 6 weeks.
Shergill Harbhajan K, Grover Suparna, Chhabra Ajay
Full Text Available It is a rare occurrence for the rudimentary horn of uterus to harbour a pregnancy and the usual outcome is devastating leading to a spontaneous rupture in second trimester with the patient presenting in shock with massive intra-peritoneal haemorrhage and if appropriate management is not instituted in time it may lead to high rate of mortality. We report an unusual case of rupture rudimentary horn pregnancy who presented as a chronic ectopic with an adnexal mass and surprisingly with no sign of shock. Diagnosis is often difficult in such a situation which puts the treating gynaecologist in dilemma. High clinical suspicion supplemented with radiological findings helped clinch the diagnosis and laparotomy was performed followed by resection of the rudimentary horn to prevent future complications.
Full Text Available Abstract Background Mixed epithelial and stromal tumour (MEST represents a recently described benign composite neoplasm of the kidney, which predominantly affects perimenopausal females. Most tumours are benign, although rare malignant cases have been observed. Case report A 47-year-old postmenopausal female presented to the urologist with flank pain. A CT scan of the abdomen showed a 30-mm-in-diameter uniform mass adjacent to the pelvis of the left kidney. Surgical exploration showed a tumour arising from the lower anterior hilus of the left kidney. The tumour could be excised by preserving the kidney. By intraoperative frozen section the tumour showed characteristic features of MEST with epithelial-covered cysts embedded in an "ovarian-like" stroma. Additional immunohistochemistry investigations showed expression for hormone receptors by the stromal component of the tumour. Discussion MEST typically presents in perimenopausal women as a primarily cystic mass. Commonly, the tumour arises from the renal parenchyma or pelvis. The tumour is composed of an admixture of cystic and sometimes more solid areas. The stromal cells typically demonstrate an ovarian-type stroma showing expression for the estrogen and progesterone receptors. Conclusion MEST represents a distinctive benign tumour entity of the kidney, which affects perimenopausal woman. The tumour should be distinguished from other cystic renal neoplasms. By imaging studies it is difficult to distinguish between a benign or malignant nature of the tumour. Thus, intraoperative frozen section is necessary for conservative surgery, since the overall prognosis is favourable and renal function can be preserved in most cases.
Desai, Kiran; Gupta, Kavita; Manjunatha, B S; Palan, Soniya
Central ossifying fibroma is a benign neoplasm, having slow growing nature. Some rare lesions show very aggressive nature, multifocal appearance and reach up to a very massive size. So, these kinds of cases require special attention for their treatment. A unique case of central ossifying fibroma with aggressive nature, multifocal appearance is reported. This case shows growth both in maxilla and mandible with maxillary lesion massive in size involving maxillary sinus. There have not been any such cases reported so far in the literature showing bimaxillary growth of ossifying fibroma. PMID:23774709
Ghosh, Prithwijit; Saha, Kaushik; Bhowmik, Sourav
Sarcoma-like mural nodule (SLMN) is a very uncommon and misleading benign entity which may be associated with benign, borderline or malignant mucinous neoplasm of the ovary. It should be distinguished from other malignant mural nodules with sarcoma, carcinosarcoma or anaplastic carcinoma for proper management. We report a rare case of SLMN in a borderline mucinous tumor of the ovary in a 30-year-old lady. In spite of having confusing histopathological features the final diagnosis was made depending on the younger age of the patient, well circumscription of the nodule, absence of vascular invasion and immunohistochemical profile.
Sathyakrishna, Basavapatna Ramakrishnaiah; Boggaram, Suryanarayan Giridhar; Jannu, Nagamalleswara Rao
Right iliac fossa (RIF) pain is the most common presenting complaint in the general surgical ward. Though there are several causes for the RIF pain, acute appendicitis is the first and common diagnosis. Here we present one of the rare differential diagnoses of RIF pain in a young female patient with one month history of intermittent right lower abdominal pain. Patient's symptoms and clinical findings indicated acute appendicitis, though ultrasonography didn't show any significant abnormality. Through diagnostic laparoscopy we found that there was torsion of anterior abdominal wall parietal peritoneal pedunculated lipoma located in RIF region. The clinical presentation, examination, investigation, laparoscopic findings and further management is presented in this case report.
Full Text Available Spontaneous pneumomediastinum is an unusual and benign condition in which air is present in mediastinum. A 20-year-old male patient presented to ED with complaint of hoarseness and odynophagia from the day before, after weightlifting. The patient was nonsmoker and denied history of other diseases. On physical examination he had no dyspnea with normal vital signs. Throat examination and pulmonary auscultation were normal and no crepitation was palpable. We could not find subcutaneous emphysema in neck and chest examination. In neck and chest X-ray we found that air is present around the trachea. There was no apparent pneumothorax in CXR. In cervical and chest CT free air was present around trachea and in mediastinum. Subcutaneous emphysema was also evident. But there was no pneumothorax. The patient was admitted and went under close observation, oxygen therapy, and analgesic. The pneumomediastinum and subcutaneous emphysema gradually resolved within a week by conservative therapy and he was discharged without any complication. Many different conditions could be trigged because of pneumomediastinum but it is rarely seen in intense physical exertion such as weightlifting and bodybuilding. Two most common symptoms are retrosternal chest pain and dyspnea. But the patient here complained of hoarseness and odynophagia.
A Winnifred Christy
Lipoma occurring in the palate is rare with only six cases reported in the literature so far. This case of oral lipoma occurring in the hard plate is reported for its rarity and can be considered as a rare possibility in the differential diagnosis of various swellings of the palate.
Peripheral Primitive Neuroectodermal Tumors (PNETs) are rare lesions that arise from outside the central nervous system and normally do not affect the genitourinary system. Primary renal presentations are extremely rare but given their aggressive behavior and characteristic cytomorphologic and genetic features should be considered well-defined distinct clinical entities in order to distinguish them from other primary tumors featuring round cells in the kidney. We report one case of PNET invol...
Full Text Available Polypoid melanoma represents a distinct variant of melanoma characterized by an ulcerated exophytic nodule. Although this melanocytic tumor is usually restricted to the papillary dermis, it presents with a thick Breslow level and aggressive course. Its rapidly growing vertical phase and its amelanotic nature frequently simulate benign or non-aggressive entities; leading to a delay in biopsy and resulting in an increased risk of metastasis at the time of the diagnosis.
Full Text Available The purpose of research refers to comparison of Ukrainian and the USA standards of preparation and presentation of governmental entities financial reports with the aim of implementation of foreign experience regarding financial data disclosure of a governmental entity activity in domestic reporting system of budgetary resources disposers. In order to achieve the aim the author applied the following general methods of cognition: induction, deduction, analogy, comparison, and historical one. Special economic methods include grouping, tabular, graphical, information and logical analysis. The research resulted in detailed analysis of the standards of the USA governmental entities financial reports presentation and comparison of their statements with domestic governmental accounting standards. As a result of performed research, the key differences in the Ukrainian and the USA rules of presentation of governmental entities financial reports are considered. The author determined the dependence of structure and a public level of the financial reports on the character of the state political and economic systems.
Ashima Datey Chakrabarty
Full Text Available The common causes of stridor in adults are abscesses or swelling of upper airway, tumors, paralysis or malfunction of vocal cords. Laryngospasm due to hypocalcemia is a rare cause of stridor in adults, although occasionally reported in the neonates. We report an elderly lady having stridor and laryngospasm, secondary to acquired hypoparathyroidism and secondary hypocalcemia, without risk factors for hypoparathyroidism such as recent neck surgery or irradiation. We did an extensive review of literature to find only a few cases of acquired primary hypoparathyroidism in adults with the only complaint being stridor. This case underlines the fact that a common symptom like stridor rarely occurs due to uncommon causes. This case is being reported for its rarity and amenability to complete cure in event of correct diagnosis.
Arif, Farzana; Wu, Susan; Andaz, Shahriyour; Fox, Stewart
Primary epithelial myoepithelial carcinoma of lung is a rare entity and is thought to arise from the submucosal bronchial glands distributed throughout the lower respiratory tract. Because of the rarity of this tumor, we describe one case of epithelial myoepithelial carcinoma arising in the bronchus intermedius and presenting as an endobronchial mass. A 57-year-old male patient presented with an incidental finding of an endobronchial mass located in the lumen of the right lower lobe bronchus and caused near total luminal occlusion of the bronchus. An endobronchial carcinoid tumor was entertained clinically. Subsequently the patient underwent an uneventful videothoracoscopic lobectomy of lower and middle lobes of the right lung. Morphologically and immunohistochemically the tumor was characterized by two cell populations with epithelial and myoepithelial cells forming duct-like structure. The final diagnosis of epithelial myoepithelial carcinoma of lung was rendered.
Ankur Nandan Varshney
Full Text Available Extranodal NK/T-cell Lymphoma of nasal type is a rare and comparatively a new entry among group of Non-Hodgkin lymphomas. The disease is characterized by a clinically aggressive course with involvement of upper aero-digestive tract and classical immune-phenotyping with CD2, CD3 and CD56 positivity. Being a rare entity, treatment entities are yet not formulated in guidelines. We hereby report a case of extranodal NK/T-cell lymphoma with predominant T cell markers who was initially treated with CHOP regime of non-Hodgkin lymphoma and later successfully treated with SMILE regime.
Güzel, Aygül; Köksal, Nurhan; Aydın, Davut; Aslan, Kerim; Gören, Fikret; Karagöz, Filiz
Gingivitis due to sarcoidosis is a relatively rare condition. Gingivitis or isolated gingival involvement may be the first sign of systemic sarcoidosis. We report the case of a 37 year-old woman with isolated gingivitis due to sarcoidosis confirmed by biopsy. Following treatment with a systemic corticosteroid (prednisolone 40 mg/day), all clinical and radiologic findings were completely improved. In cases of chronic and intractable gingivitis, systemic sarcoidosis should be suspected. It should be confirmed with a biopsy, and the patient should be referred to a chest disease clinic to exclude other organ involvement.
Dorenbeck, U.; Bretschneider, T.; Feuerbach, S. [Department of Diagnostic Radiology, University Hospital of Regensburg, Franz-Josef-Strauss-Allee 11, 93042 Regensburg (Germany); Leingaertner, T.; Kraemer, B.K. [Department of Internal Medicine II, University Hospital of Regensburg, Franz-Josef-Strauss-Allee 11, 93042 Regensburg (Germany)
A case of rare calcification of the tentorium cerebelli, the thoracal dura mater of the spine, and the sclera in an adult male patient with tertiary hyperparathyroidism is presented. The often reported feature in the skull is that of a combination of osteopenia and osteosclerosis giving a granular bone texture. Extensive dura calcification with this condition has very rarely been reported. It is the aim of this paper to document the latter in a patient with chronic renal failure and tertiary hyperparathyroidism. (orig.)
Full Text Available Giant lipomas are benign soft tissue tumours. They are found relatively rarely on the posterior part of the neck. Bleeding pressure ulcer in this giant tumour is a rare presentation. Surgical interventions in these tumours are very challenging because, sometimes, extension to the spinal cord and malignant change may occur, especially in old age. Knowledge of the anatomy and meticulous surgical techniques are needed for such giant lipomas.
M. P. V. Prabhat; Chintamaneni Raja Lakshmi; Sai Madhavi, N.; Sujana Mulk Bhavana; Gummadapu Sarat; Kodali Ramamohan
Focal epithelial hyperplasia is a rare contagious disease caused by human papilloma virus. Usually HPV involves either cutaneous or mucosal surfaces, whereas concomitant mucocutaneous involvement is extremely rare. We report such a unique case of multifocal epithelial hyperplasia involving multiple sites of oral cavity along with skin lesions in a 65-year-old female. We also discuss the probable multifactorial etiology and variable clinical presentations of the lesions, including evidence of...
Full Text Available Uterine lipoleiomyomas are rare benign tumours that are composed of various mixtures of smooth muscle and mature fat tissue. Leiomyomas, which arise primarily in the ovary, are extremely rare tumours that account for 0.5–1% of all benign ovarian tumours. To the best of our knowledge, we present the first case of an ovarian leiomyoma coexisting with a uterine lipoleiomyoma in the postmenopausal period. A 59-year-old, gravida 4, para 3, postmenopausal woman exhibited pelvic discomfort and increased frequency of micturition. A pelvic examination revealed a solid, tender mass on the left side that could not be clearly separated from the uterus. She underwent a laparotomy with an initial diagnosis of a left ovarian mass. She had previously undergone a total abdominal hysterectomy and bilateral salpingo-oophorectomy. A histopathological examination revealed a uterine lipoleiomyoma, composed of variable amounts of smooth muscle cells and mature adipocytes and a right ovarian leiomyoma composed of interlacing bundles and fascicles of spindle cells. The coexistence of these two rare entities in the same individual may represent a common pathway as a stimulating agent. This case may help to clarify the pathogenesis of these lesions.
Full Text Available Primary squamous cell carcinoma (SCC of the renal parenchyma is a very unusual entity which needs to be differentiated from primary SCC of renal pelvis, SCC from another primary site, and urothelial carcinoma with extensive squamous differentiation. We are most probably describing the second case of primary SCC of the renal parenchyma in a 51-year-old male who presented with heaviness of right upper abdomen with intermittent pain in right flank. Contrast-enhanced computed tomography (CECT revealed a mass in the right lower pole of the kidney and histopathology following nephrectomy displayed the features of well-differentiated squamous cell carcinoma without urothelial involvement.
Full Text Available Paragangliomas are rare neuroendocrine neoplasms arising in extra-adrenal chromaffin cells of autonomic nervous system and histologically akin to chemodectomas. They are rare, affecting about 1 in 2,000,000 population. It is a generic term applied to tumors of paraganglia regardless of the location. In rare instances, paragangliomas present around and involve the pancreas, thereby mimicking any one of the more common primary pancreatic lesions. Pancreatic paraganglioma is an extremely rare tumor. It grows slowly, so radical resection is recommended to achieve curability with good prognosis. These neoplasms present considerable diagnostic difficulty not only for the clinician and radiologist but also for the pathologist. Here, we report a case of a 55-year-old woman who presented with a left-sided abdominal swelling for 3 months duration, initially having clinical suspicion of an ovarian tumor. The radiological imaging revealed a lesion in the tail of pancreas with a differential diagnosis of pancreatic carcinoma and metastatic tumor. Only after exploratory laparotomy, the diagnosis was made as a rare case of pancreatic paraganglioma on the basis of histological examination and immunohistochemistry.
Borgohain, Mondita; Gogoi, Gayatri; Das, Dipak; Biswas, Manjusha
Paragangliomas are rare neuroendocrine neoplasms arising in extra-adrenal chromaffin cells of autonomic nervous system and histologically akin to chemodectomas. They are rare, affecting about 1 in 2,000,000 population. It is a generic term applied to tumors of paraganglia regardless of the location. In rare instances, paragangliomas present around and involve the pancreas, thereby mimicking any one of the more common primary pancreatic lesions. Pancreatic paraganglioma is an extremely rare tumor. It grows slowly, so radical resection is recommended to achieve curability with good prognosis. These neoplasms present considerable diagnostic difficulty not only for the clinician and radiologist but also for the pathologist. Here, we report a case of a 55-year-old woman who presented with a left-sided abdominal swelling for 3 months duration, initially having clinical suspicion of an ovarian tumor. The radiological imaging revealed a lesion in the tail of pancreas with a differential diagnosis of pancreatic carcinoma and metastatic tumor. Only after exploratory laparotomy, the diagnosis was made as a rare case of pancreatic paraganglioma on the basis of histological examination and immunohistochemistry. PMID:24083178
Prabhat, M. P. V.; Raja Lakshmi, Chintamaneni; Sai Madhavi, N.; Bhavana, Sujana Mulk; Sarat, Gummadapu; Ramamohan, Kodali
Focal epithelial hyperplasia is a rare contagious disease caused by human papilloma virus. Usually HPV involves either cutaneous or mucosal surfaces, whereas concomitant mucocutaneous involvement is extremely rare. We report such a unique case of multifocal epithelial hyperplasia involving multiple sites of oral cavity along with skin lesions in a 65-year-old female. We also discuss the probable multifactorial etiology and variable clinical presentations of the lesions, including evidence of HPV 16 expression, as detected by polymerase chain reaction. The present report illustrates the need for careful examination and prompt diagnosis of the disease, as it might be associated with high risk genotypes such as HPV 16 and 18. PMID:24455323
Raja Shekhar R.Sappati Biyyani
Full Text Available Background: Primary gastrointestinal T-cell lymphomas are extremely rare entity and are much less common than B-Cell lymphomas. Case History: A primary T-cell lymphoma was diagnosed in an octogenarian African American male with a history of diabetes mellitus type-II, remote history of prostate cancer, hypertension, obesity and hyperlipidemia. He had symptoms of dysphagia, early satiety, lossof appetite and loss of weight. He was Helicobacter pylori IgG antibody positive and on treatment. Result of first biopsy duringendoscopy showed only heavy lymphoid infiltrate. But, due to high suspicion of malignancy, a second upper gastrointestinal endoscopy and biopsy was performed .This biopsy from the large deep 3cm friable ulcer with nodular base was taken which showed atypical lymphoid cells positive for CD3 and CD7 and negative for CD5, CD4 , CD8 and CD56 . The combination of the histological, immunohistological stain results and the gene rearrangement results confirmed T cell lymphoma. The patient died after 5 months after5 cycles of chemotherapeutic agents of severe dehydration and complications from sepsis.
Full Text Available Background and Aims: The aim of the study is to search the lesion localization of the pure isolated facial paresis-dysarthria syndrome in patients who were admitted to our neurology clinic in a prospective study. Methods: Over a period of six years, the patients who had no prominent sensorimotor dysfunction were examined by neurologists and underwent computerized tomography (CT and/or magnetic resonance imaging (MRI. Results: Eleven patients out of more than 2000 had the aforementioned clinical picture. Lacunar infarctions were identified at the corona radiata in nine patients, and at the internal capsule in two patients. As reported previously, facial paresis was usually mild and temporary. Six of our eleven patients were seen at the outpatient clinic one month later. Four of them had completely recovered and the other two had mild dsyarthria without any facial paresis. The other five could not be reached after leaving the hospital. Conclusions: Dysarthria-facial paresis is a rare clinical entity and possibly a variation of dysarthria-clumsy hand syndrome, and we suggest that pure facial paresis (FP and pure dysarthria should be considered as very extreme examples of this syndrome.
Bishnu Prasad Das
Full Text Available Aggressive angiomyxoma is a rare, locally invasive mesenchymal tumor predominantly presenting in women of reproductive age and also having a moderate-to-high risk for local relapse. Hence, it needs to be differentiated from other mesenchymal tumors occurring in this region. We present here a case of a 40-year-old female presenting with a large, fleshy, pedunculated mass on the right labia majora.
Das, Bishnu Prasad; Baruah, Dipankar; Medhi, Kaberee Bhuyan; Talukder, Bharat
Aggressive angiomyxoma is a rare, locally invasive mesenchymal tumor predominantly presenting in women of reproductive age and also having a moderate-to-high risk for local relapse. Hence, it needs to be differentiated from other mesenchymal tumors occurring in this region. We present here a case of a 40-year-old female presenting with a large, fleshy, pedunculated mass on the right labia majora.
Kanwar A. J
Full Text Available A 25 year old female presented with an asymptomatic reddish brown nodule on the nose. History of ulceration and bleeding suggested a diagnosis of granuloma pyogenicum. The lesion was excised. Histology revealed features of Spitz naevus.
Full Text Available Bilateral acalculus ureteric obstruction is described as rare sequelae of acute appendicitis in two paediatric patients aged 6 and 11 years presented with features of anuria. Imaging and endoscopic evaluation confirmed bilateral ureteric obstruction secondary to bladder wall oedema as an inflammatory reaction to appendix. Both cases recovered following bilateral ureteric stenting and are doing well.
Bahl, Sumit; Shah, Vandana; Anchlia, Sonal; Vyas, Siddharth
Cystic hygroma is a benign congenital malformation of the lymphatic system that occurs in infant or children younger than 2 years of age. Although cystic hygroma is well recognized in pediatric practice, it seldom presents de novo in adulthood. These are commonly present in head and neck but can be present anywhere. Cystic hygroma is very rare in adults, but it should be considered in the differential diagnosis of adult neck swellings. Patients presenting with a painless, soft, fluctuant, and enlarging neck mass should have a careful history and physical examination along with radiological imaging to assist with diagnosis. Surgical intervention is the treatment of choice for this rare condition. Here, we are reporting a case of cystic hygroma in a 32-year-old male patient in the neck region. The objectives of this case report are to discuss the clinical presentation, diagnosis, histopathological findings and management of this malformation.
Arslan, Yusuf; Altintoprak, Fatih; Serin, Kursat R; Kivilcim, Taner; Yalkin, Omer; Ozkan, Orhan V; Celebi, Fehmi
Ectopic liver tissue (ELT) is a rare condition, which is usually not diagnosed preoperatively, but coincidentally during abdominal surgery. While the location of ELT can vary, it is usually localized on the gallbladder wall or in close proximity. ELT is associated with various complications, a major complication being extrahepatic hepatocellular carcinoma. A 59-year-old female underwent elective surgery for chronic cholecystitis with stones. During laparoscopic exploration, a 2-cm-diameter ELT was detected in the anterior gallbladder wall and a laparoscopic cholecystectomy was performed. The case is presented due to the rare nature of ELT and as a reminder of ELT-related complications.
Pavan Kumar Gujjar
Full Text Available Neurofibroma (NF is a benign neoplasm derived from peripheral nerve cells. NF may extend either as a solitary lesion or as part of a generalized syndrome of neurofibromatosis. Intraorally, the intraosseous variant of neurofibroma is a very rare tumor. The literature provides only few cases of solitary intraosseous neurofibroma of the mandible. We report a case of 28-year-old female who was diagnosed with a solitary intraosseous neurofibroma involving the lower left quadrant of the mandible. The present case is rare in regard to its dimensions and its location.
Pralong, G; Fournier, C; Dayer, E; Meier, P
Some autoimmune diseases may be found presenting simultaneously antibodies (Abs) against basal membrane and anti-neutrophil cytoplasm (ANCA). The clinical picture is that of the Goodpasture syndrome with anti-basement membrane Abs associated with an ANCA-associated small-vessel vasculitis (micro-PAN). The arisen of these two pathological entities is nevertheless too frequent to be the fruit of the only fate. The pathophysiological hypothesis remains that of the micro-PAN initially creates lesions of the basement membrane facilitating the formation of Abs against some constituents of this latter. The prognosis remains controversial, leaving open this issue. This article aims to present a recent literature revue dealing with the simultaneous presence of these two auto-immune diseases.
Full Text Available This report presents a sixty year old female with unusual appearance of bilateral xanthelasma palpebrarum of periorbital region. These lesions were approximately 7 cm × 3 cm in dimensions. Patient’s main complaints were aesthetic appearance and hindrance in downward gaze. Considering the size of the lesions surgical excision was planned and the raw area covered with full thickness skin grafts from supra clavicular region. The aesthetic and functional outcome was good. The aim of this case report is to put forward an unusual presentation of xanthelasma, at the same time to keep in mind the surgical excision with full thickness skin grafting as an excellent treatment modality for periorbital xanthelasma palpebrarum especially in large lesions where other modalities have their own limitations.
Astigueta, Juan Carlos; Abad-Licham, Milagros; Silva, Eloy; Alvarez, Víctor; Piccone, Francis; Cruz, Enrique; Redorta, Joan Palou
It is very uncommon for urothelial carcinoma to develop in an ureterocele. It is generally discovered in an imaging study or in connection with haematuria. We found very few reports in the literature. Here, we report on the case of a 71-year-old male who initially presented with haematuria and low back pain and who then underwent transurethral resection for an intraureterocele tumour. Pathology confirmed urothelial carcinoma.
Srinivasa Prasad T
Full Text Available Ectopic eruption of teeth into a region other than the oral cavity is rare although there have been reports of teeth in the nasal septum, mandibular condyle, coronoid process, palate, chin and maxillary sinus. Occasionally, a tooth may erupt in the maxillary sinus and present with local sinonasal symptoms attributed to chronic sinusitis. We present a case of an ectopic maxillary third molar tooth that caused chronic purulent sinusitis in relation to the right maxillary sinus.
Anil Kumar Sharma
Full Text Available Intraparenchymal schwannomas arising in the brainstem are very rare, and only eight cases have been reported in literature till now. We report an intraparenchymal brainstem schwannoma presenting with the classical clinical presentation of an intrinsic brainstem lesion, and discuss its clinicoradiological characteristics and histological origins. We highlight the importance of an intraoperative frozen section diagnosis in such cases. Intraoperative tissue diagnosis significantly may alter the surgical strategy, which should be aimed at near total intracapsular decompression of the schwannoma.
Full Text Available Granulocytic sarcoma (GS is an extremely rare condition involving infiltration of myeloblasts or immature myeloid cells in an extramedullary site. It is also known as chloroma, myeloid sarcoma or extramedullary myeloid tumor. It usually occurs concomitantly with acute myelogenous leukemia or with the onset of blastic phase of chronic myelogenous leukemia. On rare occasions, it evolves even before the onset of leukemias, and when it precedes leukemias without any overt signs, it is referred to as the primary type. Although GSs can involve any body part, localization in the oral cavity is extremely rare. The recognition of this rare primary entity is important because early aggressive chemotherapy can cause regression of the tumor and improve survival. Here, we report a rare case of GS in a nonleukemic 62-year-old female who presented with generalized gingival enlargement involving both maxilla and mandible.
Sacchidanand, S; Sharavana, S; Mallikarjun, M; Nataraja, H V
Cutaneous tuberculosis continues to be an important public health problem even with the availability of highly effective anti-tuberculous drugs. It constitutes 0.1% of all cases of extrapulmonary tuberculosis. Lupus vulgaris is the most common form of cutaneous tuberculosis that occurs in previously sensitized individuals with a moderate degree of immunity against tubercle bacilli. The different types of lupus vulgaris include plaque, ulcerative, vegetative, papular and nodular, and tumor forms. A 40-year-old man presented with large multiple plaques over right upper limb, right side of chest and back, and right lower limb for the past 30 years. Histopathology showed numerous noncaseating granulomas with Langhan's type of giant cells. The Mantoux test showed strong positivity and there was excellent response to anti-tuberculous treatment. This case is being reported because of its extreme chronicity of 30 years duration, unusually large size and multiplicity of lesions.
Thomas, Vijy Paul
A case of Morgagni hernia presenting in adulthood is presented. This form of congenital diaphragmatic hernia is rare in itself and is known to present in adulthood in some cases. But presentation after having undergone three surgeries-PDA ligation, appendicectomy and a full term pregnancy followed by LSCS is even rarer. This case is therefore by any yardstick an extremely rare one.
Celli, Romulo; Cai, Guoping
Ewing sarcoma/primitive neuroectodermal tumor represents a spectrum of undifferentiated tumors with similar biology that together represent the second most common sarcoma in the pediatric-young adult age range. Very rarely, this tumor presents as a primary neoplasm of the kidney. The clinical presentation of this tumor is not specific, and other renal tumors may present with a similar histologic appearance. Establishing the correct diagnosis is critical because renal Ewing sarcoma/primitive neuroectodermal tumor carries a strikingly dismal prognosis and thus dictates a specific treatment strategy. A low threshold for the use of ancillary molecular tests is recommended, particularly in diagnostically problematic cases. Important considerations with regards to morphology, immunohistochemistry, and molecular alterations will be reviewed here and should be taken into account before rendering this rare and lethal diagnosis.
Jain, Aditya; Rohilla, Rajesh; Devgan, Ashish; Wadhwani, Jitendra
Tuberculosis tenosynovitis of the wrist and hand is an unusual condition. It mimics a number of other clinical conditions such as ganglion, De quervan's disease, carpal tunnel syndrome, granulomatous gout, neurofibroma, and haemangioma. We present case of 58-year-old male patient with swelling over thenar region of left hand where the initial fine needle aspiration cytology (FNAC) report suggested diagnosis of ganglion/synovial cyst, but the patient did not improve with conservative treatment. FNAC was repeated which showed granulomatous inflammation consistent with the clinical diagnosis of tuberculosis and antitubercular therapy for 9 months was given. Swelling disappeared and normal range of movements was attained in adjacent joints at final follow-up of 12 months from initiation of antitubercular treatment (ATT). The literature on the management of tubercular tenosynovitis has mostly incorporated surgical method as one of the first-line treatments. We report an unusual case, where the tenosynovitis of wrist completely responded within 12 months of ATT without any need of surgical treatment.
Full Text Available Acromegaly is diagnosed clinically by the universal presence of acral enlargement and typical coarse facies. We report a short, elderly female developing acromegalic facies for last 10 years without acral overgrowth. She is a patient of primary hypothyroidism, well controlled for last 20 years. Acromegaly was proven by high level of serum insulin like growth factor-1 (IGF-1 and elevated and nonsuppressed level of growth hormone (GH, with other hormonal profile being undisturbed. She had mild insulin resistance and systemic hypertension in absence of any visual field defect. Magnetic resonance imaging (MRI of brain revealed pituitary hyperplasia without any detectable adenoma. No source of ectopic secretion of GH or growth hormone releasing hormone (GHRH could be localized. Therefore, atypical presentation of acromegaly needs high degree of suspicion even if some of the common features are lacking. Here, we have biochemically proved acromegaly with typical facies, short stature but no acral overgrowth and pituitary adenoma despite longstanding disease activity, and thus eluding diagnosis for years.
Aiyer, Siddharth N; Shetty, Ajoy Prasad; Kanna, Rishi; Maheswaran, Anupama; Rajasekaran, S
Spinal cord herniation following surgery is an extremely uncommon clinical condition with very few reports in published literature. This condition usually occurs as a spontaneous idiopathic phenomenon often in the thoracic spine or following a scenario of post traumatic spinal cord/nerve root injury. Rarely has it been reported following spinal cord tumor surgery. To document a case of cervical spinal cord herniation as a late onset complication following spinal cord tumor surgery with an atypical presentation of monoparesis. Case report. We describe the clinical presentation, operative procedure, post operative outcome and review of literature of this rare clinical condition. A 57-year-old man presented with right upper limb monoparesis due to a spinal cord herniation 6 years after a cervical intradural meningioma excision. The patients underwent surgery to reduce the herniation and duroplasty with subsequent complete resolution of symptoms. Spinal cord herniation must be considered as differential diagnosis in scenarios of spinal cord tumor excision presenting with late onset neurological deficit. These cases may present as paraparesis, Brown-sequard syndrome and rarely as in our case as monoparesis.
Full Text Available Calcifying odontogenic cyst (COC, Central odontogenic fibroma (COF and aggressive central giant cell granuloma (CGCG are rare pathologic diseases affecting the jaws. While the Co-existence of two of them is reported in the literature, existence of all three conditions in one patient is an extremely rare entity. In the present report, initial biopsy revealed fibrosarcoma, therefore mandibular resection was performed for the subject. Sectional Histopathologic evaluation revealed the co-existence of three conditions through histopathologic evaluation. This report emphasizes the importance of precise microscopical evaluation of jaw lesions and thorough sectional examination of the lesions to reach the precise diagnosis. Treatment modalities and follow-up radiographs are also provided to help clinicians manage these entities.
M. P. V. Prabhat
Full Text Available Focal epithelial hyperplasia is a rare contagious disease caused by human papilloma virus. Usually HPV involves either cutaneous or mucosal surfaces, whereas concomitant mucocutaneous involvement is extremely rare. We report such a unique case of multifocal epithelial hyperplasia involving multiple sites of oral cavity along with skin lesions in a 65-year-old female. We also discuss the probable multifactorial etiology and variable clinical presentations of the lesions, including evidence of HPV 16 expression, as detected by polymerase chain reaction. The present report illustrates the need for careful examination and prompt diagnosis of the disease, as it might be associated with high risk genotypes such as HPV 16 and 18.
Full Text Available Adenomyoepithelioma of the breast is a rare tumor. Malignant change arising in this lesion is infrequent and only a few cases have been reported. We discuss a case of a 56-year-old female presenting with a firm breast mass, which was interpreted as myoepithelial carcinoma arising in a background of adenomyoepithelioma, based on morphological and immunohistochemical studies. This case is being highlighted for its rarity and distinct morphological spectrum.
López-Gómez, Javier; Contreras, Juan S.; Figueroa-Ruiz, Marco; Servín-Torres, Erick; Velázquez-García, José; Bevia-Pérez, Francisco; Delgadillo-Teyer, Germán
INTRODUCTION: Hemophilic pseudotumor is a rare complication that occurs in patients with severe hemophilia. Results from multiple episodes of bleeding into the bones and soft tissues. PRESENTATION OF CASE: A 31 years old male patient, with severe hemophilia A. Diagnosed with an abdominal tumor 10 years ago during routine screening, that progressively grew to encompass the entire abdominal area, with symptoms of intestinal obstruction. DISCUSSION: Hemophilic pseudotumor appears as a pain...
Pinto, Antonione Santos Bezerra; Department of Oral Pathology – São Leopoldo Mandic Institute and Research Center – Campinas – SP – Brazil.; De Araújo, Vera Cavalcanti; Department of Oral Pathology – São Leopoldo Mandic Institute and Research Center – Campinas – SP – Brazil.; Santos, Fabrício Passador; Department of Oral Pathology – São Leopoldo Mandic Institute and Research Center – Campinas – SP – Brazil.; Filho, José Ferreira de Menezes; Antonio Carlos Institute of Tocantins – Araguaína – TO – Brazil.; Siqueira, Viviane; Department of Oral Pathology – São Leopoldo Mandic Institute and Research Center – Campinas – SP – Brazil.; Soares, Andresa Borges; Department of Oral Pathology – São Leopoldo Mandic Institute and Research Center – Campinas – SP – Brazil.
A case of solitary fibrous tumor is reported. Solitary fibrous tumor is a rare neoplasia characterized by the proliferation of fusiform cells of mesenchymal origin accounting for at least 2% of all soft tissue tumors. In this present case, the initial diagnosis was salivary gland tumor because of the location in the hard palate. Histologically, the tumor was composed by conjunctive tissue with proliferation of oval and fusiform cells. The immunohistochemical analysis was positive for CD34 and...
Blanc, Gaelle; Girard, Nicolas; Alexandre, Christian; Vignon, Eric
Retroperitoneal fibrosis is a rare inflammatory and fibrotic process in the retroperitoneal peri-aortic tissues, associated with ureters and other abdominal organs' entrapment. Here we report an original observation of a 55-year-old patient presenting with chronic lombalgia disclosing idiopathic retroperitoneal fibrosis. After one-year follow-up, treatment with corticosteroids led to a complete clinical, biological, and radiological response. Pathogenesis and therapeutic options in idiopathic retroperitoneal fibrosis are discussed.
Full Text Available Intramedullary spinal epidermoid cysts are rare, with only few cases having been reported in the literature. We are reporting a case of a 10-year-old female child who presented with symptoms of meningitis with progressive paraparesis. Magnetic resonance imaging of the spine revealed an intramedullary epidermoid cyst from C6 to D5. Near-total excision of the tumor was performed. Histopathological report confirmed the diagnosis of epidermoid cyst. The patient showed progressive recovery.
Khan, Fazal; Hamid, Arsalan; Fatima, Benish; Hashmi, Shiraz; Fatimi, Saulat
A 25-year-old man presented with a 2-month history of dysphagia and past history of pulmonary and intestinal tuberculosis. A barium swallow showed a point of constriction 42 mm above the gastroesophageal junction. Computed tomography revealed large opacities in bilateral lung fields, encroaching more on the esophagus. The lesion progressively compressed the esophagus as it moved inferiorly. A right posterolateral thoracotomy was performed for sub-anatomical resection of the mass. A biopsy revealed homogenous whirling hyalinized collagen fibers, highly suggestive of pulmonary hyalinizing granuloma, with no evidence of malignancy. Pulmonary hyalinizing granuloma should be considered in the differential diagnosis of longstanding dysphagia.
Full Text Available The calcifying epithelial odontogenic tumor is a rare benign odontogenic tumor that was first described by Pindborg in 1955. It accounts for less than 1% of all odontogenic neoplasms. The tumor is characterized histologically by the presence of polygonal epithelial cells, calcification, and eosinophilic deposits resembling amyloid. Noncalcifying Pindborg tumor is very rare and only three cases have been documented in the English language literature so far. We present an additional case of noncalcifying Pindborg tumor and review the previously reported cases. Because noncalcifying Pindborg tumor is believed to be an aggressive variant, a definitive resection of the tumor with tumor-free surgical margins and long-term follow-up is recommended.
Kaushal, Seema; Mathur, Sandeep R; Vijay, Maneesh; Rustagi, Ankur
The calcifying epithelial odontogenic tumor is a rare benign odontogenic tumor that was first described by Pindborg in 1955. It accounts for less than 1% of all odontogenic neoplasms. The tumor is characterized histologically by the presence of polygonal epithelial cells, calcification, and eosinophilic deposits resembling amyloid. Noncalcifying Pindborg tumor is very rare and only three cases have been documented in the English language literature so far. We present an additional case of noncalcifying Pindborg tumor and review the previously reported cases. Because noncalcifying Pindborg tumor is believed to be an aggressive variant, a definitive resection of the tumor with tumor-free surgical margins and long-term follow-up is recommended.
Ammirati, Enrico; Manassero, Alberto; Giammò, Alessandro; Marson, Francesco; Gurioli, Alberto; Carone, Roberto
Primary female bladder neck obstruction is a rare clinical condition characterized by the absence/incomplete bladder neck opening during the voiding phase of micturition. We present the cases of two women complaining dysuria, abdominal straining and sensation of incomplete bladder emptying. Videourodynamic evaluation was fundamental for a correct diagnosis. Videourodynamic evaluation showed a high detrusor pressure during emptying phase, partial use of abdominal strain, very low urine flow rate and significant postvoid residual; imaging showed a defect in the physiological funneling of the bladder neck, absent or incomplete. The first step therapy is represented by oral alpha-blockers and clean intermittent self-catheterization in case of high postvoid residual. Surgical operations, such as bladder neck incision and resection, represent the last option. In our experience, bladder neck obstruction is a rare condition in women and only a complete clinical evaluation associated with videourodynamic study can lead to an appropriate diagnosis and treatment.
Full Text Available Mycobacterium leprae, the causative agent of leprosy (Hansen's disease, is a slow growing intracellular acid-fast bacillus that affects the skin, peripheral nerves and respiratory tract. In patients with suppressed cell-mediated immunity, the infiltration of the Bacilli can produce disseminated illness such as leprous neuromyositis. We reported a case of 56-year-old gentleman presenting with pyrexia of unknown origin, asymmetric sensory motor axonal polyneuropathy and was on chronic exogenous steroid therapy. On evaluation, his skin, muscle, nerve and bone marrow biopsy showed numerous globi of acid-fast Bacilli suggestive of leprous neuromyositis, a rare form of disseminated Hansen's disease. We reported this case in view of its rarity, atypical manifestation of a relatively rare disease and literature review on poor electrophysiological correlation in the diagnosis of leprous neuromyositis as compared to the histopathological examination.
Full Text Available Hypercalcemia is often a clue to the presence of unsuspected illness. We present an interesting case of an HIV-positive transgender female with a rare cause of silicone-induced granulomatous hypercalcemia. Although there have been a few case reports of silicone injections in dialysis patients causing hypercalcemia, this metabolic derangement secondary to silicone granulomas continues to be a unique entity with an unclear pathophysiology. We present a 45-year-old transgender HIV-positive female, with extensive silicone injections who presented with symptomatic hypercalcemia. Workup for malignancy and hyperparathyroidism was negative. 1,25-Dihydroxyvitamin D level and 24-hour urine calcium level were elevated. CT scan showed extensive high-density reticulonodular densities in the buttocks and gluteal muscle fascia extending upwards to the lumbar region, along with prominent external iliac and inguinal lymph nodes. Nuclear imaging showed diffuse heterogeneity and increased uptake in the buttocks, most consistent with granuloma calcifications, and an inguinal lymph node biopsy confirmed a foreign body giant cell reaction. The patient was started on prednisone and this resulted in decrease in serum and urinary calcium levels. Physicians should have a high index of suspicion for silicone-induced hypercalcemia considering the growing prevalence of body contour enhancement with injections, implants, and fillers using this material.
Full Text Available CASE: A 30 years old female G 4 P 2 L 2 A 1 at 34 wks o f pregnancy with complains of a cute pain abdomen more on right side and constipation, fever on & off & vomiting. After through investigations exploratory laparotomy was done which reveled spontaneous rupture right sided uterine artery with 1000ml of haemoperitoneum. Hysterectomy was performed after delivering the baby due to uncontrolled bleeding & difficult homeostasis. Because of maternal vital signs become unstable & homeostasis was difficult hysterectomy was performe d & blood transfusion administered. Although very rare , hemoperitoneum should be included in the differential diagnosis when a pregnant women experiences acute onset , severe abdominal pain , even without an episode of abdominal trauma . BACKGROUND : Spontaneous rupture of uterine artery during pregnancy is rare, presenting symptom include acute onset abdominal pain & maternal hypovolemic collapse due to hemoperitoneum. A typical case of subculture uterine artery rupture @ 34wks . of gestation occurred in a women. CONCLUSION : Spontaneous rupture of the uterine vessels during pregnancy is a rare complication & may lead to maternal & fetal morbidity & mortality. Diagnosis and treatment are based on the clinical symptoms of actuate abdominal pain & laborato ry test of hypovolemic shock signs.
Makker, Jasbir; Sakam, Sailaja; Arety, Prasanthi; Niazi, Masooma; Balar, Bhavna
Blue nevus, a pigmented skin lesion, affects the dermal melanocytes that are rich in melanin. Its occurrence on skin has been well described in literature. Less commonly, involvement of mucosal surfaces especially genitourinary tract has also been noticed. Here we present a rare case of a blue nevus involving the rectum. So far there has been only one prior description of the blue nevus involving the gastrointestinal mucosa. Differentiation of this lesion from melanoma is the key. Simple excision of the blue nevus with a biopsy forceps during the colonoscopy is an effective management.
Full Text Available Alternaria alternata is one of the rarest fungi associated with paranasal sinusitis. Alternaria species are pigmented (also known as dematiaceous or phaeoid filamentous fungi, which are well-known soil saprophytes and plant pathogens that infrequently cause infection in humans mainly, cutaneous lesions. We present a case of osteomyelitis of maxilla caused by a rare fungus- A. alternata in a diabetic patient with poor glycemic control who was successfully treated with antifungal and surgical debridement over the period of 6 months.
Full Text Available Mucocele forms because of salivary gland mucous extravasation or retention and is usually related to trauma in the area of the lower lips. Salivary duct cyst, however, is a type of mucous retention cyst which is almost never located on the lower lip. The aim of this paper is to report this extremely rare salivary duct cyst present on the lower lip and to critically review the literature to build important concepts that would help clinicians in the diagnosis and treatment of this pathology.
A. N. Sulabha
Full Text Available Supernumerary teeth are defined as any teeth in excess of normal number. Mesiodens is a supernumerary tooth, in the central region of premaxilla between two central incisors. Dens invaginatus is a developmental anomaly resulting from invagination in the surface of tooth crown before calcification has occurred. Radiographically, it is observed as infolding of a radioopaque ribbon like structure, with equal density as enamel, extending from cingulum into a root canal and sometimes reaching the root apex. This paper aims to present a rare association of dens invaginatus with two mesiodentes in a child causing the eruption disturbance and unaesthetic appearance in anterior maxilla.
Sulabha, A. N.; Sameer, C.
Supernumerary teeth are defined as any teeth in excess of normal number. Mesiodens is a supernumerary tooth, in the central region of premaxilla between two central incisors. Dens invaginatus is a developmental anomaly resulting from invagination in the surface of tooth crown before calcification has occurred. Radiographically, it is observed as infolding of a radioopaque ribbon like structure, with equal density as enamel, extending from cingulum into a root canal and sometimes reaching the root apex. This paper aims to present a rare association of dens invaginatus with two mesiodentes in a child causing the eruption disturbance and unaesthetic appearance in anterior maxilla. PMID:23198162
Askin Esen Hasturk
Full Text Available Gelatin sponge, oxidized cellulose and microfibrillar collagen are used to achieve hemostasis during neurosurgical procedures. Hemostatic agents may produce clinically symptomatic, radiologically apparent mass lesions. The differential diagnosis should include the foreign body along with recurrent tumor. We present a case of intracranial hemostatic agents found in a 56-year-old male patient seven years after undergoing a craniotomy for a left posterior parietal convexity meningioma. Preoperative magnetic resonance imaging (MRI suggested the presence of a recurrent tumor. We emphasize that although it is rare, a granuloma due to a foreign body reaction can result in a false image of tumor recurrence.
Cytomegalovirus is the most common viral infection after kidney transplantation. Clinical presentations of cytomegalovirus infection range from asymptomatic infection to organ-specific involvement. Most symptomatic infections manifest as fever and cytopenia. The gastrointestinal tract is the most common site of tissue-invasive infection, often presenting as diarrhea or gastrointestinal bleeding. Gastrointestinal obstruction, perforation, thrombosis of large gastrointestinal veins, splenic artery thrombosis, and pancreatitis are rare gastrointestinal presentations of cytomegalovirus infection. Renal-allograft ureteral stricture and skin involvement are other rare presentations of cytomegalovirus infection. hemophagocytic syndrome, thrombotic microangiopathy, adrenal insufficiency, and renal allograft artery stenosis are other rare symptoms of cytomegalovirus infection.
Reis, C.; Carneiro, E.; Fonseca, J.; Salgado, A. [Hospital S. Joao, Departments of Neuroradiology, Porto (Portugal); Pereira, P.; Vaz, R. [Hospital S. Joao, Department of Neurosurgery, Porto (Portugal); Pinto, R. [Hospital S. Joao, Department of Orthopaedics, Porto (Portugal); Capelinha, A.F.; Lopes, J.M. [Hospital S. Joao, Department of Pathology, Porto (Portugal)
Epithelioid hemangioendothelioma (EHE) is a rare vascular soft-tissue tumour of intermediate malignancy. Neurofibromatosis type I (NF-1) is a genetic syndrome associated with soft tissue sarcoma and higher risk of developing neoplasia. Lateral meningoceles are uncommon entities, being mostly associated with NF-1. We report a case of a 31-year-old woman, with NF-1 and past history of right thalamic/peduncular astrocytoma WHO grade II, admitted to the Neurosurgery Department in December 2003 due to severe low back pain, irradiating to the left leg without a radicular pattern. Thoraco-lumbar magnetic resonance imaging (MRI) showed a large left posterior paravertebral expansive lesion, bilateral and multiple thoraco-lumbar lateral meningoceles and dural ectasias with scalloping of the vertebral bodies. Biopsy of the paravertebral mass lesion disclosed EHE. We present this case because of the novel association between NF-1 and EHE, and the unusual aggressiveness of the neoplasia. Additionally, we highlight the co-existence of bilateral and multiple lateral meningoceles. (orig.)
Full Text Available Aggressive natural killer cell leukemia (ANKL is a rare neoplasm of mature natural killer cells, with an extremely poor overall survival, which is almost always EBV related, with majority of cases reported in East Asia. Here we report the case of an ANKL presenting in a young Hispanic male with secondary hemophagocytosis. Aggressive clinical course, high EBV DNA levels and leukemic presentation, often with associated hemophagocytosis, should raise suspicion of an NK/T-cell neoplasm like ANKL. Due to significant diagnostic overlap with extranodal NK/T-cell lymphoma, nasal type (ENKL, accurate diagnostic classification is crucial due to differing treatment and prognosis. L-asparaginase including chemotherapy followed by allogeneic stem cell transplantation appears to slightly prolong overall survival, but relapse is almost inevitable. Clinical monitoring of EBV DNA levels shows good correlation with disease activity.
Full Text Available Primitive Neuroectodermal Tumor of the kidney is a rare entity. Very few cases of primary renal PNET have been reported to date. Most literature about rPNET is isolated case reports. We report a case of rPNET in a 39-year-old male with a pre-operative diagnosis of renal cell carcinoma with renal vein thrombosis. The patient underwent radical nephrectomy with thrombolectomy, and histopathological examination revealed a highly aggressive tumor composed of monotonous sheets of round cells. Tumor cells were positive for CD 99 and FLI-1, hence confirming the diagnosis of Primitive Neuroectodermal Tumor. Post-surgery, patient was given VAC/IE-based adjuvant chemotherapy. In view of highly aggressive nature of this tumor, prompt diagnosis and imparting effective chemotherapy regimen to the patient is required, and it is important to differentiate PNET from other small round-cell tumors because of different therapeutic approach.
Full Text Available Segmental odontomaxillary dysplasia (SOD is an uncommon, nonhereditary, rare developmental disorder primarily involving the posterior part of the maxilla and related dental components. It is a rare condition of uncertain etiology that results in painless unilateral expansion of the posterior dentoalveolar complex, gingival hyperplasia, lack of one or both premolars in the affected area, delayed eruption of adjacent teeth and malformations of the primary molars. Radiographically, the affected bone is thickened and irregular in outline, with a coarse trabecular pattern that is vertically oriented resulting in a relatively radioopaque granular appearance. The treatment is focused on extraction of the involved teeth, while in some milder cases the teeth may be retained for a long period. The treatment plan should be based on the degree of involvement as well as the functional and esthetic needs in each case. Considering the rarity of the condition, we report three cases of SOD with variable presentations in a 45-year-old, 24-year-old and 23-year-old individual.
Rao Uma NM
Full Text Available Abstract Background Primary testicular angiosarcomas are extremely rare, and their clinicopathologic features are not well described. Our objective was to further define the clinical features and pathologic spectra of primary testicular angiosarcomas. Methods Six previously reported case reports were identified in the English language medical literature using MEDLINE and a subsequent bibliographic search of all pertinent reports and reviews was performed. After excluding 2 cases because they did not involve the testis, we identified 4 previously reported cases of true primary testicular angiosarcoma. We also searched the electronic medical archival records of our institution and identified one additional unreported case of true primary testicular angiosarcomas. Data were extracted on the demographics, predisposing factors, clinical presentation, gross pathology, microscopic pathology, immunophenotype, therapy, and outcomes of each of these 5 cases of true primary testicular angiosarcomas. Results Primary testicular angiosarcomas were found at a mean age of 43.4 years. None of the cases was associated with exposure to radiation, arsenic, thorium dioxide, or vinyl chloride. However, 1 case was associated with hydrocele. It typically presented with painless mass (mean size, 6.3 cm. Histologically, all showed classic anastomosing channels lined by plump hyperchromatic cells, though most showed epithelioid cytology and some showed solid architectural pattern. One patient had multiple metastatic recurrences but eventual outcome was not available, and 1 patient died a month after diagnosis from stroke but no autopsy was performed. The remaining 3 patients were alive at the time of publication of their respective cases (mean, 17 months. Conclusion Primary testicular angiosarcomas are typically rare tumors of men of all ages that appear to segregate into 2 groups; one associated with teratoma and occurring in young people, and the other occurring in the
Bansal, Ankur; Kumar, Manoj; Sokhal, Ashok; Purkait, Bimalesh; Kanodia, Gautam
ABSTRACTBilateral single system ureteral ectopia (BSSEU) is an uncommon entity. Ureteric calculi in BSSEU are never reported so far. We herein report a case of BSSEU with left lower urtereic calculi managed by bilateral ureteric tapering and reimplantation with stone removal.
Praveen S Rathod
Full Text Available Primary hydatid cyst in the pelvis is rare, and usually presents with pressure symptoms affecting the adjacent abdominal organs. We describe a rare hydatid cyst which was eroding the sacral hallow, protruding into the right sciatic foramen and presenting as a radiating pain and weakness of right lower limb due to compression of the lumbosacral nerve roots. Laparotomy with removal of cyst and postoperative treatment with albendazole is effective in controlling the disease and preventing recurrence.
Full Text Available Although glandular and stromal proliferations of prostate are very common in adult men, neoplastic proliferations of prostatic stroma are distinctly uncommon. These tumors are now grouped as Prostatic Stromal Proliferations of Uncertain Malignant Potential (PSPUMP. Phyllodes tumor of the prostate is a rare neoplasm in this group with cellular, sarcomatoid stroma and benign hyperplastic glands. It is a locally expansile tumor with clinical course varying from benign to aggressive. We report a case of a 45-year-old man presented with retention of urine and abdominal lump. On laparotomy it was a huge tumor of 4 kg and was histologicaly characterized by cellular pleomorphic stroma and hyperplastic epithelium. Immunohistochemistry demonstrated prostate specific antigen in the glands. It was diagnosed as cystosarcoma phyllodes tumor of prostate. This is extremely uncommon tumor similar in histology to that of breast and it′s clinical course varies with the grade. The patient was without recurrence one year after surgery.
Sheik, Shahila; Al-Riyami, Nihal; Mathew, Namitha R; Al-Sukaiti, Rashid; Qureshi, Asim; Mathew, Mariam
A hydatidiform mole with a coexisting live fetus is a rare occurrence and the optimal management for this condition is not yet known. We report the case of a 32-year-old woman (gravida 3, para 2) who presented to the Sultan Qaboos University Hospital, Muscat, Oman, in March 2012 at 13 gestational weeks with abdominal pain and vaginal bleeding. An ultrasound examination revealed a hydatidiform mole pregnancy coexisting with a live fetus. After extensive counselling, the patient and her husband opted for a conservative management approach. Unfortunately, a hysterotomy had to be performed at 17 gestational weeks due to severe haemorrhage. The postoperative period was uneventful and histopathology results confirmed one complete mole with a coexisting fetus and normal placenta. The patient's serum β-human chorionic gonadotropin level remained normal for 18 months following her surgery.
Salati, Massimiliano; Orsi, Giulia; Reggiani Bonetti, Luca; Di Benedetto, Fabrizio; Longo, Giuseppe; Cascinu, Stefano
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract, and are characterized by a broad spectrum of clinical, histological and molecular features at presentation. Although focal and scattered calcifications are not uncommon within the primary tumor mass, heavy calcification within a GIST is rarely described in the literature and the clinical-biological meaning of this feature remains unclear. Cases with such an atypical presentation are challenging and may be associated with diagnostic pitfalls. Herein, we report a gastric GIST with the unusual presentation of prominent calcifications that was identified incidentally on imaging during a post-trauma diagnostic work-up. The patient underwent laparoscopic surgery with a radical resection of the mass, which was subsequently characterized by histological analysis as spindle-shaped tumor cells, positive for CD117/c-KIT, CD34 and DOG1, and with calcified areas. Given the intermediate risk of recurrence, no adjuvant therapy was recommended and the patient underwent regular follow-up for 22 mo, with no evidence of relapse. Our case can be considered of interest because of the rarity of clinical presentation and the uniquely large size of the GIST at diagnosis (longest diameter exceeding 9 cm). In closing, we discuss the pathophysiology and clinical implications of calcifications in GISTs by reviewing the most up-to-date relevant literature. PMID:28344749
Full Text Available Primary melanoma of the anal canal is rare and highly malignant condition, which is 1% of all invasive tumors in this site. This condition is often mistaken for benign conditions as either hemorrhoids or rectal polyp. Thyroid-stimulating hormone stimulation causes high proliferation of malignant melanoma. The association of hypothyroidism with primary malignant melanoma of anal canal is very rare. We are reporting such a very rare case.
Afsar, Fatma Sule; Ergin, Malik; Ozek, Gulcihan; Vergin, Canan; Karakuzu, Ali; Seremet, Sila
ABSTRACT Objective: To report a case of late-onset self-healing Langerhans cell histiocytosis. Case description: A 4½-month-old female patient presenting with an eythematopurpuric eruption underwent a skin biopsy for histopathology and was first diagnosed with isolated cutaneous Langerhans cell histiocytosis. Her lesions regressed within a few months and she was retrospectively diagnosed with late-onset self-healing Langerhans cell histiocytosis after being without skin or systemic involvement in a follow-up four years later. Comments: Self-healing Langerhans cell histiocytosis, which is characterized by clonal proliferation of Langerhans cells and presents with cutaneous lesions, is a rare self-limited variant of histiocytosis and can only be diagnosed retrospectively, after the patient remains free from systemic involvement for several years. Although it presents at birth or during the neonatal period, only a few cases of its late-onset type regarding the age of onset have been reported. Purpuric lesions that appear after the neonatal period serve as a clue for late-onset self-healing Langerhans cell histiocytosis and the patients should be monitored regularly for systemic involvement if the diagnosis is confirmed by a cutaneous biopsy.
Grecu, F; Filip, B; Moţoc, I; Andriescu, Nadia; Lăpuşneanu, A; Ursaru, Manuela
The Amyand hernia is an uncommon variant of the inguinal hernia, rarely recognised before the surgical treatment because of the confusion with a strangled hernia. In spite of this, the clinical presentation seems to follow a well determined pathway, so it is possible to state that the uncorrect diagnosis is to be attributed to the ignorance of this variant of hernia. We present two consecutive case reports of acute appendicitis founded in an inguinal hernia sac. The clinical presentation depended on the inflammation extension inside the hernia sac and the presence or not of peritoneal contamination. The patients were admitted for a painful pseudotumor in the inguinal region with irreducibility, mimicking strangled inguinal hernia with acute inflammatory syndrome. Intraoperatively we have found a hernia sac with a phlegmonous/gangrenous appendix inside. Appendectomy was performed, followed by hernioplasty (retrofunicular technique) without prosthetic material). The operation followings were favorable. We conclude that amyand hernia must be considered as differential diagnosis of apparently strangled inguinal hernias. Technical precautions and antibioprophylaxy applied during surgery may prevent septic complications after hernioplasty. The hernia repair must be performed without prosthetic material and using exclusively resorbable sutures.
Full Text Available We describe a case presenting complaint of complete lower body paraparesis, which was discovered to have splenic marginal zone lymphoma (SMZL. While paraneoplastic syndromes are more common in tumors, such as small cell lung cancer, very few reports exist on this condition with SMZL. We describe such a rare entity with a clinical course spanning twenty-four months after diagnosis.
Patel, Hiten Mohanbhai; Banerjee, Shubhabrata; Bulsara, Shahzad; Sahu, Tapish; Sheorain, Virender K; Grover, Tarun; Parakh, Rajiv
Aberrant right subclavian artery is an uncommon entity incidence ranging from 0.5 to 2.5%. Management of thoracic aortic injury in the presence of such anomalies can be a challenge. We present here a case of traumatic aortic injury, which was incidentally found to have an asymptomatic aberrant right subclavian artery. The patient was managed by an endovascular repair of thoracic aortic injury with an endograft and a right carotid to subclavian artery bypass as a hybrid procedure. A 40-year male patient was brought to the emergency in shock with an alleged history of road traffic accident an hour back. After initial resuscitation as per advance trauma life support protocol, imaging revealed thoracic aortic injury with aberrant right subclavian artery with multiple rib and bilateral humerus fracture. After primary stabilization of arm fractures, the patient was shifted to a hybrid operation room. As the aortic injury was within 10 mm of the origin of both subclavian arteries, it was decided to cover the origin of both subclavian arteries and land the endograft distal to the left carotid artery origin. Since there was a right dominant vertebral artery on imaging, right carotid to right subclavian artery bypass was done with expanded polytetrafluoroethylene graft to prevent posterior circulatory stroke along with thoracic endovascular aortic repair to seal the thoracic aortic injury. After endovascular repair of thoracic aortic injury, left subclavian artery perfusion was maintained through left vertebral artery; and hence, revascularization of left subclavian artery was deferred. After management of all fractures, the patient was discharged 3 weeks after the date of admission without any complications. At 6 months follow-up, patient was stable and images showed patent bypass graft and sealed aortic injury. In a trauma setting with multiple injuries, hybrid procedure with a thoracic endograft is associated with low mortality and morbidity; hence, it is the treatment
Mahesh Kumar Mittal
Full Text Available Extramedullary plasmacytoma (EMP is a rare plasma cell neoplasm of soft tissue without involvement of bone marrow or other systemic characteristics of multiple myeloma. It accounts for only 4% of all plasma cell tumors. We have reported herein a rare case of EMP involving soft tissues of the neck.
Travis William D
Full Text Available Abstract Primary lung myoepithelial carcinomas are rare neoplasms arising from the salivary glands of the respiratory epithelium. Given the rare occurrences and reports of these tumors, appropriate recommendations for resection are difficult to formulate. Although classified as low-grade neoplasms, these tumors have a significant rate of recurrence and distant metastasis.
Asma, A Affee; Kannah, E
Schwanommas arising from cervical sympathetic chain are tumours that are rare in occurrence. These lesions are usually difficult to differentiate from a vagal schwanomma and a carotid body tumour during the initial workup. In this report, a rarely seen huge cervical sympathetic chain schwanomma case with partial Horner's syndrome is being presented in detail, which to our known knowledge, is one of the few cases reported in literature.
Uner, Meral; Saglam, Arzu; Meydan, Bilge Can; Aslan, Kerim; Soylemezoglu, Figen
Atypical teratoid rhabdoid tumor (AT/RT) is a rare and aggressive tumor usually occurring at younger ages. Pleomorphic xanthoastrocytomas (PXA) on the other hand are quiescent tumors with benign behavior. AT/RTs arising in the setting of PXA are exceptional. We present the case of a 23-year-old female patient, the fourth in the literature, speculated as having AT/RT arising within a PXA, as demonstrated by the presence of INI1 mutation. The patient presented with a short history of headache, which increased over time, and emerging seizures. She had a contrast-enhancing mass in the left temporal area demonstrated by MRI. Pathological examination demonstrated a dimorphic tumor containing a spindle-pleomorphic component reminiscent of PXA and a rhabdoid component with INI1 loss showing features of AT/RT. Both components shared the same BRAF mutation, supporting their common origin, and hence the case was speculated as an AT/RT arising in the setting of a PXA by secondary genetic change of inactivation of INI1. She had a poor outcome despite surgery and died 8 months after her diagnosis. .
Full Text Available Abstract Mammary sarcoma is extremely rare and the diagnosis is established only after metaplastic carcinomas and malignant phyllodes tumours are excluded. A rare case of not otherwise specified-type sarcoma with CD10 expression in the left breast in a 45-year-old female was presented. It was a high-grade tumour composed of spindle cells histologically. The immunohistochemical results showed that CD10, vimentin and EGFR were positive diffusely and SMA presented focally, whereas epithelial markers and other myoepithelial or myogenic markers were all negative. The electron microscope investigation demonstrated fibroblast-like features. The exact entity of the tumour remains to be studied because it resembles undifferentiated sarcoma or sarcomatoid metaplastic carcinoma to some degree, as well as high-grade malignant phyllodes tumour in particular. Virtual slides The virtual slide(s for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/9019879588725702
Full Text Available Mucosa associated lymphoid tissue (MALT lymphomas are rare neoplasms. They are most common in the stomach followed by small intestine and colon. The symptoms are nonspecific and generally do not present with intussusception. Here we report a rare clinical entity in which a 35-year-old female presented to the emergency with severe abdominal pain which was sudden in onset. History revealed that she had been having vague mild abdominal pain for 2 years. Ultrasonography showed ileocolic intussusception with hypoechoic lesion of 54 × 46 mm seen at the lead point. Emergency laparotomy with the right hemi-colectomy was done. The specimen was sent for histopathological examination which revealed a diagnosis of MALT lymphoma. Awareness of the varied clinical presentation helps in formulating the appropriate therapeutic strategy.
Full Text Available Odontomas are the most common odontogenic tumors. They are usually asymptomatic and are often discovered during routine radiography. Eruption of an odontome into the oral cavity is rare. Odontomas are the most common odontogenic tumors. They are usually asymptomatic and are often discovered during routine radiography. Eruption of an odontome into the oral cavity is rare. We report an unusual case of erupting compound composite odontoma. we report an unusual case of erupting compound composite odontoma.
Bilreiro, Carlos; Bahia, Carla; Castro, Miguel Oliveira e
We present the case of an 80 year old woman with hip pain, caused by a longitudinal femoral insufficiency stress fracture, depicted with radiographs, CT and MR. This type of fracture is very rare, with only a few cases reported. We conducted a literature review and compared the findings with the present case. PMID:27069976
Full Text Available Urachus fistulas are rare, especially in adulthood. In grown-ups urachus fistulas are usually a reflection of Crohn’s disease. We present a patient in whom an urachus fistula was the first presentation of diverticulitis of the sigmoid colon. The need for proper preoperative diagnostic imaging is discussed.
Full Text Available Peripheral primitive neuroectodermal tumor (PNET is an uncommon tumor and the overall incidence is 1% of all sarcomas. PNET of the adrenal gland is an even rarer entity. A 37-year-old female was evaluated for an episode of loin pain. Ultrasonography showed a large heterogenous left adrenal mass with internal echogenic components. Computed tomography did not show any fat density within to suggest a myelolipoma. Biopsy suggested a poorly differentiated neoplasm with a possibility of PNET of the adrenal gland.
Comer, John D; Cui, Xiaoyan; Eisen, Carolyn Sharyn; Abbey, Genevieve; Arleo, Elizabeth Kagan
A 73-year old man with a history of multiple genitourinary malignancies was found to have a left retroareolar soft tissue mass on CT assessment of disease, and dedicated breast imaging was recommended. Diagnostic mammography and ultrasonography confirmed a solid mass, for which biopsy was recommended. Pathologic analysis demonstrated a spindle cell neoplasm with an immunoreactivity pattern consistent with myofibroblastoma. While this entity is benign, nonspecific imaging features necessitate tissue sampling for pathologic diagnosis, and, given pathologic rarity, open communication between the radiologist and pathologist is important to establish the correct diagnosis and to recommend appropriate management. Copyright © 2016 Elsevier Inc. All rights reserved.
Siddiahgari, Sirisha Rani; Yerukula, Pallavi; Lingappa, Lokesh; Moodahadu, Latha S
Extra nodal presentation of Non Hodgkins Lymphoma (NHL) is a rare entity, and data available about the NHL that primarily involves of middle ear and mastoid is limited. We report a case of diffuse large B cell lymphoma (DLBCL), in a 2 year 8 month old boy, who developed otalgia and facial palsy. Computed tomography revealed a mass in the left mastoid. Mastoid exploration and histopathological examination revealed DLBCL. This case highlights the importance of considering malignant lymphoma as one of the differential diagnosis in persistent otitis media and/facial palsy.
Sachdeva, Pallavi; Minocha, Priyanka; Jain, Rohit; Sitaraman, Sadasivan; Goyal, Manisha
Proteus syndrome (PS) is an extremely rare and complex disorder. Approximately 200 cases have been reported, and it seems to affect people of all ethnic and racial groups. PS is characterized by segmental overgrowth of multiple tissues and organs including vascular malformations, lipomatous overgrowth, hyperpigmentation, and various types of nevi. We hereby present a 7-year-old boy who presented with seizures and overgrowth of one-half of the body. Although classical physical features have been described, epilepsy and other neurological manifestations are rarely reported features of PS. Early detection of association of epilepsy and hemimegalencephaly with PS can prevent/minimize the neurological complications, disability, morbidity, and mortality. PMID:28553400
Mihalek, Andrew D; Haney, Carissa; Merino, Maria; Roy-Chowdhuri, Sinchita; Moss, Joel; Olivier, Kenneth N
Amyloid primarily affecting the lungs is a seldom seen clinical entity. This case discusses the work-up of a patient presenting with exercise-induced haemoptysis and diffuse cystic lung disease on radiographic imaging. The common clinical and radiographic findings of diffuse cystic lung diseases as well as a brief overview of pulmonary amyloid are presented. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/
Chandrakant Patil; Rashmi Kharat Patil; Prasad Deshmukh; Jyotirmoy Biswas; Bassin John
Tuberculosis has global presence and no part of human body is immune to it, most frequent site beings lungs.Nasopharyngeal tuberculosis is a rare type of extrapulmonary tuberculosis comprising only less than1% of tuberculosis found in the upper respiratory tract.The authors are presenting here a case of primary tuberculousis affecting the nasopharynx (adenoids) which is one of the rare differential diagnosis of nasopharyngeal mass.Isolated nasopharyngeal tuberculosis is a rare condition even in the endemic areas.In literature there are varied clinical presentations of nasopharyngeal tuberculosis.Tuberculosis should be one of the differential diagnosis of nasopharyngeal lesion.Biopsy and histologic study should be performed in every patient to avoid misdiagnosis.When treated properly, nasopharyngeal tuberculosis carries a excellent prognosis,and complete resolution of disease is the rule.
Full Text Available In this article we proposed to achieve a synthesis of the key moments in the evolution of the concept of balance, of the theories formulated over time, of the presentation forms of the balance sheet, a taxonomy of balance sheets, supplemented by a breakdown of the concept of financial position and of the structures related to the assessment of the financial position. In other words, we will consider a gradual presentation of the concept of balance sheet over time and a presentation of the balance as the main tool for highlighting the patrimonial situation, of the assets of enterprise owners and of the picture on the financial position of an economic entity.
Mohit K Joshi
Full Text Available Skeletal metastasis as a primary presentation of gall bladder carcinoma is rare. A 50-year-old lady presented with neck pain and weakness in her right upper limb of 3 months duration. Clinical and imaging work-up suggested locally advanced gall bladder carcinoma with metastasis to cervical vertebra and sternum. Only one case till date has been reported where the patient presented with neurological symptoms due to pathological fracture secondary to metastasis from an occult gall bladder carcinoma. Although rare, an occult gall bladder cancer may present with neurological symptoms due to pathological fracture of spine secondary to metastasis. We present a brief review of literature of patients who presented with skeletal metastases in clinically silent gall bladder malignancy. Palliative care issues in advanced gall bladder carcinoma have also been discussed.
Guray Togral; Murat Arikan; Elif Aktas; Safak Gungor
Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare low-grade, malignant soft tissue tumor that is usually observed in the extremities of adult patients. Magnetic resonance imaging findings for this tumor type have rarely been reported. We report a case involving the distal left femur of a middle-aged man and tumoral invasion of the bone, which, to our knowledge, has been previously described only once. He was treated with distal femoral tumor resection and reconstruction with a modular prosthesis. Histopathologic diagnosis confirmed MIFS. We reviewed literature of the diagnostic imaging and bone invasion findings associated with this tumor type.
Mrelashvili, Anna; Braksick, Sherri A; Murphy, Lauren L; Morparia, Neha P; Natt, Neena; Kumar, Neeraj
Rathke's cleft cysts (RCC) are usually benign, sellar and/or suprasellar lesions originating from the remnants of Rathke's pouch. Rarely, RCC can present with chemical meningitis, sellar abscess, lymphocytic hypophysitis, or intracystic hemorrhage. We describe an unusual presentation of RCC in which the patient presented with a clinical picture of chemical meningitis consisting of meningeal irritation, inflammatory cerebrospinal fluid profile, and enhancing pituitary and hypothalamic lesions, in addition to involvement of the optic tracts and optic nerve.
Nadia Shirazi; Saurabh Varshney; Meena Harsh; S. S Bisht
Schwannomas are benign neoplasms arising from Schwann cells of the peripheral, cranial and autonomic nerves. We report a case of schwannoma in the sino-nasal tract, a very rare site of tumour origin with unusual pseudoangiomatous histopathological changes, which we came across in a 22 years male with progressive nasal obstruction.
Full Text Available Intraosseous mucoepidermoid carcinoma of jaw bones is a rare lesion. Abundance of clear cells in an intraosseous mucoepidermoid carcinoma may complicate its histopathologic diagnosis. It becomes extremely important to distinguish this lesion from other clear cell lesions of jaw region. Here, we report a case of clear cell variant of intraosseous mucoepidermoid carcinoma in the mandible.
Full Text Available Presenting a 47-year-old peri-menopausal lady with hypomenorrhoea, temporal baldness, alopecia, hirsutism. The histopathology was polycystic ovaries with stromal hyperthecosis. Hughesdon described hyperthecosis as a severe form of PCOS. Hyperthecosis is rare in young women, with clinical features similar to PCOS. However, these women are usually more virilised.
Ekrem Cengiz Seyhan
Full Text Available Synovial sarcoma (SS is a rare tumor originating from mesenchymal tissue and accounting for approximately 5–10% of all soft tissue sarcomas. A rare case of primary pulmonary SS in an asymptomatic 18-year-old man admitted to our hospital for investigation of a 6 × 6.5 cm, oval-shaped, well-delineated pleural based peripheral mass in the left lower lobe in his thorax CT is presented. Left lower lobectomy was done. Immunohistochemically, tumor cells were positive for cytokeratin, epithelial membrane antigen (EMA, and vimentin so that the histopathological diagnosis was compatible with biphasic spindle cell type SS in the lung.
Full Text Available Moyamoya disease is a rare, chronic cerebrovascular occlusive disease of unknown etiology. It is characterized by progressive stenosis of the arteries of the circle of Willis leading to ischemicstrokes in young people and cerebral hemorrhage, which is more frequent in adults. Secondarily,an abnormal network of fine collateral vessels arises at the base of the brain. The term moyamoyarefers to the angiographic appearance of the cerebral vasculature. We present such a disease in an 18-month-old Iranian girl with global developmental delay, which is a very rare presentationof moyamoya disease. She was diagnosed by magnetic resonance imaging (MRI and magnetic resonance angiography (MRA.
Full Text Available Polymorphous low-grade adenocarcinoma is a rare type of minor salivary gland malignancy. The characteristic features of these tumors are the varied histomorphology and the malignant, albeit indolent behavior. It occurs commonly in the minor salivary glands, with the palate (58.5% being the most common intra oral site. Maxillary area (2%, mandibular mucosal area (1.5% and posterior trigone region (0.5% are the least affected areas. An occasional case has been reported arising from an intraosseous location i.e the maxilla, and only two cases have been reported in the English literature originating from the mandible. One such very rare case of polymorphous low-grade adenocarcinoma of the mandible, which radiographically has a soap bubble appearance, is reported here.
Full Text Available A rare case of acinic cell carcinoma of minor salivary gland with cervical lymph node metastasis in a 50-year-old man is reported and the literature regarding this type of tumor is reviewed. These tumors arise from either an intercalated duct stem cell or the reserve cell of the salivary gland terminal tubule but not from both simultaneously. Rarely these neoplasms arise from more mature acinar cells. It is clear that these tumors behave ominously. The 25 year determinate survival rate is 50%, with a 20% incidence of metastasis. Surgical excision is the treatment of choice. Radiotherapy, especially neutron therapy, has a place in the treatment of this tumor but the role of chemotherapy is not exactly known at this time.
Full Text Available Frontotemporal dementia is an important neurodegenerative disorder accounting for a significant proportion of dementia cases with onset before 60 years of age. Apart from the well recognized behavioral changes the disease has many other distinctive features like predominant language involvement alone or associated features of motor neuron disease or parkinsonism etc. which at times may be the presenting manifestation itself. In the following article we describe a rare presenting manifestation; prosopagnosia, in the setting of frontotemporal degeneration
Shende, Prajakta; Gandhewar, Manisha; Gaikwad, Pradip; Nanaware, Sandip; Risbud Joshi, Prachi
Tuberculosis (TB), being a global health problem, represents variedly. Its presentation as a labial swelling secondary to pubic bone TB has been reported rarely in literature. We report a case of pubic bone TB presenting as a labial swelling in a woman of reproductive age. Early diagnosis with fine needle aspiration cytology, acid-fast bacillus (AFB) staining, AFB culture and magnetic resonance imaging with early initiation of treatment resulted in a favourable outcome.
Park, Jong Chun; Chae, Eun Jin; Song, Joon Seon [University of Ulsan College of Medicine, Asan Medical Center, Seou (Korea, Republic of)
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described, very rare type of fibrotic interstitial lung disease predominantly involving subpleural areas of both upper lungs. IPPFE has distinctive radiologic and pathologic features: progressive subpleural opacity with fibrotic changes, predominantly in upper lungs, and dense elastic component on histology. We experienced one case of surgically confirmed IPPFE, with progression of radiologic findings on the serial CT examinations. We herein report the characteristic radiologic features of IPPFE with pathologic and clinical manifestations.
Arslan, Yusuf; Altintoprak, Fatih; Serin, Kursat R; Kivilcim, Taner; Yalkin, Omer; Ozkan, Orhan V; Celebi, Fehmi
Ectopic liver tissue (ELT) is a rare condition, which is usually not diagnosed preoperatively, but coincidentally during abdominal surgery. While the location of ELT can vary, it is usually localized on the gallbladder wall or in close proximity. ELT is associated with various complications, a major complication being extrahepatic hepatocellular carcinoma. A 59-year-old female underwent elective surgery for chronic cholecystitis with stones. During laparoscopic exploration, a 2-cm-diameter EL...
Full Text Available Background. Pulmonary arterial dissection with chronic pulmonary arterial hypertension as its major cause is a very rare but life-threatening condition. In most cases the main pulmonary trunk is the affected site usually without involvement of its branches. Segmental or lobar pulmonary artery dissection is extremely rare. Case Presentation. We report a unique case of left lower lobe pulmonary artery dissection in a 70-year-old male, with confirmed chronic pulmonary hypertension. To confirm dissection MDCT pulmonary angiography was used. Multiplanar reformation (MPR images in sagittal, coronal, oblique sagittal, and curved projections were generated. This case report presents morphologic CT features of rare chronic left lobar pulmonary artery dissection associated with chronic pulmonary hypertension at a place of localised pulmonary artery calcification. CT pulmonary angiography excluded signs of thromboembolism and potential motion or flow artefacts. Conclusion. To the best of our knowledge, no case of lower lobe pulmonary artery dissection with flap calcification has been reported yet. CT imaging of the chest is a key diagnostic tool that is able to detect an intimal flap and a false lumen within the pulmonary arterial tree and is preferred in differential diagnosis of rare complications of sustained pulmonary arterial hypertension.
Jennifer C. Kam
Full Text Available Giant sigmoid diverticulum (GSD is a rare complication of diverticulosis. These lesions arise from herniations of the mucosa through the muscle wall which progressively enlarge with colonic gas to become large air-filled cysts evident on plain X-ray and CT scans. We present a rare case of a 72-year-old female presenting with abdominal distention, abdominal tenderness, and fever who developed a type 1 giant sigmoid diverticulum (pseudodiverticulum that subsequently formed an intra-abdominal abscess and an accompanying type 2 diverticulum as well. The patient was treated with surgical resection of the diverticulum with a primary anastomosis and abscess drainage. The patient’s postoperative course was uneventful. This case helps to support the need for the consideration of GSD in patients aged 60 and older with a history of diverticulosis and presenting with abdominal discomfort and distension.
Wang, Bonnie; Moon, Seong-Jin; Olivero, William C; Wang, Huan
Cerebral venous thrombosis (CVT) remains a diagnostic and therapeutic challenge for clinicians. Manifesting in a remarkably wide spectrum of symptoms and signs, CVT often presents in a misleading fashion-if unrecognized or misdiagnosed, it carries potentially fatal consequences. Visual loss is quite rare as the initial presentation of CVT and is typically a finding more frequent in chronic cases with associated papilledema on funduscopy Ferro, Lopes, Rosas and Fontes (Delay in Hospital Admission of Patients with Cerebral Vein and Dural Sinus Thrombosis. Cerebrovasc Dis 2005;19: :152-6). We report a rare case of acute cortical blindness as the initial presentation of CVT in an 18-year-old female patient and review the current literature.
Sattar, Sidra; Akhunzada, Naveed Z.; Javed, Gohar; Uddin, Zeeshan; Khan, Yasir A.
Background: Pilocytic astrocytoma (PA) is the most prevalent central nervous system (CNS) tumor in pediatric population and accounts for an approximate of 5–6% of all gliomas. This neoplasm can occur at all levels of the neuraxis, with majority (67%) arising in the cerebellum and optic pathway. PAs are World Health Organization Grade I tumors and are the most benign of all astrocytomas characterized by an excellent prognosis. Other differentials include subependymal giant cell astrocytoma (SEGA), ependymoma, meningioma, and low-grade gliomas such as pilocytic or diffuse astrocytoma; calcification is more commonly regarded as a feature of benign or slow-growing tumors. Case Description: We present a case of a 17-year-old female presenting with an unusual cause of hydrocephalus, a rare case of a calcified pilocytic astrocytoma as an intraventricular tumor. Conclusion: PA rarely presents as an intraventricular tumor and should be included in the differential diagnosis of a large mass with massive intratumoral calcification. PMID:28680735
Pradipta Ghosh; Katsumi Miyai; Mario Chojkier
Advanced gastric cancer usually presents with symptoms due to direct extension into adjacent viscera, distant metastases from lymphatic or hematogenic dissemination and peritoneal seeding. However, portal hypertension as a presentation of metastatic gastric cancer is rare and usually seen in association with other malignancies, e.g. hepatocellular and pancreatic carcinoma. We report a case of signet ring adenocarcinoma of the stomach that presented with esophageal and duodenal varices and bleeding due to portal hypertensive gastropathy. Pagetoid spread of cancer cells likely caused early metastasis and the unusual presentation. We also discussed the pathophysiology of development of portal hypertension in association with malignancies.
Aleem, Mohammed Abdul; Fatima, Ather; Kumudachalam, P; Priyadarshini, Ramya
Nasal angiomyolipoma (AML) are extremely rare tumors and so far <15 cases have been reported in the literature, and this is the first instance that Nasal AML is reported from India. We report two cases of AML arising in the nasal cavity described in 60-year-old male and 50-year-old female patient. Grossly, they were well circumscribed, lobulated masses, and microscopically, they were composed of an intimate mixture of mature fat, smooth muscle cells, and thick-walled varying sized blood vessels. Immunoexpression pattern and histopathology were characteristic. Both the patients had complete resolution of symptoms after endoscopic excision of the tumor.
Chiummariello, S; Figus, A; Menichini, G; Bellezza, G; Alfano, C
Scrotal calcinosis (SC) is a rare benign disease that affects patients in childhood or early adulthood. It is characterized by slow-growing yellowish-white nodules consisting of deposits of calcium and phosphates, within the scrotal skin. The nodules vary in number, and can be solitary or grouped. Owing to the age of onset and anatomical location, SC may be a source of embarrassment and lead to social isolation. Because of its rarity, the aetiology of SC is still controversial. We report a very rare case of an SC in a 59-year-old white man who presented with multiple nodules with different clinical patterns in the scrotum, which had been present for > 42 years. Despite the rarity and the multiple long-lasting lesions, surgical excision of the scrotal nodules can offer a very good aesthetic outcome in a single procedure even under local anaesthesia.
Full Text Available Pheochromocytoma is a rare tumor in which seen an incidence of 1 per 100.000 in the general population. Pheochromocytoma is a catecholamine producing neuroendocrine tumor arising from adrenal medulla. A 46-year-old man experienced headache, sweating and palpitation referred to our hospital. Familial pheocromocytoma was detected. We presented this case as whole family members had suffered from morbidities and deaths due to this disorder. [Cukurova Med J 2015; 40(3.000: 593-596
Nisahan, Balasingam; Thirunavukarasu, Kumanan; Selvaratnam, Gowry
Typhoid fever can cause a number of renal manifestations heretofore dubbed 'nephrotyphoid'. Haematuria in the absence of renal impairment is extremely rare among typhoid patients. We report a case of an adult who presented with a prolonged febrile illness and microscopic haematuria. Blood culture confirmed the diagnosis of typhoid and the patient was treated successfully with ceftriaxone. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.
Full Text Available Prostatic abscess is a rarely described condition and is commonly caused by gram-negative organisms such as enterobacteria. However, as the prevalence of methicillin resistant Staphylococcus aureus (MRSA increases in the community, unusual infections due to this organism have been recently published. In this report, we describe a patient with diabetes mellitus type 2, who presents with diabetic ketoacidosis—later found to be due to a prostatic abscess from which MRSA was cultured.
Full Text Available Lyme disease is a common disease that is faced by the physician but also acts a mimicker of many other disease processes. Facial palsies, especially bilateral, are a relatively rare presenting symptom of Lyme disease and may warrant further investigation. A thorough history and physical examination coupled with precision testing may aid the physician when faced with a patient with the diagnostic dilemma of facial diplegia.
Sharma, Gaurav; Jain, Kanu; Nagpal, Archna; Baiju, Chandrababu Sudha
Lipoma is the most common tumor of mesenchymal tissues of body, but its occurrence in oral cavity is infrequent. Buccal mucosa is the most common intraoral site of lipoma followed by tongue, floor of the mouth, and buccal vestibule. The involvement of mucogingival junction is rare. We present a unique case report of oral lipoma occurring on mandibular mucogingival junction with review of literature which has emphasis on differential diagnosis. PMID:27143835
Cox, Deven; Ching, Brian H.
Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), also known as Herlyn-Werner-Wunderlich syndrome, is a rare syndrome with only a few hundred reported cases described since 1922. Only a handful of these cases have been associated with pyocolpos. Mullerian duct anomalies have an incidence of 2–3%. While OHVIRA constitutes 0.16–10% of these Mullerian duct anomalies. Symptoms usually present shortly after menarche when hematocolpos develops during menstruation resulting in dysmenorrh...
Full Text Available Spontaneous rupture of the spleen with hemoperitoneum is a very rare, but serious manifestation of dengue fever (DF. We report a case of a young female who was presented with atraumatic abdominal pain, hypovolemic shock, anemia, ascites and hepatosplenomegaly with a recent history of a febrile illness. Subsequent investigations proved the presence of hemoperitoneum with spontaneous splenic rupture with seropositivity for DF. Early diagnosis and conservative management in this case resulted in a favorable outcome.
Two thirds of patients complain of bone pain, especially lower back pain. MM could be diagnosed after a pathologic fracture occurs in one third of patients. Presentation with symptoms related to hyperviscosity, hypercalcemia and bleeding tendency could also be observed. A rare presentation of MM is peri-orbital ecchymotic lesion (raccoon eye. Here, we report a 64 years old, male patient presented with unilateral raccoon eye and high erythrocyte sedimentation rate (ESR to internal medicine outpatient. The patient was referred to hematology outpatient and was diagnosed with multiple myeloma.
Hariharanadha Sarma Nandyala
Full Text Available Presence of lobules of adipose tissue either focally or diffusely is very rare in the thyroid gland. Fat accumulation can be macroscopic or microscopic. Focal infiltrates of fat have been reported in conditions such as adenolipoma, intrathyroid lipoma, and encapsulated papillary carcinoma. Diffuse lipomatosis has been reported in conditions such as amyloid goitre, heterotopic fat nests, thyrolipoma and liposarcoma. The exact mechanism of fat accumulation is not known although there are many theories postulated. Investigations such as ultrasound, computed tomography scan, and magnetic resonance imaging can detect the presence of macroscopic fat in the thyroid gland. Accurate diagnosis of the type of fat accumulation is necessary because tumorous and nontumorous conditions fall into the differential diagnosis. Only nine cases of papillary carcinoma associated with lipomatosis of thyroid are reported so far. We report possibly the first case of diffuse lipomatosis of the thyroid gland with a focus of papillary microcarcinoma.
Kruti D Dave
Full Text Available Acute bilateral renal cortical necrosis following acute pancreatitis is extremely rare condition. Among all cases of acute renal failure, the incidence of renal cortical necrosis was 3.8% in one of the study in North India. Till date, only eight cases of bilateral cortical necrosis following acute pancreatitis were reported in the literature. We report a case of a 27-year-old male patient with abdominal pain, nausea and vomiting since 2 days duration and anuria since 24 h. Serum amylase and lipase were raised, and elevated serum creatinine was noted on admission. Contrast-enhanced computed tomography scan of the abdomen revealed changes of acute pancreatitis with hypoenhancement of renal cortex compared to medulla on both sides (reverse rim sign-stronger enhancement of the renal medulla compared to cortex, suggest an acute renal cortical necrosis.
Konoglou, Maria; Cheva, Aggeliki; Zarogoulidis, Paul; Porpodis, Konstantinos; Pataka, Athanasia; Mpaliaka, Aggeliki; Papaiwannou, Antonios; Zarogoulidis, Konstantinos; Kontakiotis, Theodoros; Karaiskos, Theodoros; Kesisis, Georgios; Kolettas, Alexander; Giouleka, Alina; Madesis, Athanasios; Vretzakis, George; Sakkas, Leonidas; Tsakiridis, Kosmas
Epithelial-myoepithelial tumors of the lung are rare neoplasms whose biological behavior and clinical course still remain to be defined. Epithelial-myoepithelial carcinoma (EMCa) is a low-grade malignant tumour. According to literature, most commonly occurs in salivary glands, particularly in parotic gland, but it can also occur in unusual locations such as breast, lachrymal gland, nose, paranasal sinus, lung, bronchus and, as in our case, trachea. There are no many documented case reports of a primary myoepithelial carcinoma in the trachea. We report a case of a 34-year-old man diagnosed with this unusual location of an epithelial-myoepithelial tumor. The tumour was removed by segmental tracheal resection and end-to-end anastomosis.
Nandyala, Hariharanadha Sarma; Madapuram, Srinivasulu; Yadav, Megha; Katamala, Sudheer Kumar
Presence of lobules of adipose tissue either focally or diffusely is very rare in the thyroid gland. Fat accumulation can be macroscopic or microscopic. Focal infiltrates of fat have been reported in conditions such as adenolipoma, intrathyroid lipoma, and encapsulated papillary carcinoma. Diffuse lipomatosis has been reported in conditions such as amyloid goitre, heterotopic fat nests, thyrolipoma and liposarcoma. The exact mechanism of fat accumulation is not known although there are many theories postulated. Investigations such as ultrasound, computed tomography scan, and magnetic resonance imaging can detect the presence of macroscopic fat in the thyroid gland. Accurate diagnosis of the type of fat accumulation is necessary because tumorous and nontumorous conditions fall into the differential diagnosis. Only nine cases of papillary carcinoma associated with lipomatosis of thyroid are reported so far. We report possibly the first case of diffuse lipomatosis of the thyroid gland with a focus of papillary microcarcinoma.
Verma, Rajesh; Bhandari, Aveg; Tiwari, Navin; Chaudhari, Tejendra S
Wilson disease (WD) is one of the few inherited but treatable disorder mainly affecting the liver and brain resulting in severe disability or death if left untreated. Hence, it is important to keep a high index of suspicion for diagnosing this clinical entity in appropriate clinical settings. The clinical presentation can be quite variable and they may present solely with neurological features sans hepatic symptoms. Such neurological manifestations usually follow subacute to chronic course. Acute onset anarthria as the heralding and predominant presenting feature has been rarely reported in the literature. We reported a case of a 12-year-old girl who presented with acute onset anarthria and dystonia of 1-month duration. On further evaluation, a diagnosis of WD was made. The patient showed partial improvement after she was started on copper chelating agents and anticholinergics.
Jessica Leigh Baumann
Full Text Available Enteric duplication cysts are rare congenital malformations that can occur at any point along the digestive tract, most commonly the small bowel. They are characterized by the presence of an outer layer of smooth muscle and an inner lining of mucosa that may resemble any portion of the digestive tract. Less commonly, cases have been reported that also contain mucosal components of nonintestinal origin. This entity is typically diagnosed in young children, but occasionally presents in adolescence and young adulthood. We present a rare case of a 21-year-old male who presented with nonspecific symptoms of abdominal discomfort and weight loss and was later found to have a 9 cm nonenhancing mass in the distal ileum on CT imaging. Laparoscopic dissection of the mass revealed a cystic lesion lined mainly by pseudostratified ciliated columnar respiratory-type epithelium, with patchy areas of squamous epithelium as well as villous columnar epithelium resembling small bowel. The unique histology and advanced patient age make this case a unique presentation of what is already a rare pathological entity.
Full Text Available Autoimmune diseases are known to have association with each other but it is very rare to see multiple autoimmune diseases in one patient. The combination of at least three autoimmune diseases in the same patient is referred to as multiple autoimmune syndrome. The case we are reporting features multiple autoimmune syndrome with five different conditions. The patient had type 1 diabetes mellitus, autoimmune hemolytic anemia, systemic lupus erythematosus, vitiligo, and psoriasis. Psoriasis has rarely been reported previously under the spectrum of autoimmune syndrome. Although the relationship of autoimmune conditions with each other has been explored in the past, this case adds yet another dimension to the unique evolution of autoimmune pathologies. The patient presented with a combination of five autoimmune diseases, which makes it consistent type three multiple autoimmune syndromes with the addition of psoriasis. The current case is unique in this aspect that the combination of these five autoimmune disorders has never been reported in the past.
Singh, Neha; Nayak, Hemanta K; Bagchi, Avishek; Kar, Premashis
Poorly differentiated neuroendocrine tumour in the periampullary region of the duodenum is a rare entity. This entity usually present in old men. Here we report a periampullary poorly differentiated neuroendocrine cancer (PDEC) of duodenum presenting in a young man with subacute history of jaundice, abdominal pain, pancreatitis and constitutional symptoms. MRI localised the tumour and endoscopy-guided biopsy of the lesion proved the diagnosis. Although palliative surgery and chemotherapy were planned, the patient opted to leave against medical advice.
Bhat, Manzoor A; Laway, Bashir A; Allaqaband, Faheem A; Kotwal, Suman K; Wani, Imtiyaz A; Banday, Khursheed A
Sheehan's syndrome occurs as a result of ischemic pituitary necrosis secondary to severe postpartum bleeding. It is one of the most common causes of hypopituitarism, characterized by variable clinical presentation. Acute kidney injury occurs rarely in Sheehan's syndrome and most of the cases have been found to be precipitated by rhabdomyolysis. We here present a case of Sheehan's syndrome with acute kidney injury where theprecipitating cause was chronic hypocortisolemia. We believe this is the first reported case of Sheehan's syndrome in which acute kidney injury was precipitated by adrenal insufficiency.
Bahrani, Saeideh; Tabrizi, Nasim; Moein, Houshang; Zare, Mohammad; Barekatain, Majid; Basiratnia, Reza; Rahimian, Elham; Mehvari Habibabadi, Amirali; Moein, Payam
Background. Hypothalamic hamartomas (HHs) are rare tumor-like malformations that may present with complex partial seizures refractory to anticonvulsants in adulthood. The condition may be misdiagnosed because of rarity. Case Presentation. We report a 25-year-old man with complaint of seizures presented by falling, tonic spasm of limbs, oral automatism, vocalization, and hypermotor activities. His seizures started at the age of one month and presented as eye deviation and upper limbs myoclonic jerk, followed by frequent seizures with variable frequency. The patient had delayed developmental milestones and was mentally retarded. He was hospitalized and underwent video-EEG monitoring and neuroimaging, and the diagnosis of HH was made. The patient became candidate for surgery after that. Conclusion. In this case, the underlying etiology of seizures was diagnosed after 25 years. HH is a rare condition and neurologists may encounter very small number of these cases during their practice. Therefore, they should consider it in patients who present with suspected signs and symptoms. PMID:28246530
Full Text Available Background. Hypothalamic hamartomas (HHs are rare tumor-like malformations that may present with complex partial seizures refractory to anticonvulsants in adulthood. The condition may be misdiagnosed because of rarity. Case Presentation. We report a 25-year-old man with complaint of seizures presented by falling, tonic spasm of limbs, oral automatism, vocalization, and hypermotor activities. His seizures started at the age of one month and presented as eye deviation and upper limbs myoclonic jerk, followed by frequent seizures with variable frequency. The patient had delayed developmental milestones and was mentally retarded. He was hospitalized and underwent video-EEG monitoring and neuroimaging, and the diagnosis of HH was made. The patient became candidate for surgery after that. Conclusion. In this case, the underlying etiology of seizures was diagnosed after 25 years. HH is a rare condition and neurologists may encounter very small number of these cases during their practice. Therefore, they should consider it in patients who present with suspected signs and symptoms.
Full Text Available Abstract Background Glial heterotopias are rare, benign, congenital, midline, non-teratomatous extracranial glial tissue. They may masquerade as encephalocoele or dermoid cyst and mostly present in nose. Herein, we present an unusual case of glial heterotopia of the orbit with unilateral blindness. Case presentation A 6 year-old-boy presented with a progressive painless mass over the nose and medial aspect of the left eye noticed since birth. On examination, the globe was displaced laterally by a firm, regular, mobile, non-pulsatile and non-tender medial mass. The affected eye had profound loss of vision. Computed tomography scan showed a large hypodense mass in the extraconal space with no intracranial connectivity and bony erosion. The child underwent total surgical excision of the mass and histopathological examination confirmed glial heterotopia of the orbit. Conclusion Though the incidence of this condition is rare, the need of appropriate diagnosis and management of such mass to prevent the visual and cosmetic deterioration is warranted. To our knowledge this is the first reported case of Glial heterotopia of orbit causing unilateral blindness.
Govind K Babu
Full Text Available Multiple myeloma (MM is a plasma cell neoplasm and constitutes 10% of hematologic malignancies. Malignant myelomatous pleural effusions are very rare and occur in <1% of cases of MM. In this article, we report a rare case of a patient who initially presented with pleural effusion and was subsequently found to be secondary to MM with an underlying raised IgG paraprotein. The patient symptomatically improved and was in partial remission with palliative radiotherapy, VTD chemotherapy, and bisphosphonates.
Full Text Available Background. Tuberculosis is a chronic disease which may have varied presentations. Though pulmonary tuberculosis is the commonest, extrapulmonary tuberculosis involving skeletal system is often seen. Individuals with poor nourishment and immunological status are especially susceptible for disseminated and multicentric tuberculosis. Case Report. We here present a case of tuberculosis involving multiple anatomical locations in an immune-competent patient which was diagnosed with radiological studies and confirmed with histological examination. Patient was put on multidrug antitubercular therapy and responded well to the treatment with improvement in clinical and radiological picture. Clinical Relevance. This report of a rare case makes us aware of the varied presentations which tuberculosis can present with. It should be kept as a differential diagnosis in patients with cough and fever but not responding to conventional treatment. This is even more important in countries with poor socioeconomic conditions.
Mohammad Reza Mohaghegh
Full Text Available Ependymomas are glial tumors derived from ependymal cells lining the ventricles and the central canal of the spinal cord. Two thirds of ependymomas arise in the infratentorial or intraventricles, whereas one-third are located in supratentorial space. But supratentorial "cortical" ependymomas are very rare. We report a case of a cortical ependymoma in a 17-year-old boy. The patient presented with transient recurrent right weakness and diplopia. This tumor was located in the left parieto-occipital region and he had gross total excision. Microscopy and immunohistochemistry showed grade III differentiation ependymoma.
Mohaghegh, Mohammad Reza; Chitsaz, Ahmad; Okhovat, Ali Asghar; Pour, Elnaz Babaei
Ependymomas are glial tumors derived from ependymal cells lining the ventricles and the central canal of the spinal cord. Two thirds of ependymomas arise in the infratentorial or intraventricles, whereas one-third are located in supratentorial space. But supratentorial "cortical" ependymomas are very rare. We report a case of a cortical ependymoma in a 17-year-old boy. The patient presented with transient recurrent right weakness and diplopia. This tumor was located in the left parieto-occipital region and he had gross total excision. Microscopy and immunohistochemistry showed grade III differentiation ependymoma.
Full Text Available How to Cite This Article: Eslamiyeh H, Ashrafzadeh F, Akhondian J, Beiraghi Toosi M. Homocystinuria: A Rare Disorder Presenting as Cerebral Sinovenous Thrombosis. Iran J Child Neurol. Spring 2015;9(2:53-57.AbstractObjectiveHomocystinuria is an inborn error of amino acid metabolism caused by cystathionine beta-synthase deficiency that affects methionine metabolism. The clinical features are heterogeneous ranging from mental retardation, ectopia lentis, and osteoporosis to vascular events such as deep vein thrombosis, sagital sinus thrombosis, and myocardial infarction. Cerebral sinovenous thrombosis (CVST is an unusual disorder in children and requires prompt and accurate management. Some causal factors for thedevelopment of CVST differ between children and adults. The majority of cases with CSVT are found to have an underlying cause for thrombosis like dehydration, infections, prothrombotic and hematologic disorders, malignancy and trauma.Although homocystinuria is usually associated with ischemic strokes, CVST as initial clinical presentation of homocystinuria is rare in children.In this article, we presented a 10-year old boy with seizure, hemiparesis, and ataxia due to CSVT caused by homocystinuria.
Shin, Jeong Hun; Kim, Seok Hwan; Park, Jinkyu; Lim, Young-Hyo; Park, Hwan-Cheol; Choi, Sung Il; Shin, Jinho; Kim, Kyung-Soo; Kim, Soon-Gil; Hong, Mun K; Lee, Jae Ung
Cardiogenic unilateral pulmonary edema (UPE) is a rare clinical entity that is often misdiagnosed at first. Most cases of cardiogenic UPE occur in the right upper lobe and are caused by severe mitral regurgitation (MR). We present an unusual case of right-sided UPE in a patient with cardiogenic shock due to acute myocardial infarction (AMI) without severe MR. The patient was successfully treated by percutaneous coronary intervention and medical therapy for heart failure. Follow-up chest Radiography showed complete resolution of the UPE. This case reminds us that AMI can present as UPE even in patients without severe MR or any preexisting pulmonary disease affecting the vasculature or parenchyma of the lung.
Khatib, Yasmeen; Dande, Madhura; Patel, Richa D; Gite, Vinod
Vascular malformations (VMs) are structural malformations of vascular development causing soft tissue abnormality with functional and esthetic impairment. They are named by their predominant vessel type as arterial, venous, lymphatic or mixed types. VMs of the parotid gland are extremely rare and constitute a distinct entity of parotid pathology that requires specific diagnostic tools and management. Till 2013, only fifty cases of VMs of the parotid have been described in the literature. We present a case of a venolymphatic malformation of the parotid gland extending into the parapharyngeal space in a 21-year-old male who presented with a swelling on the left side of the face extending into the neck. Diagnosis was suggested by ultrasonography and computed tomography scan and was confirmed by magnetic resonance imaging examination. Complete surgical excision of the lesion was done with a favorable outcome. Diagnosis was confirmed based on histopathology and immunohistochemical studies. PMID:27601828
Full Text Available Vascular malformations (VMs are structural malformations of vascular development causing soft tissue abnormality with functional and esthetic impairment. They are named by their predominant vessel type as arterial, venous, lymphatic or mixed types. VMs of the parotid gland are extremely rare and constitute a distinct entity of parotid pathology that requires specific diagnostic tools and management. Till 2013, only fifty cases of VMs of the parotid have been described in the literature. We present a case of a venolymphatic malformation of the parotid gland extending into the parapharyngeal space in a 21-year-old male who presented with a swelling on the left side of the face extending into the neck. Diagnosis was suggested by ultrasonography and computed tomography scan and was confirmed by magnetic resonance imaging examination. Complete surgical excision of the lesion was done with a favorable outcome. Diagnosis was confirmed based on histopathology and immunohistochemical studies.
Shaik, Sameeulla; Raviraj, Jayam; Dirasantchu, Suresh; Venkata, Suman S
Ellis-van Creveld (EVC) syndrome, a form of skeletal and chondroectodermal dysplasia, is an autosomal recessive disorder characterized by a tetrad of disproportionate dwarfism, postaxial polydactyly, ectodermal dysplasia, and heart defects. In the present article, we hereby present a case of a 13-year-old girl of Indian ethnicity with EVC syndrome with a remarkable number of classical oral and dental features, with unusual findings such as taurodontism and talons cusp. Such dental findings were reported in few cases only. Despite the fact that oral manifestations play an important role in the diagnosis of EVC, only a few detailed reports have been published in the dental literature.
Shaik, Sameeulla; Raviraj, Jayam; Dirasantchu, Suresh; Venkata, Suman S.
Ellis–van Creveld (EVC) syndrome, a form of skeletal and chondroectodermal dysplasia, is an autosomal recessive disorder characterized by a tetrad of disproportionate dwarfism, postaxial polydactyly, ectodermal dysplasia, and heart defects. In the present article, we hereby present a case of a 13-year-old girl of Indian ethnicity with EVC syndrome with a remarkable number of classical oral and dental features, with unusual findings such as taurodontism and talons cusp. Such dental findings were reported in few cases only. Despite the fact that oral manifestations play an important role in the diagnosis of EVC, only a few detailed reports have been published in the dental literature. PMID:27076836
Full Text Available Ellis-van Creveld (EVC syndrome, a form of skeletal and chondroectodermal dysplasia, is an autosomal recessive disorder characterized by a tetrad of disproportionate dwarfism, postaxial polydactyly, ectodermal dysplasia, and heart defects. In the present article, we hereby present a case of a 13-year-old girl of Indian ethnicity with EVC syndrome with a remarkable number of classical oral and dental features, with unusual findings such as taurodontism and talons cusp. Such dental findings were reported in few cases only. Despite the fact that oral manifestations play an important role in the diagnosis of EVC, only a few detailed reports have been published in the dental literature.
Full Text Available Fistula in ano is an inflammatory condition affecting perianal re gion and adjacent structures. It is a cause of significant morbidity, requiring repeated surgical treatments due to its high recurrence rate. Most perianal fistulous disease have external openings located within 2.5 cm of the anal verge. It is rare for an anal fistula to involve the scrotum. Such involvement can occur frequently in patients with inflammatory bowel disease, especially Crohn’s disease and rarely in ulcerative colitis. Isolated involvement of scrotal region is quite rare without concomitant inflammatory bowel disease. This has been rarely reported in the literature. We present 2 isolated cases of trans sphincteric fistula in ano with external opening in the vicinity of scrotum. Both our cases did not reveal any signs of inflammatory bowel disease.
Full Text Available We report a case of multiple bilateral fibroadenomata on the arm away from the milk line in a 33 year old female who in addition presented with fibrocystic disease of the breast and secondary infertility. Possible origin of these cutaenous fibroadenomas have been hypothesized. This is the second report of its kind being reported in the English literature.
Laryngeal Tuberculosis generally occurs in association with Pulmonary Tuberculosis and that too in an immuno-compromised host.Here,We are presenting a case report of an immunocompetent host,initially thought of suf-fering from Pulmonary Tuberculosis but was finally diagnosed a case of Primary Laryngeal Tuberculosis on serial investigations with review of literature.
Sumeet Bhargava; Rajul Rastogi; Gaurav Jindal; Amrit Kumar Singh; Vaibhav Rastogi
Tuberculous infection manifesting as an isolated intramedullary tuberculoma of the spinal cord is distinctly unusual. We report a case of a 35 year old woman presenting with an insidious onset of myelopathy, where MRI showed characteristic imaging findings suggestive of intramedullary tuberculosis.
VR Guttikonda; Taneeru, S; Gaddipati, R; Madala, J
Juvenile ossifying fibroma (JOF) is an uncommon, benign, bone-forming neoplasm with an aggressive local growth that is distinguished from other fibro-osseous lesions primarily by its age of onset, clinical presentation and aggressive behaviour. JOF is considered as a variant of the ossifying fibroma (OF) and the former includes psammomatoid JOF (PsJOF) and Trabecular JOF (TrJOF). Both variants involve the craniofacial bones with the trabecular variant being more common in the jaws and the psa...
Haroon, Saroona; Tariq, Muhammad Usman; Memon, Aisha; Fatima, Saira; Hasan, Sheema Habibul
Objectives: To present the clinicopathological experience of Olfactory Neuroblastoma (ONB) with emphasis on histopathological and immunohistochemical features. Methods: A descriptive cross-sectional study was done on 36 cases of ONB, selected by non-probability purposive sampling. Theses cases of ONB were retrieved and reviewed from surgical pathology database of Aga Khan University Hospital reported between January 1993 and March 2015. Results: Tumor size and age of presentation was wide in range without any distinct bimodal distribution. Nasal cavity was most common site along with involvement of paranasal sinuses. More than 50% cases had Kadish stage A. Microscopically, most cases were Grade-1 and majority showed partial or complete lobular architecture. Neurofibrillary matrix was observed in 2/3rd of cases. Among immunohistochemical markers, Neuron Specific Enolase was most frequently expressed. Unusual positive expression of Cytokeratin AE1/AE3 and Cytokeratin CAM5.2 was also seen focally in few cases. Conclusion: The ONB has great variability of histological and clinical presentation, and immunohistochemical markers are useful to differentiate from more common small round blue cell tumours of nasal cavity. PMID:27375694
Full Text Available HHV8/EBV-associated germinotropic lymphoproliferative disorder (GLD is a challenging diagnosis given its rarity, the particular clinical presentation, and the lack of expression of markers usually used in establishing hematopoietic lineage. We report a new case of HHV8/EBV GLD in an immunocompetent 78-year-old woman. The diagnosis was made in an incidentally discovered lymphadenopathy. Histological examination showed a nodular lymphoid proliferation centered by aggregates of atypical plasmablastic cells admixed with small lymphoid cells. Tumor cells were strongly positive with EMA, HHV8, LMP1, CD38, CD138, and kappa light chains. They were negative with common lymphoma-associated markers (CD20, CD3, CD15, CD30, CD10, and bcl2. In situ hybridization confirmed the monotypic kappa light chains and the EBV infection (EBER+. A polyclonal pattern of Ig gene rearrangement was detected by PCR analysis. In the adjacent lymph node parenchyma, some germinal centers mimicked Castleman disease. In this case, the differential diagnosis was discussed with an early stage of large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease. The clinical presentation, the immunophenotype, and the molecular results helped to make the accurate diagnosis. Through the review of the nine previously reported cases in literature, we discuss the clinical and pathologic features and the differential diagnosis of HHV8/EBV GLD.
Tianshen Hu, BS
Full Text Available Several theories have been put forth to explain the complex yet symmetrical malformations and the myriad of clinical presentations of caudal duplication syndrome. Hereby, reported case is a 28-year-old female, gravida 2 para 2, with congenital caudal malformation who has undergone partial reconstructive surgeries in infancy to connect her 2 colons. She presented with recurrent left lower abdominal pain associated with nausea, vomiting, and subsequent feculent anal discharge. Imaging reveals duplication of the urinary bladder, urethra, and colon with with cloacal malformations and fistulae from the left-sided cloaca, uterus didelphys with separate cervices and vaginal canals, right-sided aortic arch and descending thoracic aorta, and dysraphic midline sacrococcygeal defect. Hydronephrosis of the left kidney with left hydroureter and inflammation of one of the colons were suspected to be the cause of the patient’s acute complaints. She improved symptomatically over the course of her hospitalization stay with conservative treatments. The management for this syndrome is individualized and may include surgical intervention to fuse or excise the duplicated organs.
Nandyala Hariharanadha Sarma
Full Text Available Testicular lymphoma is the second most common extra nodal lymphomas. It is a highly lethal disease with a median survival of 1-2 years. In human immunodeficiency virus (HIV patients, primary testicular lymphomas are estimated to comprise > 6% of testicular tumors, and they tend to occur in younger patients. Testicular lymphoma can occasionally be the initial manifestation of the disease in HIV patients.Plasmablastic lymphoma (PBL, which is considered as a variant of diffuse large B-cell lymphoma is a highly aggressive tumor with poor prognosis. PBL has a well-established association with HIV infection and occurs most commonly in the oral cavity. The presentation at extra nodal sites and absence of usual hematolymphoid markers makes its diagnosis more difficult. PBL of the testis as the primary lesion in HIV patients has not been reported so far. We report a case of PBL presenting as a primary testicular lesion in a HIV patient with a grave prognosis.
Sah, Birendra P; Sharma, Bikram; Iannuzzi, Michael C
We report a case of orbital sarcoidosis in a 66 year old male who presented with one month history of right eye swelling and intermittent diplopia. MRI revealed an enhancing infiltrative soft tissue mass in the inferior aspect of the right orbit and biopsy of the mass demonstrated non-necrotizing granulomas. Chest CT scan was normal and PET scan showed no other organ involvement. He was treated with tapering doses of prednisone over six months. Although relapse occurred while tapering prednisone to 20 mg per day, he responded well to the addition of azathioprine with complete resolution of visual difficulties and orbital the mass on repeat MRI. Sarcoidosis, presenting as an isolated orbital mass is rare, can be successfully treated and should be included in differential diagnosis.
Full Text Available Cryptococcal meningoencephalitis is a leading of morbidity and mortality in immunocompromised individuals worldwide. However, there are few documented cases in immunocompetent patients. We present a rare case of disseminated Cryptococcus with progression to meningoencephalitis in an immunocompetent patient, with a possible atypical presentation. Magnetic resonance imaging of the brain and electroencephalogram to rule out brain metastasis were negative. Lumbar puncture resulted positive for Cryptococcus neoformans antigen at titers of 1:2048 and a detailed history later revealed occupational exposure to bird dander by cleaning floors and cages. Diagnosis is challenging, with delays often resulting in increased morbidity and mortality. Cerebrospinal fluid and serum Cryptococcus antigen play a key role in both diagnosis and determining treatment efficacy. Furthermore, current treatment guidelines are used for immunocompromised individuals. Due to the significant side effects of these medications, further research is needed to determine the optimal treatment duration for immunocompetent patients to minimize the need for unnecessary therapy.
Chan, Jin Ei; Palakodeti, Sandeep; Koster, Matthew J
Antisynthetase syndrome (ASS) is recognized as a subgroup of idiopathic inflammatory myopathies (IIMs). It is associated with autoantibodies directed against aminoacyl-transfer ribonucleic acid (tRNA) synthetase enzymes. We report the first case of anti-PL-7/anti-SSA 52kD ASS presenting as acute digital ischemia, an association not described previously. Occlusive vasculopathy is a rare but serious manifestation that can be seen at presentation in patients with ASS and may herald the onset of severe interstitial lung disease (ILD). Comprehensive evaluation should be performed to confirm the presence of subclinical myositis. Extensive myositis-specific antibody testing is strongly recommended even if initial screening autoimmune serologies are unrevealing.
Full Text Available A 27-year-old African American male presented with a sudden onset of blisters. He had a past medical history of uncontrolled diabetes mellitus type I, diabetic vasculopathy, and neuropathy. The physical examination revealed nonerythematous skin denudations on both elbows and lateral aspect of arm bilaterally. Investigations which included skin biopsies confirmed the diagnosis of bullosis diabeticorum. The bullae were treated with hydrotherapy and healed with no complications in 4 weeks. We present this case to illustrate the rare occurrence of diabetic bulla in a diabetic patient especially with poor glycemic control. The case is also a reminder of the importance of diabetes screening in nondiabetic patients who are diagnosed with diabetic bulla.
Suhail Majid Jan
Full Text Available Tuberculosis (TB is a chronic specific granulomatous disease and a major cause of death in developing countries. The clinical presentation of TB lesions of the oral cavity varies widely and can manifest as ulcerations, diffuse inflammatory lesions, granulomas and fissures. Oral lesions generally appear secondary to primary TB infection elsewhere, although primary infection of the oral mucosa by Mycobacterium tuberculosis has also been described. We hereby report a case of primary TB of the gingiva manifesting as gingival enlargement. Diagnosis was based on histopathological examination, complete blood count, X-ray chest and immunological investigations with detection of antibodies against M. tuberculosis. Anti-tuberculous therapy was carried out for over 6 months and was followed by surgical excision of the residual enlargement under local anesthesia. After 1-year follow-up, there was no recurrence of the disease. This case report emphasizes the need for dentists to include TB in the differential diagnosis of various types of gingival enlargements.
Full Text Available Intraductal papillary neoplasm of the bile duct (IPNB is a specific type of bile duct tumor. It has been proposed that it could be the biliary counterpart of the intraductal papillary neoplasm of the pancreas (IPMN-P. This hypothesis is supported by the presence of simultaneous intraductal tumors of both the bile duct and pancreas. There have been five reports of patients with simultaneous IPNB and IPMN-P. In all of these cases, biliary involvement was limited to the intrahepatic and perihilar bile duct, which had characteristics similar to IPMN-P and usually had slow progression in nature. Herein, we present the first case of extensive intraductal neoplasm involving the extrahepatic bile duct, intrahepatic bile duct, and entire length of the pancreas with a poor outcome, even after being treated aggressively with radical surgery and adjuvant chemotherapy. Additionally, we summarize previous case reports of simultaneous intraductal lesions of the bile duct and pancreas.
Marguet, Florent; Proust, François; Crahes, Marie; Basset, Carole; Joly-Helas, Géraldine; Chambon, Pascal; Laquerrière, Annie
We report on a 51-year-old woman who presented with a cervical spinal cord tumor clinically suspected to be a metastasis. Histological examination revealed an anaplastic meningioma containing epithelial nests arranged in a gland-like pattern suggestive of adenocarcinoma. This component strongly expressed cytokeratins whereas the meningothelial component was vimentin--epithelial membrane antigen--and progesterone receptor-immunoreactive, suggesting either anaplastic meningioma with adenocarcinoma-like metaplasia, or adenocarcinoma metastasis in a meningioma, but the search for a primitive neoplasia including thoracic-abdominal-pelvic computed tomography and mammography was negative. Anaplastic meningiomas with adenocarcinoma-like metaplasia are uncommon lesions, 4 cases having been reported in the literature so far. Their immunohistochemical and chromosomal characteristics are similar to those observed in secretory meningiomas. When available, fluorescence in situ hybridization detects the same chromosomal alterations in the two components, confirming a common clonal origin. This observation demonstrates the necessity to perform the correct diagnosis of malignant meningioma with adenocarcinomatous metaplasia, whose prognosis and treatment radically differ from those of metastatic adenocarcinoma located in a meningioma. Copyright © 2014 Elsevier Masson SAS. All rights reserved.
Di Muzio, A; Delli Pizzi, C; Lugaresi, A; Ragno, M; Uncini, A
Six patients presented with amyotrophy confined to a single lower limb and characterized by insidious onset, slow progression and later stabilization. Wasting was out of proportion with disability and there were no sensory, pyramidal tract or bulbar signs. All cases were sporadic, and there was no history of poliomyelitis. CK, anti-ganglioside antibodies, motor and sensory conductions were normal. Quantitative EMG and muscle biopsy revealed neurogenic features also in clinically unaffected limbs. Muscular CT showed selective or predominant, asymmetrical involvement of posterior leg muscles and caput longus of biceps femoris. Monomelic amyotrophy of lower limb is a clinically localized variant of spinal muscular atrophy with a particularly benign course. Although in the early stage there are no clinical or laboratory findings which allow differential diagnosis with other motor neuron diseases, the history of an amyotrophy clinically localized for more than 3 years to a lower single limb and the characteristic muscular CT pattern suggest the diagnosis since the first observation and indicate a favorable prognosis.
Federico Perfetto; Roberto Tarquini; Franceco Mancuso; Simonetta di Lollo; Silvia Tozzini; Giampiero Belesi; Giacomo Laffi
We reported a case of non-Hodgkin's lymphoma where liver involvement was the predominant clinical manifestation. A 27-year old man presented wiht markedly elevated serum aspartate aminotrasferase, alanine aminotransferase and lactate dehydrogenase, reduced prothrombin activity, thrombocytopenic purpura and hepato-splenomegaly without adenopathy. Viral, toxic,autoimmune and metabolic liver diseases were excluded.Bone marrow biopsy showed an intracapillary infiltration of T-lymphocytes with no evidence of lipid storage disease.Because of a progressive spleen enlargement, splenectomy was performed. Histological examination showed lymphomatous intrasinuses invasion of the spleen.Immunohistochemical investigation revealed the T phenotype of the neoplastic cells: CD45+, CD45RO+,CD3+, CD4-, CD8-, TIA1-. About 50 % of the lymphoid cells expressed CD56 antigen. The diagnosis of hepatosplenic T cell lymphoma was done. The patient was treated with chemotherapy, which induced a complete remission. Eighteen months later, he had a first relapse with increased aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase,thrombocytopenic purpura and blast in the peripheral blood.In spite of autologous bone marrow transplantation, he died twenty months after the diagnosis. Even in the absence of a mass lesion or lymphoadenopathy, hepatosplenic Tcell lymphoma should be considered in the differential diagnosis of a patient whose clinical course is atypical for acute hepatic dysfunction.
Kalyan B Bhattacharyya
Full Text Available Orthostatic tremor (OT, is usually a disease of old age and is characterized by quivering movements of the legs during quiet standing or in the state of isometric contraction in the lower limbs. This is relieved on walking or on lying down. It is diagnosed by surface electromyography, particularly over the quadriceps femoris muscles which shows a distinctive frequency of 13 to 18 Hz on standing. Some investigators consider it as a variant of essential tremor (ET and the two conditions often co-exist. The disease is usually non-familial. Two brothers presented with tremor in the lower limbs on standing and on the outstretched hands without any family history. Subsequently, they were proved to be suffering from OT and ET by clinical examination and surface EMG. Simultaneous occurrence of OT and ET in two young brothers without any family history in the previous generation has not been described before and they also appeared at a much earlier age than what is described in the literature.
Full Text Available Malignancy in pregnancy is rare. Carcinomas in pregnancy are mostly kidney cell mass. Renal cell carcinoma (RCC is the commonest malignancy in pregnancy. Because of softness and increased vascularity, rupture of renal cell carcinoma is not uncommon. Here we are presenting a rare case of renal cell carcinoma in pregnancy with spontaneous rupture resulting in massive hemoperitoneum and serious outcome because of late presentation renal cell carcinoma seldom ruptures. A 26 year old woman G2P1L1 with term pregnancy was referred to hospital 80kms away from periphery with non-progression of labour. There was antenatal record suggesting hypertensive disorder of pregnancy in second trimester. On examination, patient was in hypovolemic shock with profuse distension of abdomen. Diagnosis of abruption grade 3 or rupture uterus was made and immediate laparotomy was done. On opening the abdomen, there was hemoperitoneum but uterus was intact. Emergency LSCS done extracted a stillborn baby. There were no retro placental clots also. There was lot of necrotic tissue in the abdomen and there was a tumour arising from lower pole of left kidney which had invaded the renal vessels and had ruptured. Peripartum hysterectomy and left nephrectomy was done. Women did not respond to treatment and died. The objective of presenting this case is the dilemmas faced by the obstetrician in case of shock in 2nd stage of labour. Simple diagnostic tool like renal ultrasound will help to detect at an early stage which could improve the outcome. All cases of hypertensive disorders of pregnancy should be investigated for secondary causes of hypertension. Abdominal USG must be done for all cases of hypertensive disorders of pregnancy in 2nd trimester. Prompt diagnosis and early treatment is the key in management of such condition in pregnancy. [Int J Reprod Contracept Obstet Gynecol 2016; 5(5.000: 1677-1679
Full Text Available Histoplasmosis, a fungal disease caused by Histoplasma capsulatum, is endemic in North and South America. Except few scattered cases, the disease is considered to be a non-entity in India. Furthermore, disseminated histoplasmosis is rare in the immunocompetent individuals. We report an adolescent boy presenting as middle lobe consolidation which did not respond to antibiotics. His condition deteriorated with the development of mediastinal lymphadenopathy, pleural effusion and hepatosplenomegaly. A diagnosis of progressive disseminated histoplasmosis was established by his clinical findings as well as bronchoscopic biopsy, transbronchial needle aspiration cytology and bronchoalveolar lavage culture demonstrating Histoplasma capsulatum. The case represents a unique example of progressive disseminated histoplasmosis in an immunocompetent individual in India.
Full Text Available Simple bone cyst represent approximately 3% of all primary bone tumors sampled for biopsy and nearly always occur during the first two decades of life. They are common in metaphyseal region of long bones. Clavicle is rare s ite for this lesion and not many have been reported in literature. We report a case of Histopathologically confirmed Simple bone cyst in a 65 yr s old manual laborer who presented with pain and swelling of long duration. The occurrence of this lesion at unu sual age and at unusual location carries a lot of diagnostic dilemma and various differentials like ABC , Eosinophilic granuloma , and enchondroma were considered only to confirm simple bone cyst on HPE . The symptoms were relieved after resection of lesion. Clavicle was reconstructed with tricortical iliac crest bone graft. At 18th month follow up the patient had no recurrences and had a good functional outcome .
Full Text Available Krukenberg tumors mostly occur after 40 years. Metastatic ovarian tumors in young age are very rare and reported to be 2% of all the cases. Thirty percent of all ovarian neoplasms occurring during childhood and adolescence are malignant. A 25-year-old woman, parity- 2, presented with abdominal distension, pain in abdomen and amenorrhea. On examination, 18 weeks lump was palpable, firm to hard in consistency, non-tender and mobile. On ultrasonography bilateral ovarian tumors were reported, without any peritoneal free fluid. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed. Microscopic examination revealed signet ring cells with glandular differentiation, diffusely invading the ovarian parenchyma. Tumor cells exhibited strong, diffuse immunopositivity for CEA with focal strong immunopositivity for CK7 and CK20 and immunonegativity for SATB2. Diagnosis of Krukenberg tumor was made. Endoscopic biopsy confirmed the diagnosis of adenocarcinoma stomach. This case is reported because of its rarity in younger age group.
Full Text Available The Klippel-Trenaunay syndrome (KTS is a congenital disorder characterized by capillary malformation, varicosities and bony and soft tissue hypertrophy. This disease has several morbidities like bleeding, deep venous thrombosis, embolic complications and in some cases enlargement of limb that may require amputation. Vascular malformations are segmented and never cross midline. However, we came across a case, a 45-year-old male, who presented with varicosity of veins and deformity of left lower limb besides cavernous hemangiomas (port-wine stains scattered all over his face, chest, back, gluteal region, groin and legs since birth. Multiple paravertebral soft tissue masses and bladder hypertrophy were also noted due to involving neurofibromatosis. Simultaneous occurrence of KTS and neurofibromatosis is rarely seen in clinical practice.
Full Text Available Primitive neuroectodermal tumors (PNETs are a group of highly malignant tumors composed of small round cells of neuroectodermal origin that affect soft tissue and bone. PNET of the larynx is extremely rare. We report a case of a 41-year-old male who presented with the complaints of progressively increasing stridor of 3 months duration, which was diagnosed as a case of neuroectodermal tumor in the subglottis. Patient was subjected to microlaryngeal surgery and the tumor was excised. Postoperatively, patient was given three cycles of chemotherapy comprising of ifosfamide, etoposide, and mesna, along with granulocyte colony stimulating factor, with 21 days interval. After chemotherapy repeat computed tomography scan showed no evidence of the tumor and no lymphadenopathy. Patient is symptom free for 18 months following completion of treatment. He is under regular follow-up and is undergoing monthly serial endoscopic evaluation.
Tkachenko, Lara; Moisi, Marc; Rostad, Steven; Umeh, Randle; Zwillman, Michael E; Tubbs, R. Shane; Page, Jeni; Newell, David W.; Delashaw, Johnny B
A 69-year-old female with a history of breast cancer and hypertension presented with a rare case of herpes simplex encephalitis (HSE) isolated to her left parietal lobe. The patient’s first biopsy was negative for herpes simplex virus (HSV) I/II antigens, but less than two weeks later, the patient tested positive on repeat biopsy. This initial failure to detect the virus and the similarities between HSE and symptoms of intracranial hemorrhage (ICH) suggests repeat testing for HSV in the presence of ICH. Due to the frequency of patients with extra temporal HSE, a diagnosis of HSE should be more readily considered, particularly when a patient may not be improving and a concrete diagnosis has not been solidified. PMID:27774355
Mohd Jafar Memon
Full Text Available Pancreatic neoplasms are rare in children and have a different histo-logic spectrum and prognosis than those in adults. Pancreatoblastoma is the most common pancreatic neoplasm in young children. Solid pseudopapillary neoplasm occurs in adolescent girls. It is heterogeneous in internal architecture, with a mixture of solid and cystic hemorrhagic and necrotic elements. All pancreatic neoplasms in children are capable of producing metastases, usually to the liver and lymph nodes; however, on the whole, these tumors have a better clinical outcome than most pancreatic tumors in adults. We present a case of solid pseudopapillary neoplasm with a liver metastasis in a 13 year old male patient. [Int J Res Med Sci 2016; 4(7.000: 3090-3093
Debadatta, Mohapatra; Mishra, Ajay K
Meige's syndrome is a rare neurological syndrome characterized by oromandibular dystonia and blepharospasm. Its pathophysiology is not clearly determined. A 35-year-old female presented to psychiatric department with blepharospasm and oromandibular dystonia with clinical provisional diagnosis of psychiatric disorder (Conversion Disorder). After thorough physical examination including detailed neurological exam and psychiatric evaluation no formal medical or psychiatric diagnosis could be made. The other differential diagnoses of extra pyramidal symptom, tardive dyskinesia, conversion disorder, anxiety disorder were ruled out by formal diagnostic criteria. Consequently with suspicion of Meige's syndrome she was referred to the department of Neurology and the diagnosis was confirmed. Hence, Meige's syndrome could be misdiagnosed as a psychiatric disorder such as conversion disorder or anxiety disorder because clinical features of Meige's syndrome are highly variable and affected by psychological factors and also can be inhibited voluntarily to some extent.
Fisahn, Christian; Tkachenko, Lara; Moisi, Marc; Rostad, Steven; Umeh, Randle; Zwillman, Michael E; Tubbs, R Shane; Page, Jeni; Newell, David W; Delashaw, Johnny B
A 69-year-old female with a history of breast cancer and hypertension presented with a rare case of herpes simplex encephalitis (HSE) isolated to her left parietal lobe. The patient's first biopsy was negative for herpes simplex virus (HSV) I/II antigens, but less than two weeks later, the patient tested positive on repeat biopsy. This initial failure to detect the virus and the similarities between HSE and symptoms of intracranial hemorrhage (ICH) suggests repeat testing for HSV in the presence of ICH. Due to the frequency of patients with extra temporal HSE, a diagnosis of HSE should be more readily considered, particularly when a patient may not be improving and a concrete diagnosis has not been solidified.
Full Text Available Paraganglioma is a rare extra-adrenal pheochromocytoma which originates from chromaffin cells within the ganglia of the sympathetic trunk and of the celiac, renal, suprarenal, and hypogastric plexuses. Pancreatic paragangliomas are rarer still. And even then, paragangliomas are mostly reported to be nonfunctional. We report a case of a 64-year-old woman with underlying disease of hypertension who presented with biliary colic. Contrast-enhanced computer tomography showed an enhancing mass in the uncinate process of the pancreas. Pylorus-sparing Whipple procedure was performed for complete tumor excision. Hypertensive crisis developed after Whipple, which improved after continuous intravenous nicardipine infusion. Pathology revealed a paraganglioma. A 24-h catecholamine urine test showed increased norepinephrine and vanillylmandelic acid level. Functional paraganglioma was diagnosed.
Rade R. Babić
Full Text Available The results of the radiological study of the urinary system anomalies are presented on the material consisting of 8,568 urographies done from 1990 to 2001 at the Institute for Radiology, Niš. The paper shows exceptionally rare anomalies of the urinary system: a horse-shoe shaped kidney with pyelocaliceal systems in its arms and isthmus, heterolateral ectopia of the kidney with fusion, abdominal-medial ectopia of the kidney with ventral malrotation and cup hyperplasia, hypoplastic cup, triple pyeolcaliceal system, M. Lenarduzzi and blind-ending of the Y-shaped urethra. The author concludes that, for the sake of performing every day professional work, it is necessary to possess detailed knowledge of the rarest urinary system anomalies.
Vinay, Keshavamurthy; Chandrasegaran, Ariganesh; Kanwar, Amrinder J; Saikia, Uma N; Kaur, Harsimran; Shivaprakash, M R; Dogra, Sunil
Mucormycosis is an uncommon systemic mycosis affecting the immunocompromised individuals. It is usually caused by organisms of the genera Rhizopus and Mucor, although rarely other organisms have also been implicated. Mycoses due to these angioinvasive fungi have an acute onset, rapidly progressive course with high mortality rate. A rare and less well known is the chronic subtype of primary cutaneous mucormycosis (PCM). Herein, we report a case of PCM clinically presenting as a chronic, giant destructive plaque in a young immunocompetent male and coin the term chronic granulomatous mucormycosis. A clinicopathological classification for cutaneous mucormycosis is also proposed.
Bhasin, Tejinder Singh; Sharma, Sonam; Chandey, Manish; Bhatia, Puneet Kaur; Mannan, Rahul
Bone Marrow Necrosis (BMN) is regarded as a rare entity in the aspirates and the trephine biopsies which are obtained from living patients and it is a rare antemortem diagnosis. Usually, BMN is associated with a poor prognosis and it has a 90% association with malignancies (mainly haematological). Its other causes include infections, drugs and other non malignant haematological causes. BMN which occurs due to unknown causes (idiopathic), is very rare. The present case report describes a case of BMN in a 14 year old male child from rural background, who came to the medicine outpatient department of a tertiary care centre in north India, with the complaints of generalized weakness, severe bone pains, especially in the ribcage area and fatigue. His peripheral blood film revealed pancytopaenia. His bone marrow aspiration yielded a watery, sero-sanguinous fluid from 2 different sites. Microscopy revealed "ghost like" haematopoietic cells lying in eosinophilic, proteinaceous material. Bone marrow biopsies from both the sites revealed extensive foci of gelatinous transformation and necrosis. A diagnosis of BMN was rendered. No underlying neoplastic / non neoplastic cause was identified, even after a thorough systematic investigation (haematological/biochemical/ radiological and immunological). Thus, a final diagnosis of BMN due to an idiopathic cause, was finally given. The patient was given supportive treatment, after which he was referred to a higher centre. BMN which arises idiopathically is usually very rare and often precedes the aetiological diagnosis and obscures the diagnosis. Traditionally, the prognoses of the patients with BMN were considered as poor, but with better treatment aids (chemotherapy and supportive treatment), the prognoses of these patients have been found to improve. Vigorous supportive care, along with a disease specific treatment, is the key to its management. This case was worth reporting, as no cause of BMN was elicited in it and we have
Burton, Kirsteen R; Wang, Xi; Dhanoa, Deljit
Spinal epidural abscess (SEA) is a rare clinical entity. It is less common when the entire epidural space is involved, known as a holocord or panspinal SEA, and it is even less common in a pregnant patient. We report a case of methicillin-resistant Staphylococcus aureus holocord SEA in a 30-year-old female at approximately 22 weeks' gestational age who presented with lumbar pain and pelvic pressure and the urge to bear down. Magnetic resonance imaging of the spine demonstrated extensive SEA and meningitis from the foramen magnum to the lumbar spine that was treated both medically and surgically. The incidence of, clinical presentation of, and risk factors for developing SEA are discussed. If untreated, expanding SEAs produce sensory symptoms and signs, motor dysfunction, and, eventually, paralysis and death. The medical and surgical management of SEA is also discussed. SEA can have an insidious and atypical presentation despite extensive involvement of the epidural space. Therefore, the diagnosis of SEA should always be considered in patients who present to the emergency department with back pain.
Full Text Available Peripheral primitive neuroectodermal tumors (PNET are rare malignant tumors, affecting mostly children and adolescents and have been described in breast in eight case reports only. In this paper, we present a case of bilateral mammary ES/PNET where distinction between primary and metastatic diseases was discussed through a literature review. The aim of this work is to demonstrate that although rare, the possibility of PNET should be kept in mind while evaluating a palpable breast abnormality in a young female.
Huang, Eric C; Ghazikhanian, Varand; Qian, Xiaohua
Giant cell-rich osteosarcoma is a rare histologic variant of conventional osteosarcoma that affects mainly the extremities. Extraskeletal giant cell-rich osteosarcoma is therefore exceedingly rare. Here, we report the first case of this uncommon tumor involving the parotid gland in a 62-year-old male who presented with initial right jaw swelling. Radiologic work-up revealed a 6.2 cm mass involving the right parotid gland. Fine-needle aspiration cytology showed numerous multinucleated giant cells in a background of dyshesive epithelioid cells and rare clusters of spindle stromal cells, suspicious for malignancy. The subsequent excisional biopsy showed histopathologic features diagnostic for giant cell-rich osteosarcoma. Diagn. Cytopathol. 2016;44:1107-1111. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.
Mony, Shruti; Patil, Pradnya D.; English, Rebekah; Das, Ananya; Culver, Daniel A.
Sarcoidosis is a multisystem granulomatous syndrome of unknown etiology with noncaseating epithelioid granulomas being the pathognomonic pathological finding. Sarcoidosis most commonly involves the lungs and involvement of the gastrointestinal (GI) tract is uncommon. Pancreatic sarcoidosis is very rare, especially when it is the presenting feature of sarcoidosis and can masquerade as pancreatic cancer. Tissue infiltration in pancreatic sarcoidosis can lead to either a diffuse nodular appearance or a mass-like lesion. We present an interesting case of a 47-year-old woman with a 10-pack-year history of smoking who presented with sharp epigastric pain, weight loss, and elevated lipase level. CT and MRI imaging showed a 4 cm × 5 cm heterogeneous pancreatic mass with a dilated pancreatic duct and peripancreatic lymphadenopathy. Endoscopic ultrasound guided FNA revealed noncaseating granulomas with no evidence of malignancy or atypical infection. CT of the chest revealed bilateral mediastinal and hilar adenopathy with calcification, without any parenchymal abnormalities, and her angiotensin-converting enzyme level was elevated at 170 U/L. The clinical picture pointed to the diagnosis of pancreatic sarcoidosis. Given the severity of gastrointestinal symptoms related to pancreatic sarcoidosis, prednisone therapy at 0.5 mg/kg/day was initiated with complete resolution of symptoms at 8 weeks.
Full Text Available Objective. We report a rare and unusual case of bronchial carcinoma presenting with symptoms of complications of sinonasal disease. Case Report. A 66-year-old lady was referred with a 1-week history of progressive ocular pain, chemosis, and visual disturbance. Computed tomography of the paranasal sinuses revealed frontal and ethmoidal sinus opacification with orbital involvement consistent with a diagnosis of orbital cellulitis secondary to sinusitis. Surgical exploration revealed that the sinuses and right orbit were filled with soft tissue and subsequent histopathological examination of the biopsies indicating metastases from an adenosquamous bronchial carcinoma. Further imaging revealed a large, asymptomatic, bronchial primary with deposits in the brain and liver. The advanced presentation of the disease limited treatment to best supportive care. Conclusion. Orbital cellulitis and sinonasal malignancies have a similar pattern of clinical presentation, posing a potential diagnostic pitfall. There are only two previously reported cases of metastatic lung carcinoma in the frontal sinus with 15 cases of sinonasal tract involvement reported overall. There are no reported cases of adenosquamous carcinoma in the sinonasal tract.
Full Text Available En coup de sabre (linear scleroderma of face is a rare type of morphea (localized scleroderma involving frontoparietal area of the forehead and scalp. Many triggering factors have been implicated in the development of morphea like trauma, immobilization, bacille Calmette–Guérin (BCG vaccination, injections of vitamin K, mechanical compression from clothing, etc. Linear scleroderma primarily affects the pediatric population, with 67% of patients diagnosed before 18 years of age. In this article, we describe a case of 26 year old female who presented with a three months history of brownish indurated plaque of skin on the frontal and forehead regions of the head. The patient gave a history of trauma at the same site six years back. The diagnosis of morphea was made clinically supported by histopathological features of the skin biopsy. Her neurological examination was normal. ANA was negative. Brain MRI didn’t reveal any abnormality. She was treated with topical tacrolimus 0.1% ointment. The late onset en coup de sabre is a rare presentation and hence reported.
Rajput R.; Jain D; Kumar A.; Mittal A
Typhoid fever is an important health problem worldwide but its incidence is more in developing countries. Hepatic involvement is common, but both hepatic and renal involvement is rare in typhoid fever. We report a case of typhoid fever presenting with hepatitis and acute renal failure. A 17 year old male presenting with fever and pain abdomen was found to have raised blood urea, creatinine, liver enzymes and bilirubin. Widal and typhidot (IgM,IgG) test were positive. His symptoms subsided and...
Smith, Casey; Streicher, Andrew; Magnuson, Allison; Newman, Susan; Bertoli, Robert
Posttransplantation lymphoproliferative disorder (PTLD) is a recognized and extremely morbid complication of solid organ transplantation, but central nervous system involvement, particularly in isolation, is rare. There are no standardized treatment strategies for PTLD, though commonly used strategies include reduction of immunosuppression, chemotherapy, rituximab, radiation, and surgery. We present a case of an unusual morphologic variant of primary central nervous system PTLD with successful response to rituximab and cranial radiation. A 69-year-old Asian male, who underwent postrenal transplant nine years earlier, presented with a one-month history of new onset seizure activity. His evaluation revealed multiple brain lesions on magnetic resonance imaging (MRI), as well as serologic and cerebrospinal fluid studies which were positive for Epstein-Barr Virus (EBV) infection. Ultimately, he underwent craniotomy with tissue biopsy with the final pathology report showing posttransplant lymphoproliferative disorder, polymorphic type. The patient was managed with reduction in immunosuppression, rituximab therapy, and cranial radiation treatments. He had demonstrated marked improvement in his neurologic function and was ultimately discharged to inpatient rehabilitation facility. PMID:28116196
Antonio Adolfo Guerra Soares Brandão
Full Text Available Chronic meningococcemia is a rare clinical presentation within the spectrumof infections due to Neisseria meningitidis, which was first described in 1902.It is defined as a chronic and benign meningococcal bacteremia withoutmeningeal signs or symptoms with at least one week’s duration, characterizedby intermittent or continuous fever, polymorphic cutaneous rash, and migratoryarthropathy. The incidence is believed to be around 1:200,000 inhabitants. Itaffects predominantly young people and adults, and it is equally distributedbetween genders. Diagnosis may be challenging in the early stages of thedisease because of the difficulty in isolating Neisseria meningitidis (it reaches74% of positivity in advanced stages. Recently, the use of PCR for detectingNeisseria sp antigen in skin biopsies specimens has been considered for thoseculture-negative cases. The authors report a case of a 54-year-old femalepatient who sought medical attention for a five-day fever followed by arthralgiaand skin lesions predominantly in the lower limbs. The patient progressed toa toxemic clinical status that improved after the administration of antibiotictherapy, which consisted of oxacillin and ceftriaxone. The diagnosis of chronicmeningococcemia was performed after the isolation of Neisseria meningitidisin two different blood sample cultures. This is, to our knowledge, the firstcase of chronic meningococcemia described in Brazil (up to the writing of thisreport.
M. Panduranga Kamath
Conclusion: Schwannomas arising in the base of tongue are rare and are not often included in the differential diagnosis. They are usually benign and have excellent prognosis as compared to the usual malignant lesions which occur in the tongue base.
Jul 29, 2011 ... while there was an associated mild pain in the second case. In both cases, the clinical ... scan (CECT) of the brain was done to rule out coexistent neurological ... Musculocutaneous and visceral cysticercosis: A rare disease?
Silva, Igor Henrique; Cardoso, Samantha; Carvalho, Camila Nunes; Carvalho, Alessandra Albuquerque Tavares; Leão, Jair Carneiro; Gueiros, Luiz Alcino
Oral mucoceles are common lesions characterized by accumulation of mucus following rupture of a minor salivary gland duct. However, congenital mucoceles are a rare and distinctive oral condition observed in newborns. This case report details the features of a congenital labial nodule diagnosed as congenital mucocele. These lesions are rare in the oral cavity and should be diagnosed in the delivery room, but many cases are referred for further evaluation. Management is simple, and recurrence is not expected.
Fathi, Afshin; Amani, Firuz; Davoodi, Mohammad; Bahadoram, Sara; Bahadoram, Mohammad
Introduction Nasopharyngeal carcinoma among the children has been rare accounting for only 1% of all pediatric malignancies. Both genetic and environmental factors have contributed to the development of nasopharyngeal carcinoma. Among the children there was a higher rate of undifferentiated histology. The mean age of nasopharyngeal carcinoma diagnosis has been 11 years old age; and the most common site was nasopharynx. Palpable lymphadenopathy, dysphasia and neural defect were common associated signs. Case Presentation A 15-year-old boy has presented with a mass that located near by the heart in the left side of mediastinum with invasion to anterior mediastinum from two years ago. In biopsy, nasopharyngeal carcinoma, non-keratinizing type, has diagnosed while there was no involvement of nasopharyngeal region. Patient has treated by 70 Gy (2.0 Gy/fraction) radiotherapy plus concomitant chemotherapy with base of docetaxel. But the mass had no regression. Then, the patient has treated with Cisplatin 100 mg/m2 IV on days 1, 22, and 43 with radiation, then cisplatin 80 mg/m2 IV on day 1 plus fluorouracil (5-FU) 1000 mg/m2/day by continuous IV infusion on days 1 - 4 every 4wk for 3 cycles and after remission interferon beta has added to treatment for 6 months duration as a maintenance therapy. After 1 year follow up; the patient was in complete remission. In the course of therapy, only hypothyroidism has occurred. Conclusions Nasopharyngeal carcinoma in childhood, without nasopharyngeal involvement, initially could be detected in other sites such as pericardium. Also good results could be respected by cisplatin and 5-fluorouracil based neoadjuvant chemotherapy before radiotherapy plus interferon beta as a maintenance therapy in childhood aggressive nasopharyngeal carcinoma.
Full Text Available Typhoid fever is an important health problem worldwide but its incidence is more in developing countries. Hepatic involvement is common, but both hepatic and renal involvement is rare in typhoid fever. We report a case of typhoid fever presenting with hepatitis and acute renal failure. A 17 year old male presenting with fever and pain abdomen was found to have raised blood urea, creatinine, liver enzymes and bilirubin. Widal and typhidot (IgM,IgG test were positive. His symptoms subsided and deranged parameters resolved with treatment of typhoid fever.
Vikram Jeet Singh Dhingra
Full Text Available Presence of one or more digit is called as polydactyly and may manifest singly or with other genetic disorders. The frequency of polydactyly varies widely among populations. It can occur as an isolated condition or as a feature of a congenital condition. Polydactyly is a rare condition, but still rare is in form of triple great toes. We describe a case in a 4-year-old child diagnosed as triphalangism foot with no other obvious visible anomaly. Osteoplasty-combined surgery, which was ideal for anatomical reconstruction. In a 16-month follow-up period child recovered very well.
Chiriac, Anca; Costache, Irina; Podoleanu, Cristian; Naznean, Adrian; Stolnicu, Simona
Bullosis diabeticorum (BD), or diabetic bulla, is 1 of the cutaneous manifestations of diabetes mellitus, reported in 0.5% of people with diabeties in the United States, mostly in adult men who have had long-lasting and uncontrolled diabetes associated with diabetic peripheral neuropathy. However, BD in young children is a mostly unrecognized entity. We present a case of BD in a 3-year-old girl with type 1 diabetes mellitus who had been treated with insulin for 7 months prior to hospitalization. Dermatologic examination revealed a large, tense bulla filled with clear fluid and not associated with pain or pruritus, localized on the lateral margin of the right hand. A 4 mm punch biopsy was taken from the peripheral edge of the bulla under local anesthesia, and histology showed subepidermal bulla and sparse nonspecific infiltrate in the dermis; direct immunofluorescence was negative to complements C3, IgM, IgG and IgA. The particularities of the present case include the bulla in the absence of local trauma or any other complications of diabetes and associated with well-controlled type 1 diabetes. The diagnosis was certified by histology, direct immunofluorescence and clinical outcome. To the best of our knowledge, BD in young children has been previously reported in only 2 other publications.
Otu, Akaninyene Asuquo; Anikwe, Jude Chinedu; Cocker, Derek
Key Clinical Message While rare, Fahr's disease should be considered as a differential diagnosis for seizures, movement disorders, or cognitive impairment in tropical settings. Classically, bilateral calcification of the basal ganglia is seen on CT. Endemic infections, metabolic, and toxic causes should be excluded. Treatment using Levodopa is often beneficial. PMID:26509011
Freeman, Andrew M.; Fenster, Brett E.; Weinberger, Howard D.; Buckner, J. Kern; Lynch, David
We relate the case of a 40-year-old man with a history of premature birth and dextroposition of the heart who presented for an evaluation of persistent hypoxia. An unrevealing pulmonary evaluation and agitated-saline echocardiogram led to cardiac magnetic resonance imaging. This revealed a very unusual finding: a persistent left superior vena cava with insertion into the left atrium and a small connecting vein between the right and left superior venae cavae. The implications, embryology, and pathogenesis of this rare condition are discussed. PMID:23109762
Full Text Available Epithelial odontogenic tumors arise from odontogenic epithelial structures. Malignant epithelial odontogenic tumors are extremely rare. Ameloblastic carcinomas may present denovo, ex ameloblastoma or ex odontogenic cyst. Most ameloblastic carcinomas are presumed to present denovo. To date less than 45 cases of ameloblastoma with metastasis have been reported. It occurs primarily in the mandible in a wide range of age groups; no sex or race predilection has been noted. It may present as a cystic lesion with benign clinical features or as a large tissue mass with ulceration, significant bone resorption, and tooth mobility. The lesion is usually found unexpectedly after an incisional biopsy or the removal of a cyst. Histologic features of ameloblastic carcinoma shows tumor cells that resemble the cells seen in ameloblastoma, but they show cytologic atypia. Moreover, they lack the characteristic arrangement seen in ameloblastoma. The clinical course of ameloblastic carcinoma is typically aggressive, with extensive local destruction. Here we describe a rare case of ameloblastic carcinoma (secondary dedifferentiated carcinoma of mandible in a 40-year-old female patient. Ameloblastic carcinoma: Secondary dedifferentiated carcinoma of the mandible.
Full Text Available Glial heterotopia represents collections of normal glial tissue in an abnormal location distant to the central nervous system or spinal canal with no intracranial connectivity. Nasal gliomas are non-neoplastic midline tumours, with limited growth potential and no similarity to the central nervous system gliomas. The nose and the nasopharynx are the most common sites of location. Existence of glial heterotopia in the lip region is a rare developmental disorder. We report a case of large glial heterotopia in the upper lip region in a full-term female newborn which had intracranial extension with a fibrotic band. After the surgery, there was no recurrence in the follow-up period of 3 years. When glial heterotopia, which is a rare midline anomaly, is suspected, possible intracranial connection and properties of the mass should be evaluated by magnetic resonance imaging. By this way, lower complication rate and better aesthetic results can be achieved with early diagnosis and proper surgery.
Dadaci, Mehmet; Bayram, Fazli Cengiz; Ince, Bilsev; Bilgen, Fatma
Glial heterotopia represents collections of normal glial tissue in an abnormal location distant to the central nervous system or spinal canal with no intracranial connectivity. Nasal gliomas are non-neoplastic midline tumours, with limited growth potential and no similarity to the central nervous system gliomas. The nose and the nasopharynx are the most common sites of location. Existence of glial heterotopia in the lip region is a rare developmental disorder. We report a case of large glial heterotopia in the upper lip region in a full-term female newborn which had intracranial extension with a fibrotic band. After the surgery, there was no recurrence in the follow-up period of 3 years. When glial heterotopia, which is a rare midline anomaly, is suspected, possible intracranial connection and properties of the mass should be evaluated by magnetic resonance imaging. By this way, lower complication rate and better aesthetic results can be achieved with early diagnosis and proper surgery. PMID:27274134
Addison's disease is a rare condition. Its onset of symptoms most often is nonspecific contributing to a diagnostic and therapeutic delay. Acute renal failure can be the first manifestation of this disease. We report the case of a patient with Addison's disease who was initially treated for acute renal failure due to multiple myeloma and whose diagnosis was adjusted thereafter. Patient's condition dramatically improved after treatment with intravenous rehydration; injectable hydrocortisone.
Full Text Available Accessory breast tissue is rare accounting to less than 1% cases seen in females. It is usually bilateral. We report a case of 24-year-old woman with a lump in the left axilla in view of its rarity and made a differential diagnosis of fibroadenoma, which following the investigations and histopathological report was confirmed as revealed fibroadenoma in the axilla. It should also be considered as a differential diagnosis for all axillary swellings.
Valent, Peter; Berger, Jörg; Cerny-Reiterer, Sabine; Peter, Barbara; Eisenwort, Gregor; Hoermann, Gregor; Müllauer, Leonhard; Mannhalter, Christine; Steurer, Michael; Bettelheim, Peter; Horny, Hans-Peter; Arock, Michel
Mast cell leukemia (MCL) is a rare, life-threatening malignancy defined by a substantial increase in neoplastic mast cells (MCs) in bone marrow (BM) smears, drug-resistance, and a poor prognosis. In most patients, the survival time is less than 1 year. However, exceptional cases may present with a less malignant course. We report on a 49-year-old female patient with MCL diagnosed in 2013. In February 2013, first symptoms, including flushing, headache, and diarrhea, were recorded. In addition, mild anemia was detected. The disease was characterized by a massive increase in well-granulated, mature, and often spindle-shaped MCs (80 %) in BM smears. The serum tryptase level amounted to 332 ng/mL. Like in most other MCL patients, no skin lesions were detected. However, unlike in other patients, tryptase levels remained stable, and no other signs or symptoms of MCL-induced organ damage were found. Sequencing studies revealed an isolated S476I point mutation in KIT but no mutation in codon 816. The patient received histamine receptor blockers but refused cytoreductive therapy. After 9 months, still no progression or organ damage was detected. However, progression with transformation to acute MCL occurred after 12 months. We propose that the chronic type of MCL with stable conditions, absence of organ damage, and a mature MC morphology is recognized as a distinct entity that should be distinguished from the acute variant of MCL.
Parwani Anil V
Full Text Available Abstract Background Primary carcinoid tumor arising within mature teratoma of the kidney is extremely rare, and their clinicopathologic features are not well described. Our objective was to further define the clinical features and pathologic spectra of primary carcinoid tumor arising within mature teratoma of the kidney. Methods Six previously reported case reports were identified using MEDLINE and a subsequent bibliographic search of all pertinent reports and reviews was performed. We also searched the electronic medical archival records of our institution and identified one additional unreported case. Data were extracted on the demographics, predisposing factors, clinical presentation, radiographic features, gross pathology, microscopic pathology, immunophenotype, therapy, and outcome of each of these seven cases. Results Primary carcinoid tumor arising within mature teratoma of the kidney was found at a mean age of 41.4 years. Of the 7 cases, 3 were female and 4 were male. Two of the 7 cases (28.6% were associated with horseshoe kidney. It typically presented with abdominal pain without carcinoid syndrome. It typical radiologic appearance was well circumscribed partly calcified Bosniak II-III lesion. Histologically, the carcinoid tumor showed monotonous small round cells arranged in classic anastomosing cords/ribbons intermixed with solid nests. Surgery was curative, no additional treatment was required, no local recurrences occurred, and no metastases occurred in all 7 cases. The 3 cases with available outcome data were alive at the time of publication of their respective cases (mean, 5 months. Conclusion Primary carcinoid tumor arising within mature teratoma of the kidney is a rare tumor that typically presents with abdominal pain without carcinoid syndrome. It is not associated with local recurrence and metastasis, is surgically curable, and has excellent prognosis.
Introduction. Pseudohypertension is a condition which mainly occurs due to thickening-calcification of tunica intima of the arterial wall, leading to a faulty measurement of the intra-arterial blood pressure. To the best of our knowledge, this is the first case report in literature, of a pseudohypertension-like presentation in association with Morphea en plaque. Case Presentation. This is a rare presentation of a young athletic female and a professional tennis player, with pseudohypertension-like presentation. The patient had a traumatic injury to the right elbow joint; the injury occurred during a professional tennis match. The injury was managed by immobilization, physiotherapy, and Low-Level Laser Therapy. Soon after that, the patient had a circumscribed sclerotic ivory plaque affecting the skin of the right cubital fossa. The histopathology analysis, together with the serological-hematological tests and the clinical picture, along with positive Osler's signs, leads to the conclusive diagnosis of Morphea en plaque. The peculiar anatomic localization of the plaque anterior to the brachial artery leads to faulty blood pressure measurement as recorded by mercurial sphygmomanometer. Conclusion. This unique presentation of Morphea en plaque carries an important message in relation to the basic medical practice and in relation to the accurate measurement of the vital signs. PMID:28127475
Full Text Available Acute pulmonary thromboembolism (PTE is a life threatening condition which requires early diagnosis and management. Electrocardiogram (ECG is helpful for suspecting the disease. The various ECG changes are sinus tachycardia, P pulmonale, Right bundle branch block (RBBB -incomplete or complete, axis shift, S1Q3T3, T wave inversion, and ST-segment depression in leads V1-4, aVF, and Lead III, supra ventricular tachycardia, low voltage QRS complex in limb leads. In addition, sinus bradycardia and complete heart block (CHB can be seen. CHB has been reported as an exceptionally rare manifestation of acute PTE. Here, we are reporting a case of 66 year old male presented with CHB with acute pulmonary thromboembolism, who reverted to sinus rhythm after thrombolysis.
Mozaffarian, Kamran; Farahani, Mohammad Javad; Vosoughi, Amir Reza
Hereditary multiple exostoses, an autosomal dominant condition, is a common benign tumor which is characterized by the development of multiple osteochondromas. Bilateral dorsal and ventral involvement of scapulae is extremely rare without any reported case in the literature. An 18-year-old girl was referred because of bilateral prominent scapulae and left-sided pain on shoulder girdle motion especially at more than 90° abduction and forward flexion. Radiograph evaluation showed multiple exostoses on ventral and dorsal surfaces of body of both scapulae; hence simple excision of the lesion was impossible. The patient was symptom-free about 18 months after partial scapulectomy of left side. Partial scapulectomy seems to be an effective treatment for the sandwiched scapula between dorsal and ventral benign tumors.
Full Text Available Synchronous medullary carcinoma of the thyroid and small-cell carcinoma of the lung is a rare phenomenon and both these tumors are characterized by poor treatment outcome and prognosis. A 45-year-old woman presented with a progressive swelling in front and side of the neck of 3-month duration without any pulmonary symptoms. The tumor of the lung was an incidental finding on routine chest radiological examination. The diagnosis of synchronous primary cancers of the thyroid and the lung were made after cytopathological examination of both the lesions. We report here a case of loco-regional sporadic medullary carcinoma of the thyroid associated with limited stage small-cell carcinoma of the lung and its therapeutic challenges.
Brinda Rao Korivi
Full Text Available Non-Hodgkin’s lymphoma of the cervix is an extremely uncommon entity, with no standard established treatment protocol. A 43-year-old asymptomatic female with a history of dual hit blastic B-cell lymphoma/leukemia in complete remission presented with an incidental cervical mass, which was initially felt to represent a cervical fibroid on computed tomography (CT. It was further evaluated with ultrasound, biopsy, and positron emission tomography-computed tomography (PET-CT, which demonstrated a growing biopsy-proven lymphomatous mass and new humeral head lesion. The patient was started on chemotherapy to control the newly diagnosed humeral head lesion, which then regressed. She then underwent radiation to the cervix with significant improvement in the cervical lymphoma. A review of cross-sectional imaging findings of lymphoma of the cervix is provided, including how to differentiate it from other more common diseases of the cervix. Clinical awareness of rare cervical masses such as lymphoma is very important in order to achieve timely diagnosis and appropriate treatment.
Vij, Ruchieka; Vij, Hitesh
Ameloblastic fibroma is an uncommon mixed odontogenic tumour, which is often confused with ameloblastoma. It exhibits both epithelial and mesenchymal components with absence of any calcified dental structure. This paper presents two cases of this rare entity with detailed review of literature.
Full Text Available Carcinoma ex pleomorphic adenoma in lacrimal gland is a rare entity unlike its salivary gland counterpart. This rare tumor poses a diagnostic challenge to clinicians as pre-operative diagnosis is difficult and diagnosis is only by careful pathological assessment. We report this uncommon lesion in a 62-year-old lady, wherein the malignant component was mucoepidermoid carcinoma. The elderly patient remained clinically and radiologically free of the tumor for two years after complete excision of the tumor but computed tomography at the end of two and a half years showed a recurrent lesion in the region of the lacrimal gland. This makes long term follow up of patients with these rare lacrimal tumors imperative with a minimum period of at least five years.
Baker, Aaron R; Ohanessian, Sara E; Adil, Eelam; Crist, Henry S; Goldenberg, David; Mani, Haresh
Dedifferentiated epithelial-myoepithelial carcinoma (DEMC) is very rare salivary gland neoplasm with only anecdotal reports. We present an analysis of DEMC, based on a case and review of literature. Our patient, an 85-year-old woman, presented with a submandibular mass of 5 years duration that was increasing in size over a 5-week period. Histologically, there were areas of typical epithelial-myoepithelial carcinoma, with dedifferentiation of both components, manifesting morphologically as salivary duct carcinoma and areas of myoepithelial carcinoma. A review of literature revealed 21 previously reported cases of DEMC. DEMC occurs at an average age of 72 years, most often in the parotid gland (72%) followed by submandibular gland (17%). Dedifferentiation more often involves the epithelial component (13/15 cases) than the myoepithelial component (5/15 cases). Although typical epithelial-myoepithelial carcinomas are fairly indolent (average disease-free survival of 11.34 years), dedifferentiation confers a poor prognosis (survival reported from 1 to 72 months).
Full Text Available We present a case of primary hyperparathyroidism with an uncommon presentation as multiple brown tumours, which may easily be mistaken for a primary bone neoplasm. A brief literature review and its clinical and surgical management are also discussed here.
Bhardwaj, Parveen; Sharma, Ravi; Sharma, Minoo
Homocystinuria is an inborn error of amino acid metabolism in which homocystine accumulates in the blood and produces a slowly evolving clinical syndrome. We are presenting a case of a 4-year-old female child who presented to us with stroke and also had megaloblastic anemia. She was diagnosed as having homocystinuria type-1, and she responded to treatment.
Reyaz Ahmed Para,Muzamil Latief,Huma Noor
Full Text Available Spontaneous spinal epidural hematoma (SSEH is a rare entity.SSEH is an accumulation of blood in the vertebral epidural space in the absence of trauma or iatrogenic procedure like lumbar puncture.The incidence of SSEH has been estimated at 0.1 patients per 100,000 individuals and represents less than 1% of spinal space-occupying lesions .Here we are presenting case of a 65year old female hypertensive on treatment with controlled blood pressure, who presented with sudden onset paraplegia and weakness of muscles of hand, associated with urinary and bowel incontinence that started 6 hours before presenting to Emergency Room .Patient was subjected to MRI spine that revealed a collection in spinal epidural space ,hypointense in T1W images and hyperintense in T2W images, extending from C6-C7 to T1-T2 suggestive of acute / subacute epidural hematoma (fig 1.In our case all possible causes of an epidural haematoma were ruled out by relevant investigations.Patient was subjected to surgical evacuation of hematoma after 48 hours of presentation.Perioperative and Postoperative period was uneventfull . Sensations over involved areas improved. Motor weakness also improved from Grade III to Grade IV+ in bilateral lower limbs and from Grade II to Grade IV in small muscles of hand and triceps, however Patient persisited with urinary incontinence.CONCLUSION: When a patient receiving anticoagulant therapy complains of sudden and unexplained neck or back pain, we should suspect the possibility of SSEH. For early diagnosis, immediate MRI is essential .In addition, we should consider SSEH as one of the important differential diagnoses in elderly patients who have developed acute myelopathy. [Natl J Med Res 2013; 3(4.000: 424-427
Naveen, Kikkeri Narayanasetty; Athanikar, Sharatchandra Bhimrao; Hegde, Spandana Prakash; Athanikar, Vidisha Sharatchandra
Type 2 lepra reaction or erythema nodosum leprosum (ENL) is an immune complex syndrome that occurs mostly in lepromatous leprosy and sometimes in borderline lepromatous leprosy patients. We present an untreated case of lepromatous leprosy, who presented with type 2 lepra reaction and livedo reticularis. Livedo reticularis, though seen in lucio phenomenon, is not a part of type 2 lepra reaction. The case is being reported for its rarity.
Geetika Bhatt; Sandhu, Varrinder S.; Mitchell, Charlene K
Hepatitis A has a variety of associated extrahepatic manifestations that clinicians should be aware of for early diagnosis and treatment. We report a unique case of hepatitis A presenting with multiple extrahepatic manifestations not previously described in a single patient. A 34-year-old male presented with sudden onset of left sided facial pain, swelling, and skin rash, with diffuse body pains and muscle weakness, and was found to be positive for hepatitis A immunoglobulin M (IgM). He was i...
Parvaiz Anwar Rather
Full Text Available Lichen myxoedematosus (LM, a form of primary cutaneous mucinosis, may present either as localized less severe form called papular mucinosis or diffuse more severe form called scleromyxoedema. The diffuse form is almost always associated with monoclonal gammopathy, whereas localized form is not. We report an atypical case of localized form of LM associated with multiple myeloma in a 66-year-old male, who presented with asymptomatic waxy papular eruption on extremities, which on histopathological examination confirmed the diagnosis of cutaneous mucinosis. After initially being put on steroids and hydroxychloroquine with minimal improvement, patient subsequently presented with encephalopathy and on evaluation revealed hypernatremia, hypercalcemia, hypergammaglobulinemia, reversal of albumin-globulin (A/G ratio, azotemia, and lytic lesions in skull X-ray. Bone marrow aspiration and biopsy confirmed multiple myeloma. Patient was successfully treated with standard treatment regimen for multiple myeloma with bortezumib and dexamethasone and his skin lesions subsided completely.
Miranda, Benjamin H; Malahias, Marco; El-Said, Tarek F A; Fahmy, Fahmy S
A rise in incidence and decrease in mortality rates from breast cancer have lead to an increase in prevalence within developed countries. Presentation is classically with a palpable breast tissue mass that may metastasize to bone, lung, liver, brain, lymph nodes, and skin. We describe a delayed diagnosis, in an 80-year-old female patient, where, on 2 occasions, the primary initial presentation was with a cutaneous squamous cell carcinoma in the right axilla. It was not until the third referral, with an ipsilateral breast lump, that breast cancer was clinically diagnosed. This was histologically identified as the primary malignancy, most likely in-keeping with squamous cell carcinoma of the breast. This unique atypical presentation represents a diagnostic challenge and highlights a clinically relevant learning point that may avoid subsequent diagnostic delay. Cutaneous axillary lesions should be treated with a high index of suspicion, necessitating the requirement for chest examination because of the possible presence of an associated primary breast carcinoma.
Full Text Available Xeroderma pigmentosum (XP is a rare autosomal recessive genodermatosis associated with hypersensitivity to ultraviolet (UV light, due to defects in deoxyribonucleic acid (DNA repair. Basaloid squamous cell carcinoma is a rare aggressive variant of squamous cell carcinoma. Patients with XP are at increased risk of developing cutaneous malignancy and are commonly associated with squamous carcinoma. We report an extremely rare case of 8-year-old child with XP along with basaloidsquamous carcinoma of skin; and review of literature related to it.
Full Text Available Morgagni hernia is an unusual congenital herniation of abdominal content through the triangular parasternal gaps of the anterior diaphragm. They are commonly asymptomatic and right-sided. We present a case of a bilateral Morgagni hernia resulting in delayed growth in a 10-month-old boy. The presentation was unique due to its bilateral nature and its symptomatic compression of the mediastinum. Diagnosis was made by 3D reconstructed CT angiogram. The patient underwent medical optimization until he was safely able to tolerate laparoscopic surgical repair of his hernia. Upon laparoscopy, the CT findings were confirmed and the hernia was repaired.
Leshen, Michael; Richardson, Randy
Morgagni hernia is an unusual congenital herniation of abdominal content through the triangular parasternal gaps of the anterior diaphragm. They are commonly asymptomatic and right-sided. We present a case of a bilateral Morgagni hernia resulting in delayed growth in a 10-month-old boy. The presentation was unique due to its bilateral nature and its symptomatic compression of the mediastinum. Diagnosis was made by 3D reconstructed CT angiogram. The patient underwent medical optimization until he was safely able to tolerate laparoscopic surgical repair of his hernia. Upon laparoscopy, the CT findings were confirmed and the hernia was repaired.
Full Text Available True Popliteal artery aneurysm is the most common of all the peripheral artery aneurysms. We present a case of proximal popliteal artery aneurysm involvement both lower limb presented with gangrene in one lower limb and incapacitating claudication pain on the other lower limb. We have successfully repaired both sides aneurysm in the same sitting with Poly Tetra Fluro Ethylene (PTFE graft, as the patient also had multiple venous perforators’ involvement on both sides, which left us only with synthetic graft repair option rather than venous graft repair.
Kuppa; Yerukala; Dema; Sujith; Venkateswar Reddy
Septic Arthritis also known as infectious arthritis, can be bacterial or fungal arthritis. The condition is an inflammation of a joint that is caused by an infection. Typically, Septic Arthritis affects one large joint in the body such as knee or hip. Generally, Septic Arthritis is present with complete absorption of the head of the ...
Conclusion: The diagnosis of eosinophilic fasciitis is challenging due to the lack of pathognomonic signs and symptoms. As spontaneous resolution has been described, watchful waiting is defendable depending on the clinical presentation. Although magnetic resonance imaging (MRI can be useful in establishing the diagnosis, a deep muscle biopsy remains the gold standard diagnostic tool.
Sangavi Santhan, Jehangir HM, Mugunthan
Full Text Available We report a 12 year old girl with a history of migraine presenting with recurrent episodes of hemiparesis preceded by headache, which fulfilled the diagnostic criteria for sporadic hemiplegic migraine in ICHD – 2 [international classification of headache disorders 2].
[5,6] Half of the patients present with symptoms such as low back or rectal pain, pain during ... tomography [CT] or magnetic resonance imaging [MRI]) play such a crucial role in ... lytic/sclerotic lesions were seen in the lumbosacral spine. CECT.
Full Text Available In 1952 Goldenher described a case with triad of pre auricular tags, mandibular hypoplasia and ocular (epibulbar dermoid and described the case as Goldenger Syndrome. Exact etiology of this disease is not known. Here we present a case of Goldenher syndrome in a 5 days old newborn who presented with all the classical features except ocular involvement. Gorlin et.al named this syndrome as oculoauriculovertebral dysplasia due to presence of additional vertebral anomalies .2 Exact etiology of this disease is not known. Most of the cases are sporadic, though autosomal recessive, autosomal dominant and multifactorial inheritance has also been suggested.2.Chromosomal analysis shows no abnormalities.3 In this report we presented a case of Goldenger Syndrome in a 5 days old newborn who presented with all the classical features except occular involvement. Journal of College of Medical Sciences-Nepal, 2013, Vol-9, No-4, 59-62 DOI: http://dx.doi.org/10.3126/jcmsn.v9i4.10239
Full Text Available We report a case of a 1 month female presented with a 4 cm irreducible left inguinal mass, Birth history was unremarkable. Ultrasonography of the left inguinal region showed the ovary with peripheral follicles and the uterus that passed through the left inguinal canal. There were no abdominal abnormalities.
Bhatt, Geetika; Sandhu, Varrinder S; Mitchell, Charlene K
Hepatitis A has a variety of associated extrahepatic manifestations that clinicians should be aware of for early diagnosis and treatment. We report a unique case of hepatitis A presenting with multiple extrahepatic manifestations not previously described in a single patient. A 34-year-old male presented with sudden onset of left sided facial pain, swelling, and skin rash, with diffuse body pains and muscle weakness, and was found to be positive for hepatitis A immunoglobulin M (IgM). He was initially started on antibiotics for concerns of bacterial parotitis but did not show any improvement. A punch biopsy of his mandibular rash and swelling was done which showed lymphohistiocytic infiltration with a few eosinophils. A trial of prednisone resulted in improvement of his symptoms. Clinicians should be aware to look for hepatitis A infection in a patient with atypical clinical picture causing a widespread systemic inflammatory response. Treatment with prednisone may result in resolution.
Full Text Available Hepatitis A has a variety of associated extrahepatic manifestations that clinicians should be aware of for early diagnosis and treatment. We report a unique case of hepatitis A presenting with multiple extrahepatic manifestations not previously described in a single patient. A 34-year-old male presented with sudden onset of left sided facial pain, swelling, and skin rash, with diffuse body pains and muscle weakness, and was found to be positive for hepatitis A immunoglobulin M (IgM. He was initially started on antibiotics for concerns of bacterial parotitis but did not show any improvement. A punch biopsy of his mandibular rash and swelling was done which showed lymphohistiocytic infiltration with a few eosinophils. A trial of prednisone resulted in improvement of his symptoms. Clinicians should be aware to look for hepatitis A infection in a patient with atypical clinical picture causing a widespread systemic inflammatory response. Treatment with prednisone may result in resolution.
Full Text Available Patients with HIV are prone to a number of unusual infectious and malignant conditions, typically resulting from declining immune function. However, there are also a few of reports of HIV associated conditions potentially created by viral release of interleukin-6 (IL-6. Herein, we present a case of HIV IL-6-related systemic inflammatory syndrome, a Kaposi sarcoma (KS-associated syndrome in the absence of multicentric Castleman disease (MCD.
Full Text Available Septic Arthritis also known as infectious arthritis, can be bacterial or fungal arthritis. The condition is an inflammation of a joint that is caused by an infection. Typically, Septic Arthritis affects one large joint in the body such as knee or hip. Generally, Septic Arthritis is present with complete absorption of the head of the Femur in infants. A case was encountered in which the complete absorption of the femoral head was seen in adults also
Elroy Patrick Weledji
Full Text Available We present a case of an unsual type of obstructed indirect inguinal hernia with impending strangulation. The operative findings revealed a sliding Maydl’s hernia with an ischemic inner ileal loop and an adherent inflamed appendix. This case highlights the importance of intraoperative examination of the intra-abdominal bowel loops proximal to the hernia sac of an incarcerated, obstructed, or strangulated hernia.
Hung, T-L; Wang, W-M; Chiang, C-P
Gout is a systemic disorder characterized by hyperuricemia and recurrent arthritis, most involvement of ankles, midfoot joint and first metatarsophalangeal joint, with monosodium urate crystals deposition in synovial fluid and other tissues. We present a case of 53-year-old male, who had several nontender, white-yellow papules and plaques over his elbows, knees and arms with chalk-like substances and crust on inflammatory base wax and wane in the past 2 years. Upon histopathology examination of the skin lesions, it reported as intradermal urate tophi and miliarial gout was diagnosed. This case highlights the importance of considering unusual cutaneous tophi in the differential diagnosis of deposition disorders.
Full Text Available A 12yrs male child presented in the emergency department of VSS Medical College Burla with complains of acute pain over left testis for 3days, associated with vomiting, there was no h/o fever, trauma or lower urinary tract symptoms. Ultrasonography of scrotum revealed features of testicular torsion with absent vascularity. He was managed with urgent scrotal exploration and found to have torsion of cord and gangrene of testis. Orchidectomy was done for testis with orchiopexy of the contralateral testis.
Shahcheraghi, Seyed Hossein; Ayatollahi, Jamshid
Brucellosis is the most widespread zoonotic infection in the world. The disease is endemic in countries bordering the Mediterranean Sea. It is an important re-emerging infectious disease. This disease is closely associated with the evolution of mankind as an agrarian society linked to the practice of shepherding and popularization of animal husbandry. The patients with this disease are typically present with chills, fever, asthenia and sweating. This paper describes a patient with brucellosis and skin rashes on the leg. A 41-year-old man presented with fever, ataxia, and dysarthria. He was a shepherd. The patient reported the loss of appetite, arthralgia and weight loss during previous five months. Finally, he was diagnosed with brucellosis by positive blood culture and high titer for Brucella agglutination test. The clinical manifestation of brucellosis is very broad, ranging from asymptomatic infection to serious debilitating disease. Current patient had skin rashes on his leg. Brucellosis must be considered in the differential diagnosis of acute infections, especially if there is a history of fresh milk product ingestion and living in an endemic region.
Seyed Hossein Shahcheraghi
Full Text Available Brucellosis is the most widespread zoonotic infection in the world. The disease is endemic in countries bordering the Mediterranean Sea. It is an important re-emerging infectious disease. This disease is closely associated with the evolution of mankind as an agrarian society linked to the practice of shepherding and popularization of animal husbandry. The patients with this disease are typically present with chills, fever, asthenia and sweating. This paper describes a patient with brucellosis and skin rashes on the leg. A 41-year-old man presented with fever, ataxia, and dysarthria. He was a shepherd. The patient reported the loss of appetite, arthralgia and weight loss during previous five months. Finally, he was diagnosed with brucellosis by positive blood culture and high titer for Brucella agglutination test. The clinical manifestation of brucellosis is very broad, ranging from asymptomatic infection to serious debilitating disease. Current patient had skin rashes on his leg. Brucellosis must be considered in the differential diagnosis of acute infections, especially if there is a history of fresh milk product ingestion and living in an endemic region.
Full Text Available Central giant cell granuloma (CGCG is an intra-osseous lesion consisting of cellular fibrosis tissue containing multiple foci of hemorrhage, multinucleated giant cells and trabecules of woven bone. This lesion accounts for less than 7% of all benign jaw tumours. Jaffe considered it as a locally reparative reaction of bone, which can be possibly due to either an inflammatory response, hemorrhage or local trauma. Females are affected more frequently than males. It occurs over a wide age range.It has been reported that this lesion is diagnosed during the first two decades of life in approximately 48% of cases, and 60% of cases are evident before the age of 30. It is considerably more common in the mandible than in the maxilla. Most lesions occur in the molar and premolar area, some of these extending up to the ascending ramus. The presence of giant cell granuloma in the mandibular body area, the entire ramus, condyle and coronoid represents a therapeutic challenge for the oral and maxillofacial surgeons. The aim of this report is to describe an unusual presentation of central giant cell granuloma involving the mandibular body, ramus, condylar and coronoid processes, and to discuss the differentiated diagnosis, the radiographic presentation and the management of this lesion.
Gul, Maryam; Chaudhry, Ammar A; Chaudhry, Abbas A; Sheikh, Mubashir A; Carsons, Steven
Numerous autoimmune diseases can affect the central nervous system (CNS), and variable clinical presentations confound the differential diagnosis. The challenging task of properly characterizing various CNS autoimmune diseases enables patients to be rapidly triaged and appropriately treated. In this review article, we aim to explore different CNS manifestations of rheumatologic diseases with emphasis on the utility of imaging and cerebrospinal fluid findings. We review the classic physical examination findings, characteristic imaging features, cerebrospinal fluid results, and serum biomarkers. In addition, we also present a unique case of newly described autoimmune entity CLIPPERS syndrome. Our case is unique in that this is the first case which demonstrates involvement of the supratentorial perivascular spaces in addition to the classic infratentorial involvement as initially described by Pittock et al (Brain. 2010;133:2626-2634).
Mizrahi, Cezar J; Cohen, José E; Gomori, J M; Shoshan, Yigal; Spektor, Sergey; Moscovici, Samuel
We review our experience with four patients who presented to our Medical Center from 2005-2015 with adult idiopathic occlusion of the foramen of Monro (FM). All patients underwent CT scanning and MRI. Standard MRI was performed in each patient to rule out a secondary cause of obstruction (T1-weighted without- and with gadolinium, T2-weighted, fluid-attenuated inversion recovery [FLAIR] and diffusion-weighted imaging [DWI] protocols). When occlusion of the FM appeared to be idiopathic, further high-resolution MRI with multiplanar reconstructions for evaluation of stenosis or an occluding membrane at the level of the FM was performed (T1-weighted without- and with gadolinium, T2-weighted 3D turbo spin-echo). Occlusion of the FM was due to unilateral stenosis and septum pellucidum deviation in two patients, to an occluding membrane in one, and to bilateral stenosis in one patient. Urgent surgical intervention is mandatory when there are signs of increased intracranial pressure while asymptomatic patients may be managed conservatively. In this patient series, truly bilateral stenotic obstruction of the FM was best managed with ventriculoperitoneal shunt and patients with membranous obstruction or unilateral stenosis with septum deviation were treated endoscopically. Copyright © 2016 Elsevier Ltd. All rights reserved.
Amal F Radwan
Full Text Available A 37-year-old hypertensive housewife presented with a sudden onset of left-sided hemiplegia, hemianaesthesia, dysarthria and urinary incontinence. The condition was preceded by recurrent attacks of motor neurological deficits over a 3-year duration. She reported a history of a fall from a height at the age of 10, which was followed by a hearing deficit and a history of two caesarean sections after eclampsia. The blood pressure was 170/100 mmHg. Laboratory investigations revealed hyperglycaemia (fasting glucose 306 mg/dl and normal kidney function tests. The computed tomography scans revealed old multiple bilateral cerebral infarcts with recent intracranial haemorrhage in the right parietal region. The inflammatory markers (ESR and CRP and immune profile (ANA, anti-ds DNA and ANCA were found to be normal. Cerebral angiography revealed a complete occlusion of the intracranial parts of both internal carotid arteries at their supraclinoid segments along with the proximal parts of the anterior cerebral artery and middle cerebral artery, with collaterals from the posterior circulation. Consequently, the diagnosis of moyamoya disease with the collaterals was confirmed. Antihypertensive medications and insulin were administered. Cerebral dehydration measures were undertaken with partial improvement. A superficial temporal artery-middle cerebral artery bypass operation was performed with some postoperative improvement. One month later, she suffered a new stroke with severe impairment of the level of consciousness; the computed tomography scans revealed a large recent cerebral infarct, her condition deteriorated rapidly and she died shortly thereafter.
Ali Raghibi; Wan Hazabbah Wan Hitam; Raja Azmi Mohd Noor; Zunaina Embong
A 58-year-old Malay lady, presented with gradual loss of vision in the right eye for 1 month duration. It was associated with right sided headache, nausea and vomiting. Patient had a history of contact with tuberculosis patient. On examination, visual acuity in right eye was no perception of light with presence of relative afferent pupillary defect. The left eye visual acuity was 6.0/7.5. There was a total restriction of extraocular movement in all direction (frozen eye) in the right eye. Both anterior segments were unremarkable. Fundoscopy in both eyes showed normal appearance of optic disc and retinal vessels. Other cranial nerves were normal. Lungs were clear and had no lymphadenopathy. Chest radiograph was normal. Erythrocyte sedimentation rate was elevated by 35 mm/h and Mantoux test was positive (20 mm). MRI of brain and orbit demonstrated thickening and irregularity of the right optic nerve on axial view with doughnut sign on coronal view. Patient was diagnosed to have optic perineuritis secondary to tuberculosis. She was treated with anti-TB drugs. Systemic corticosteroid was commenced after 2 weeks. The visual acuity was improved to hand motion after 1 month with almost complete resolution of extraocular movement.
Hasan, Roumina; Kumar, Sandeep; Rao, Anuradha ck; Kadavigere, Rajagopal
A 55-year-old woman presented with a mucopurulent sinusal discharge from the right supragluteal region, with symptoms over the previous five months. This abscess began as a slowly swelling growth, which eventually turned into a discharging sinus, and she was diagnosed with a gluteal abscess. The patient underwent incisional drainage, and intra-operatively, the sinus tract could be seen extending to the retroperitoneum. A subsequent CT scan and an MRI of the abdomen revealed a large heterogeneous retroperitoneal cystic mass on the right side of midline, extending inferiorly into the anterior thigh along the iliopsoas. Superiorly, a tubular projection extended from the lesion, indenting the ileocaecal junction, while a fluid filled cutaneous fistulous tract was seen, extending to the right flank. A diagnosis of pseudomyxoma retroperitonei, likely of retrocaecal appendicular origin, was proposed. An explorative laparotomy with an appendectomy, and the evacuation of the retroperitoneal collection were completed. The subsequent histopathology confirmed the diagnosis of appendicular mucinous cystadenoma, with pseudomyxoma retroperitonei. PMID:27660549
Zawar, Vijay; Godse, Kiran
A 34-year-old nonpregnant woman noted a mildly pruritic skin lesion on her right breast for 1 week following an episode of coryza, malaise, and low-grade fever of 3 days' duration. The latter symptoms subsided without any treatment. Ketoconazole cream prescribed by her family physician did not resolve the breast eruption after 1 week, so was stopped. A week later, multiple skin lesions erupted suddenly on her right chest wall starting near the herald patch in midaxillary line and spread distally until the midback. She then consulted the authors. She denied a history of abrasion or trauma to the affected areas. Travel, contact, sexual, and drug histories were unremarkable. She categorically denied past or family history of eczema, psoriasis, contact dermatitis, and drug eruptions. On examination, the initial lesion was an annular and well-demarcated erythematous plaque on her right breast in the upper outer quadrant. Multiple small oval scaly plaques were noted, extending distally along the ribs to the midline on her back, not crossing the midline, predominantly over the distribution of right T4 dermatome (Figure 1). Peripheral collarette scaling was noted (Figure 2). A few scattered small lesions were also noted in the vicinity of this dermatome. Three isolated small plaques were also present on the trunk, one in the supramammary area and the other two on the abdomen and back, respectively. Palmoplantar and mucosal surfaces were uninvolved. The rest of the skin and systemic examination revealed no abnormalities. Complete blood cell counts, fasting glucose, and urinalysis were normal. Venereal Disease Research Laboratory (VDRL) results were nonreactive and human immunodeficiency virus (HIV) antibodies were negative. Repeat testing ofVDRL in serial dilutions and HIV antibodies after 3 months were also nonreactive and negative, respectively. Scrapings from the initial large lesion and subsequent smaller eruptions did not show any evidence of fungal infection on
Franco-Avilés, Luis; Arce-Guridi, Héctor Tonalli; Mercado, Ulises
Introducción: el síndrome de Bouveret se caracteriza por una obstrucción de la salida gástrica, causado por un cálculo en el bulbo duodenal, después de pasar a través de una fístula colecistoduodenal. Caso clínico: reportamos el caso clínico de una mujer de 46 años de edad, quien se presentó con dolor epigástrico intermitente, nausea, vómito y pérdida de peso de 2 meses de duración. No tenía fiebre, deshidratación o ictericia. La paciente admitió uso de alcohol y abuso de metanfetamina. La amilasa, enzimas, electrolitos y creatinina estuvieron dentro de los límites normales. Siete meses antes había ingresado a Urgencias por colecistitis aguda. El ultrasonido fue reportado como colelitiasis sin dilatación de la vía biliar intra/extrahepática. Las radiografías simples de abdomen no mostraron hallazgos relevantes. Recibió tratamiento conservador y fue egresada. Se programó para cirugía laparoscópica. Durante el procedimiento se encontró un plastrón subhepático con adherencias firmes, por lo que se efectuó cirugía abierta. La vesícula se encontró adherida al bulbo duodenal y un cálculo impactado en el duodeno. Se removió el calculo por enterotomía. Habia leve distension gastrica. Debido a la inflamacion regional, el cirujano decidio efectuar Bilroth 1; no hubo complicaciones. Conclusiones: las manifestaciones clínicas del síndrome de Bouveret son inespecíficas. El diagnóstico preoperatorio es un desafío para el clínico por la rareza del padecimiento. El tratamiento debe individualizarse.
Sarkar, Santanu; Panja, Soumyajyoti; Bandyopadhyay, Arghya; Roy, Shreosee; Kumar, Sandeep
Rhinosporidiosis is a chronic granulomatous infection caused by Rhinosporidium seeberi. Sporadic cases of rhinosporidiosis has been reported from many countries but is endemic in Southern India (Madurai, Ramnad, Rajapalayam and Sivaganga), Nepal, Bangladesh and Sri Lanka. This disease commonly affects the mucous membrane of the nose or naso-pharynx and presents as a leafy, polypoidal mass. The reported extranasal sites include the oro-pharynx, eye, ear, larynx, trachea, bronchi, skin and genital mucosa. It may also become disseminated to present as a generalized form. In our case a 40-year-old female from rural West Bengal (Eastern India) presented with right sided facial swelling. Our provisional diagnosis was parotid duct cyst on the basis of careful history, scrupulous clinical examination and relevant investigations comprising CECT scan of face. Although Rhinosporidiosis was not taken into consideration in the clinical differential diagnosis, it was eventually diagnosed postoperatively by histopathological examination of surgical specimen. Two months later in follow-up, the same patient presented to us with left sided facial swelling. We managed the left facial swelling successfully with minimally invasive surgery and 100mg twice daily dapsone for 6 months. We present this case firstly because Rhinosporidiosis of parotid duct (stensen's duct) is a rare entity and secondly non-neoplastic cysts of the salivary glands are also uncommon and represent only 2-5% of all salivary gland lesions. Furthermore our case emphasizes that the clinicians should aware of this rare clinical entity specially in endemic areas, because minimally invasive techniques and medications might solve the problem while helping patients to avoid surgical excision.
Ravi Kumar TV, GadiDaksh, Jain Vinay, Rangaswamy BT
Full Text Available ABSTRACT Sternum fractures are a rare entity and occur either due to direct trauma or indirectly associated to a flexion compression injury. Earlier literatures used to describe the association of sternum fractures with upper thoracic vertebral fractures. To the best of our knowledge very few cases have been described in literature with concomitant lumbar vertebral fracture with associated sternum fracture. We hereby report a rare presentation of a flexion distraction injury leading to a concomitant sternum fracture with a L1 vertebral burst fracture.
Verma, Pradhuman; Verma, Kanika Gupta; Verma, Dinesh; Patwardhan, Nitin
Brown tumors are erosive bony lesions caused by rapid osteoclastic activity and peritrabecular fibrosis due to primary or secondary hyperparathyroidism resulting in a local destructive phenomenon. The differential diagnosis based on histological examination is only presumptive. Clinical, radiological and laboratory data are necessary for definitive diagnosis. Here, we report a very rare case of brown tumor involving maxilla and mandible, which is the result of secondary hyperparathyroidism in 30-year-old female patient with chronic renal disease. PMID:25328310
Full Text Available Malignant peripheral nerve sheath tumor (MPNST is a rare tumor that accounts for 5% of all thoracic neoplasm usually located in the posterior mediastinum and is generally associated with a poor outcome. We present a case of MPNST of the anterior mediastinum presenting in a rare location leading to diagnostic dilemmas and treated primarily by surgical resection.
Yoon, Young-Ho; Park, Won-Young; Choi, Young-Jin; Cho, Kyu-Sup
Non-Hodgkin's lymphoma (NHL) affecting the ethmoid sinus and epiglottis is uncommon. Furthermore, synchronous NHLs involving the ethmoid sinus and epiglottis are extremely rare and have not been reported previously. This article reports synchronous, primary, diffuse, large B-cell lymphoma (DLBCL) arising in the ethmoid sinus and epiglottis, which was successfully treated by immunochemotherapy. A careful examination of the head and neck is necessary to determine the existence of multiple synchronous primary tumors, because primary synchronous occurrence of DLBCL in the head and neck is unusual and can impact the prognosis adversely.
Jayalakshmy, P S; Subitha, K; Priya, P V; Johnson, Gerald
Epidermal cyst is a very common benign cystic lesion of the skin. It is usual to find ulceration of the lining epithelium, rupture of the cyst wall with chronic inflammation and foreign body giant cell reaction. But, it is very rare to see an epidermal cyst with marked accumulation of melanin pigment. Only a few cases of pigmented epidermal cyst with dense collection of melanin pigment have been published in the literature. Here, we are reporting a case of ruptured epidermal cyst with keratin granuloma formation and showing dense collection of melanin pigment.
Verma, Rajesh; Bhandari, Aveg; Tiwari, Navin; Chaudhari, Tejendra S
Wilson disease (WD) is one of the few inherited but treatable disorder mainly affecting the liver and brain resulting in severe disability or death if left untreated. Hence, it is important to keep a high index of suspicion for diagnosing this clinical entity in appropriate clinical settings. The clinical presentation can be quite variable and they may present solely with neurological features sans hepatic symptoms. Such neurological manifestations usually follow subacute to chronic course. A...
Bhagat, Sanjeev; Varshney, Saurabh; Bist, Sampan S; Gupta, Nitin
Schwannomas are rare, benign, slowly growing neurogenic tumors that originate in peripheral, spinal, or cranial nerves other than the optic and olfactory nerves. In the head and neck, these tumors usually arise from the vagus nerve and the cervical sympathetic chain in the parapharyngeal space. Cervical sympathetic chain schwannomas represent a rare subgroup of schwannomas; fewer than 60 cases have been reported in the literature. These tumors are rarely seen in children and adolescents. Because patients typically present with an asymptomatic neck mass and vague complaints, clinical suspicion is important in making the diagnosis. Neural deficits at presentation are uncommon. The presence of features of Horner syndrome before excision is very rare, having been previously reported in only 10 cases of cervical sympathetic chain schwannoma. We report an extremely rare case of a cervical sympathetic chain schwannoma in a child who presented with Horner syndrome. The lesion was successfully excised.
Full Text Available We report a case of suppurative vasitis that presented as an inguinal mass in a n elderly man. Infectious vasitis, while rarely reported in the literature, is thought to be caused by Escherichia coli, mycobacteria causing tuberculosis, and other rare urogenital pathogens such as Haemophilus influenza. We report a case of tuberculous vasitis occurring as a primary infectious suppurative vasitis is rare and presenting as an inguinal mass is unusual. Only, very few cases were reported in literature with such presentation.
Full Text Available Malignant melanoma, as the name implies, is a malignant tumor of melanocytes, found in the skin, eyes, meningeal lining and the mucosal epithelium of the aero-digestive and genitourinary tracts. Malignant melanoma is typically skin malignancy, which rarely presents at extracutaneous site. Here we present a rare case of primary retroperitoneal melanoma and review the findings in comparison with other cases described in literature.
Full Text Available Background. Spontaneous perforation of the extrahepatic biliary system is a rare presentation of gall stones. Very few cases of bile duct perforation have been reported in adults. It is rarely suspected or correctly diagnosed preoperatively. Case Presentation. A 66-year-old female presented at the surgical emergency with 3 days’ history of severe upper abdominal pain with distension and repeated episodes of vomiting, as she had evidence of generalized peritonitis and underwent an exploratory laparotomy. A single 0.5 cm × 0.5 cm free perforation was present on the anterolateral surface of the common bile duct at the junction of cystic duct. A cholecystectomy and the CBD exploration were performed. Conclusion. Spontaneous perforation of the extrahepatic bile duct is a rare but important presentation of gall stones in adults. Therefore, awareness of the clinical presentation, expert ultrasound examination, and surgery are important aspects in the management.
Pradipta Guha; Debasis Sarkar; Indranil Thakur; Partha Sardar; Sekhar Mukherjee; Sanjoy Kumar Chatterjee
@@ Introduction Nodular lymphocyte predominant Hodgkin's disease occurs in 5% of all cases of Hodgkin's disease. It occurs more commonly in male. Only 5% of all Hodgkin's lymphoma cases develop spinal cord compression and in only 0.2% cases, spinal cord compression occurs as the initial presentation. So our case is rare both in the form of the unique variety of Hodgkin's disease and also in the form of presentation.
Full Text Available Pancoast syndrome is a common presentation of bronchogenic carcinoma, but other malignancies are rarely cited as its cause. Pancoast syndrome due to non-Hodgkin′s lymphoma is rarely described in the literature. Here, we report a case of Pancoast syndrome due to non-Hodgkin′s lymphoma to increase the awareness of the clinicians regarding essentiality of tissue diagnosis of Pancoast tumor before starting the treatment.
Ashok Nakum; Kesharmal Kumawat; Hiral Chauhan; Jayesh V Parikh
Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a congenital malformation characterized by an absence of the vagina associated with a variable abnormality of the uterus and the urinary tract but functional ovaries. We are reporting atypical presentation of rare case. Patient had obstructed left inguinal hernia with genitourinary and skeletal deformity. Latter it diagnosed as Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome which is a rare disease. [Natl J Med Res 2013; 3(4.000): 409-411
Banshi Lal Kumawat
Full Text Available Sporadic Creutzfeldt-Jakob disease (sCJD can have varied clinical presentation depending upon the genotype at codon 129. The common presenting clinical features of sCJD are rapid onset cognitive impairment, ataxia, psychosis and visual signs (field defects, distortion, cortical blindness. Alien limb sign was first described in patients with corpus callosal tumors and later with other neurodegenerative conditions like corticobasal degeneration. Alien hand complaints as the presenting feature of sCJD has been described in literature, but simultaneous alien hand and leg has been rarely described as presenting feature of sCJD. We describe here a case of a 55-year-old man who presented with progressive left alien hand and leg as the sole clinical manifestation of probable sCJD.
Kumawat, Banshi Lal; Sharma, Chandra Mohan; Nath, Kunal; Acharya, Mihir; Khandelwal, Dinesh; Jain, Deepak
Sporadic Creutzfeldt-Jakob disease (sCJD) can have varied clinical presentation depending upon the genotype at codon 129. The common presenting clinical features of sCJD are rapid onset cognitive impairment, ataxia, psychosis and visual signs (field defects, distortion, cortical blindness). Alien limb sign was first described in patients with corpus callosal tumors and later with other neurodegenerative conditions like corticobasal degeneration. Alien hand complaints as the presenting feature of sCJD has been described in literature, but simultaneous alien hand and leg has been rarely described as presenting feature of sCJD. We describe here a case of a 55-year-old man who presented with progressive left alien hand and leg as the sole clinical manifestation of probable sCJD. PMID:25745324
Daniel S. Treister
Full Text Available A 15-year-old male high school football player presented with episodes of headache and complete body stiffness, especially in the arms, lower back, and thighs, immediately following a football game. This was accompanied by severe nausea and vomiting for several days. Viral meningitis was suspected by the primary clinician, and treatment with corticosteroids was initiated. Over the next several weeks, there was gradual symptom improvement and the patient returned to his baseline clinical status. The patient experienced a severe recurrence of the previous myriad of symptoms following a subsequent football game, without an obvious isolated traumatic episode. In addition, he experienced a new left sided headache, fatigue, and difficulty ambulating. He was admitted and an extensive workup was performed. CT and MRI of the head revealed concurrent intracranial and spinal subdural hematomas (SDH. Clinical workup did not reveal any evidence of coagulopathy or predisposing vascular lesions. Spinal SDH is an uncommon condition whose concurrence with intracranial SDH is an even greater clinical rarity. We suggest that our case represents an acute on chronic intracranial SDH with rebleeding, membrane rupture, and symptomatic redistribution of hematoma to the spinal subdural space.
Waseem M Hajjar
Full Text Available Congenital H-type tracheoesophageal fistula (TEF in adults is a rare presentation and can test the diagnostic acumen of a surgeon, endoscopist, and the radiologist. These undetected fistulas may present as chronic lung disease of unknown origin because repeated aspirations can lead to recurrent lung infections and bronchiectasis. Congenital TEFs should be considered in the diagnosis of infants and young adults with recurrent respiratory distress and/or infections. Here, we present the successful management of this rare case in an adult patient.
Conclusions and importance: An iris abscess is a rare clinical presentation of intravenous drug use-associated endogenous endophthalmitis and as a result may present a diagnostic challenge as it requires a high level of clinical suspicion and a detailed social history to elicit the drug abuse. Early diagnosis and aggressive therapy is the key to better visual outcomes in these patients.
Guyon's canal syndrome due to tortuous ulnar artery with DeQuervain stenosing tenosynovitis, ligamentous injuries and dorsal intercalated segmental instability syndrome, a rare presentation: a case report.
Zeeshan, Muhammad; Ahmed, Farhan; Kanwal, Darakhshan; Khalid, Qazi Saad Bin; Ahmed, Muhammad Nadeem
The Guyon's canal syndrome is a well known clinical entity and may have significant impact on patient's quality of life. We report a case of 43-year-old male who presented with complaints of pain and numbness in right hand and difficulty in writing for past one month. On imaging diagnosis of Guyon's canal syndrome because of tortuous ulnar artery was made with additional findings of DeQuervain's stenosing tenosynovitis and dorsal intercalated segmental instability syndrome with ligamentous injury and subsequently these were confirmed on surgery.Although it is a rare syndrome, early diagnosis and treatment prevents permanent neurological deficits and improve patient's quality of life.
Uzuncakmak, Tugba Kevser; Karadag, Ayse Serap; Izol, Belcin; Akdeniz, Necmettin; Cobanoglu, Bengu; Taskin, Secil
Allergic contact dermatitis is a common skin disease, which affects approximately 20% of the population. This reaction may present with several clinical manifestations. Erythema multiforme-like allergic contact dermatitis is a rare type of non-eczematous contact dermatitis, which may lead to difficulty in diagnosis.Essential oil of Laurus nobilis is widely used in massage therapy for antiinflammatory and analgesic effects. Laurus nobilis induced contact dermatitis has been reported in the literature but an erythema multiforme-like presentation is rare.
Narrotam A. Patel
Full Text Available Reporting a rare case of a 17-year-old lady with late presentation of congenital diaphragmatic hernia. She presented with vague abdominal pain and postprandial vomiting. She underwent a diagnostic upper GI scopy with no significant findings. Chest X-ray and barium study for stomach were performed. Then CT scan was performed and showed herniation of bowel loops, mesentery and spleen into the left thoracic cavity through a large defect in the posterolateral aspect of the left hemidiaphgram. After the patient was operated, content reduced and defect was repaired. Thus CDH in adolescence is rare and a high index of suspicion is required.
Full Text Available Lip pits are a rare congenital anomaly that presents on the upper or lower lip or the commissure of the lips. Lip pits are an autosomal dominant trait occurring almost always in association with cleft lip or palate. They most commonly occur in association with developmental disturbances such as Van der Woude's syndrome, popliteal pterygium syndrome, oro-facial-digital syndrome, Marres-Cremers syndrome, and Hirschsprung disease. Its occurrence in nonsyndromic individuals is extremely rare with only a handful of cases reported. The identification of lip pits with other associated anomalies is crucial for genetic counseling; we report a case of nonsyndromic presentation of bilateral lip pits.
Full Text Available Neurofibromas commonly involve peripheral nervous system. Isolated neurofibroma of vagina is very rare tumor and usually associated with Von Recklinghausen’s disease. Vulva is the most frequent location of neurofibroma of genital tract followed by clitoris and labia. We present a rare case of neurofibroma of vaginal wall presented as vaginal cyst in a 52 year old female with no history of any other symptoms related to Recklinghausen’s disease. Excision biopsy was done and on the histopathological examination non-encapsulated, well circumscribed mass composed of spindle shaped cells with wavy nuclei and bland nuclear chromatin was noted. Immunohistochemistry revealed strong positivity with S-100.
Full Text Available Cervical and supraclavicular lymph nodes metastases generally arise from carcinomas of the head and neck. Metastases to cervical lymph nodes from the prostate are very rare. The common site for lymph node metastasis from the prostate is infradiaphrag- matic. Herein, we report a 69 year-old male who presented with cervical lymph node enlargement in the setting of prostate cancer. This type of presentation although rare, may warrant investigating the prostate as a source of metastasis in cases where the head and neck are free from carcinoma.
Lindfay Laura Lau
Full Text Available A 34-year-old patient with a swelling over the upper eyelid for nearly 1 year was seen in our clinic. The history, examination and investigations were suggestive of a benign lacrimal gland tumor. The tumor and lacrimal gland were resected. Subsequent histopathological examination revealed the tumor was a primary ductal adenocarcinoma of the lacrimal gland. This is a very rare tumor with less than half a dozen cases reported so far. This case report is being presented to highlight an unusual presentation of this rare malignancy.
Vijayalaxmi Veerabasappa Suranagi
Full Text Available A 55-year-old man presented with multiple, itchy papules and macules on the trunk and extremities. Histopathologic examination of biopsy specimens taken from three different lesions showed a subepidermal blister with amyloid deposits in the dermal papillae. No systemic disease or involvement of other organs was detected. The clinical and histological findings were compatible with a bullous variant of lichen amyloidosis (LA. Primary cutaneous localized amyloidosis usually presents with papular, macular or nodular lesions. Bullous lesions associated with LA are very rare. Furthermore, patient had seven other members in the family with similar lesions, which is also a rare occurrence. We report a case with a rare combination of biphasic, bullous variant of familial LA.
Filippou, Dimitrios K; Vezakis, Antonios; Filippou, Georgios; Condilis, Nicolas; Rizos, Spiros; Skandalakis, Panagiotis
A rare case of ectopic pancreas in the ampulla of Vater presented with obstructive jaundice. Ectopic pancreas is a common congenital disorder which is referred to as pancreatic rest. The incidence in autopsy series varies from 1 to 2% (range 0.55 to 13%). Most common site of ectopic pancreatic tissue is the stomach, although it can be found anywhere in the foregut and the proximal midgut. Ectopic pancreatic tissue in the ampulla of Vater is a very rare condition. Searching in the literature, using the terms "ectopic pancreas" and "Ampulla of Vater", we found only 10 records. The authors report on a rare case of a 69 years old female with ectopic pancreas in the ampulla of Vater, presented with painless obstructive jaundice, and the diagnostic and therapeutic strategy that followed.
Full Text Available Genitourinary tuberculosis (GUTB is a rare extrapulmonary manifestation of tuberculosis (TB. Various forms of presentation are described and in most cases the disease results in calcification, atrophy, or necrosis of the renal parenchyma. The kidney is not generally palpable except in cases of hydronephrosis due to an upper ureteric stricture. We present a case of GUTB presenting as inflammatory pseudotumor. This case was initially diagnosed as renal malignancy and managed accordingly. Histopathology confirmed the diagnosis of pseudotumoral renal TB.
Daruwalla, Z J
Digital ischaemia has been sparsely reported in current literature. Its association with lymphomatous conditions has been described in even more exceptional occurrences. We present the first case of upper arm ischaemia associated with non-Hodgkin\\'s lymphoma. A brief literature review of this rare phenomenon is also accompanied with it.
Hirselj, Daniel A.; Jayanthi, Venkata R. [Nationwide Children' s Hospital, Department of Pediatric Urology, Columbus, OH (United States); Lowe, Gregory K. [The Ohio State University Medical Center, Department of Urology, Columbus, OH (United States)
Urethral duplication is an uncommon clinical finding, and classification systems have been described to explain the clinical findings. Urethral triplication is an extremely uncommon diagnosis, with few published case reports. We present the rare case of an infant noted to have a bifid urinary stream and found to have urethral triplication on voiding cystourethrography. (orig.)
Lopes, Jailson Rodrigues; Rosemberg, Laercio Alberto; Lourenco, Rafael Burgomeister; Cerri, Giovanni Guido [Universidade de Sao Paulo (InRad/HC/USP), SP (Brazil). Hospital das Clinicas. Inst. de Radiologia; Rodrigues, Marcelo Bordalo [Universidade de Sao Paulo (IOT/HC/USP), SP (Brazil). Hospital das Clinicas. Instituto de Ortopedia e Traumatologia
Verrucous carcinoma is a variant of squamous cell carcinoma seen in mucous membranes and skin, and rarely found in the hand. The present report describes a case of two lesions on the dorsum of the hand, with no contact to each other, which underwent en-block resection and were confirmed as verrucous carcinoma. (author)
Suman S Karanth; Hariharan Regunath; Kiran Chawla; Mukhyaprana Prabhu
Burkholderia cepacia (B. cepacia) infection is rarely reported in an immunocompetent host. It is a well known occurence in patients with cystic fibrosis and chronic granulomatous disease where it increases both morbidity and mortality. It has also been included in the list of organisms causing nosocomial infections in an immunocompetent host, most of them transmitted from the immunocompromised patient in which this organism harbors. We report a rare case of isolation of B. cepacia from the bronchoalveolar lavage fluid of an immunocompetent agriculturist who presented with productive cough and fever associated with a pyopneumothorax. This is the first case of community acquired infection reported in an immunocompetent person in India.
Full Text Available Gestational trophoblastic neoplasias (GTN are rare tumours that constitute less than 1% of all gynaecological malignancies. Invasive mole is a distinct subgroup of GTN, which if not diagnosed and treated early, can result in serious complications like uterine perforation and haemoperitoneum. We present a rare case of torsion theca lutein cyst in association with invasive mole of the uterus, which developed following the evacuation of a molar pregnancy with features of continued irregular vaginal bleeding, persistently high betaHcg levels along with acute abdomen. [Int J Reprod Contracept Obstet Gynecol 2015; 4(4.000: 1237-1240
Suman S Karanth
Full Text Available Burkholderia cepacia (B. cepacia infection is rarely reported in an immunocompetent host. It is a well known occurence in patients with cystic fibrosis and chronic granulomatous disease where it increases both morbidity and mortality. It has also been included in the list of organisms causing nosocomial infections in an immunocompetent host, most of them transmitted from the immunocompromised patient in which this organism harbors. We report a rare case of isolation of B. cepacia from the bronchoalveolar lavage fluid of an immunocompetent agriculturist who presented with productive cough and fever associated with a pyopneumothorax. This is the first case of community acquired infection reported in an immunocompetent person in India.
Leena, J B; Fernandes, Hilda; Swethadri, G K
Malignant peripheral nerve sheath tumors(MPNST) are uncommon neoplasms with an incidence of 0.001% in general population. Multifocality is a rare manifestation of MPNST . A case of a 65 year old patient who presented with multiple swellings involving the neck, extremity and back without associated neurofibromatosis is reported for its rarity of presentation.. Diagnosis was made by FNAC and confirmed by peroperative findings and histopathology.
Karthick C Annamalai; Shrikiran A; Suneel C Mundkur; Chaitanya Varma PV
Naphthalene moth ball poisoning in children can present with diagnostic and therapeutic challenges. A 2 year old boy who had accidentally consumed unknown number of moth balls presented 3 d later with vomiting, seizures, methemoglobinemia, hemolytic anemia and altered sensorium. He was managed with red blood cell transfusion, IV Methylene blue and Sodium bicarbonate. Clinical and laboratory parameters normalized. We describe this case as ingestional naphthalene poisoning with rare manifestation of metabolic acidosis, with a good outcome after treatment.
George Peter; Hegde Narasimha
Leptospirosis is a zoonotic infection with higher incidence in tropics. Leptospirosis, is known for its variable manifestations, and is a clinical challenge for physicians in the tropics. Experienced clinicians, at times can mistake leptospirosis for non-medical conditions. A few reports of leptospirosis presenting as acalculous cholecystitis was found in review of literature. We intent to highlight acalculous cholecystitis as a rare but clinically significant presentation of leptospirosis.
Ayse Filiz Avsar
Full Text Available Fibroepithelial polyps (FEPs are rarely seen lesions of the lower female genital tract with polypoid proliferations of stroma. These tumors usually present in the vulvovaginal region of the reproductive aged women. In this presentation, we report a case of a psoriatic woman who developed unusual multiple polypoid lesions approximately 15 cm in size arising from both left and right labia minora and unique connection of FEPs with psoriasis disease.
Petramfar, Peyman; Hosseinzadeh, Farideh; Mohammadi, S. Saeed
Introduction Pseudo-Foster Kennedy syndrome is a triad consisting of ipsilateral optic atrophy, contralateral optic disc edema, and ipsilateral anosmia in the absence of an intracranial mass. Vitamin B12 plays an important role in DNA synthesis, and its deficiency causes peripheral neuropathy, myeloneuropathy, and, very rarely, optic neuropathy. Case Presentation In this study, we describe a 34-year-old male who presented with progressive loss of visual acuity and field. Fundoscopy showed opt...
Biyani, Sumant; Jha, Sneh Kumar; Pandey, Suchit; Shukla, Rakesh
We report a case of bilateral useless hand syndrome, a rare presenting manifestation of vitamin B12 deficiency. A 38-year-old man, a strict vegetarian and a teacher by occupation, presented with acute onset clumsiness of both hands while performing fine movements. Detailed history-taking, examination of the patient and relevant investigations (complete blood count, serum vitamin B12 and MRI of the cervical spinal cord) were carried out. Laboratory analysis was suggestive of vitamin B12 deficiency and MRI demonstrated a lesion involving the posterior columns of the cervical cord. The patient was diagnosed as a case of non-compressive cervical myelopathy predominantly involving the posterior column due to vitamin B12 deficiency. Acute bilateral useless hand syndrome can be a rare presenting feature of vitamin B12 deficiency.
Full Text Available Wegener′s granulomatosis (WG is a systemic necrotizing vasculitis that affects the small blood vessels. It mainly affects the upper and lower respiratory tract and kidneys. Central nervous system (CNS involvement is rare, and has been reported only in about 8% of cases during the course of illness. Initial presentation with neurologic affection, particularly chronic hypertrophic meningitis is very unusual. We report the case of a 34 year old male who presented with chronic hypertrophic meningitis and multiple cranial nerve involvement as the initial manifestation, without respiratory and renal symptoms. This case highlights the difficulties in diagnosing a rare disease with rarer presentation, and at the same time illustrates that Wegener′s granulomatosis should be considered in the differential diagnosis of chronic meningitis.
Full Text Available Carcinosarcomas of the ureter are rare biphasic neoplasms, composed of both malignant epithelial (carcinomatous and malignant mesenchymal (sarcomatous components. Carcinosarcomas of the urinary tract are exceedingly rare. We report a unique case of a carcinosarcoma of the ureter with a chondrosarcoma and small cell tumor component arising in a 68-year-old male who presented with microscopic hematuria. CT intravenous pyelogram revealed right-sided hydroureter and hydronephrosis with thickening and narrowing of the right ureter. The patient underwent robot-assisted ureterectomy with bladder cuff excision and subsequent adjuvant chemotherapy. The patient is disease-free at 32 months after treatment. We provide a brief synoptic review of carcinosarcoma of the ureter and bladder with utilization of immunohistochemical (IHC stains and potential diagnostic pitfalls.
Full Text Available Rare genetic or inherited forms of diabetes can mimic immune mediated type 1 diabetes. Early age of onset and associated features help to differentiate these diseases from type 1 diabetes. Wolfram syndrome, an inherited neuro degenerative disorder, presents as insulin dependent diabetes mellitus, diabetes insipidus, optic atrophy and deafness. But less well described features like psychiatric manifestations can be the presentation of this disease. We present such a case. Wolfram syndrome should be considered as a differential diagnosis in insulin dependent diabetic children who present with neuropsychiatric problems.
Full Text Available Isolated adrenocorticotropic hormone (ACTH deficiency is a rare cause of hypocortisolism, mostly associated with lymphocytic hypophisitis (LYH. Autoimmune miyelofibrosis is another rare autoimmune disease causing bone marrow fibrosis. Here, we report the case of a patient who presented with common symptoms (weakness, fatigue, weight loss, vague pain and anemia and was diagnosed with both rare autoimmune disorders (lymphocytic hypophisitis and autoimmune myelofibrosis. A 34-year-old male presented with weakness, fatigue, weight loss, and diffuse musculoskeletal pain. He had mild normochromic normocytic anemia. Further investigations revealed bone marrow fibrosis. The World Health Organization criteria were not fulfilled for the diagnosis of primary myelofibrosis. Since his symptoms could not be explained by mild anemia, a thorough evaluation was performed which revealed hypocortisolism associated with undetectable ACTH. Insulin-induced hypoglycemia test yielded insufficient response of ACTH and cortisol. Sellar MRI demonstrated typical features of LYH. Resolution of all the symptoms and anemia was achieved with low-dose glucocorticoid replacement therapy. In conclusion, when evaluating a patient presenting with fatigue, weight loss, vague pain, backache, and mild anemia, hypocortisolism also should be kept in mind in the differential diagnosis. If the case is isolated ACTH deficiency, the most probable cause is LYH. In such a case, additional endocrinological or non-endocrinological autoimmune disorders are likely to be present. We report the first case of lymphocytic hypophysitis coexisting with autoimmune myelofibrosis. Turk Jem 2014; 2: 47-51
Full Text Available Acute renal failure following vasculotoxic viperine snake bites is very common in South Asia. Acute tubular necrosis and acute cortical necrosis are the common findings, with acute interstitial nephritis (AIN being a rare presentation. We conducted renal biopsies in all patients who were admitted in our institute with viperine snake bite-related acute kidney injury (AKI and who did not improve after three weeks of supportive care. Patients who had findings of AIN on renal histology were included for this study. Of a total of 42 patients, there were five patients (11.9% with AIN. Our series of five patients is the largest series of this rare presentation in the literature. All of these five patients had features of severe envenomation, severe AKI network stage of AKI and very high antivenom requirements. They had a very prolonged stay in the hospital, and four of the five patients developed chronic kidney disease on follow-up. The overall outcome in this group was worse as compared with those who did not have AIN. AIN following viperine snake bites is not a very rare presentation. The reason for the development of this pathology is unclear, but direct venom-related effects are possible. This presentation portends a poor overall long-term prognosis as demonstrated in our case series.
Full Text Available : Sheehan’s Syndrome (SS also known as postpartum Hypopituitarism or postpartum pituitary necrosis is a condition in which hypopituitarism develops after severe bleeding during or immediate after childbirth. The incidence of Sheehan’s syndrome is 0.5% of all cases of Hypopituitarism. Reporting a rare case of SS who presented post Obstetric hysterectomy within 5 months with signs of agalactia, severe hypoglycemia, hyponatremia.
Uzuncakmak, Tugba Kevser; Karadag, Ayse Serap; Izol, Belcin; Akdeniz, Necmettin; Cobanoglu, Bengu; Taskin, Secil
Allergic contact dermatitis is a common skin disease, which affects approximately 20% of the population. This reaction may present with several clinical manifestations. Erythema multiforme-like allergic contact dermatitis is a rare type of non-eczematous contact dermatitis, which may lead to difficulty in diagnosis.Essential oil of Laurus nobilis is widely used in massage therapy for antiinflammatory and analgesic effects. Laurus nobilis induced contact dermatitis has been reported in the lit...
Full Text Available Rhino-orbital-cerebral zygomycosis afflicts primarily diabetics and immunocompromised individual, but can also occur in normal hosts rarely. We here presented an interesting case of facial nerve palsy and multiple cold abscesses of neck due to rhino-orbital-cerebral zygomycosis in an otherwise healthy man. Although some reports of facial nerve paralysis in conjunction with rhino-orbital-cerebral zygomycosis exist, no case of bilateral complete facial paralysis has been reported in the literature to date.
Full Text Available Sometimes anaesthesiologists come across rare congenital anomalies in their practice. The inherent complications associated with the disorder necessitate tailor-made approaches for providing anaesthesia to even seemingly simple surgical interventions. Here, we share our experience of anaesthesia management of an infant with congenital laryngomalacia and recently diagnosed osteogenesis imperfecta type 1 who had presented to us with an acute abdomen for a semi-emergency herniotomy.
Full Text Available Hemophagocytic lymphohistiocytosis (HLH is a rare hyper-inflammatory syndrome which is characterized by inappropriate proliferation of lympho-histiocytes which phagocytize hematopoietic cells and thereby give rise to the clinical picture of fever, hepatosplenomegaly and cytopenias. HLH can be primary or secondary due to infections by bacteria, viruses, parasites and fungi. Here we present a case report of simultaneous infection of Plasmodium vivax and visceral leishmaniasis complicated by secondary HLH in a Border Security Force jawan (BSF soldier.
Full Text Available Persistent gout may progress into a condition termed as chronic tophaceous gout, which involves the deposition of nodular mass of uric acid crystals called tophi in various soft tissue areas of the body. Generally, the anti-cyclic citrullinated peptide (anti-CCP antibody testing is considered as a highly specific confirmatory test for RA. We present here a rare case report of chronic tophaceous gouty arthritis with anti-CCP positivity.
Chaudhary, Nagendra; Mahato, Shyam Kumar; Khan, Salamat; Pathak, Santosh; Bhatia, B D
Neurocysticercosis (NCC) is one of the most common parasitic infestations (Taenia solium) of central nervous system (CNS) in children. Seizures are the common presenting symptoms. Hydrocephalus and optic atrophy are rare complications which may require neurosurgical interventions. We report a case of NCC with hydrocephalus and bilateral optic atrophy associated with vision loss in a Nepalese patient who improved with anti-parasitic therapy followed by ventriculo-peritoneal (VP) shunting.
Veasey, John Verrinder; Lellis, Rute Facchini; Boin, Maria Fernanda Feitosa de Camargo; Porto, Pedro Loureiro; Chen, Jessica Chia Sin
Syphilis is a sexually transmitted disease caused by Treponema pallidum and divided into three stages according to the duration of the disease: primary, secondary and tertiary. Secondary syphilis has diverse clinical presentations, such as papular-nodular lesions. This presentation is rare, with 15 cases reported in the literature over the past 20 years. We report a case of secondary syphilis with papular-nodular lesions in a healthy 63-year-old patient, who has presented treponema in immunohistochemical examination of the skin lesions. PMID:27192520
Krisht, Khaled M; Palmer, Cheryl A; Couldwell, William T
The authors describe a rare case of combined pituitary chronic lymphocytic leukemia (CLL) and prolactinoma in a 77-year-old man presenting with apoplexy. This case highlights the importance of evaluating the pituitary gland in patients with CLL who present with clinical manifestations of apoplexy as well as the need to carefully evaluate pathological specimens from the gland for the presence of lymphocytic cells in those patients. This is the first reported case of a combined CLL-prolactinoma pituitary lesion presenting with apoplexy.
Full Text Available The occurrence of malignancies in somatic elements of mature cystic teratoma of ovary is rare. The malignancies that may be encountered in dermoid cyst include squamous cell carcinoma, adenocarcinoma, adenosquamous carcinoma, melanoma, sarcoma, carcinoid, and germ cell neoplasms. The development of transitional cell carcinoma (TCC in dermoid cyst is extremely rare with only four such cases having been reported in literature so far. Here we report the fifth case of such an occurrence in a 50-year-old postmenopausal multiparous female patient. She presented with pain and gradual swelling of abdomen for 1 month. Abdominal computed tomography revealed a solid space occupying lesion with few cystic components at right pelvis, raising the possibility of an ovarian neoplasm. The level of CA-125 was slightly raised (56∙45 U/ml. Total abdominal hysterectomy and bilateral salpingo-oopherectomy was performed. Microscopic examination showed cyst wall lined by stratified squamous epithelium. Beneath the cyst wall, a tumor mass was present, histological features of which resembled that of high-grade TCC (stage pT1aNXMX. On immunohistochemical analysis, the tumor was found to be positive for CK7 and CK20 and negative for WT-1. These results were consistent with a diagnosis of TCC arising in urothelium of mature cystic teratoma. Reporting of such extremely rare cases is important for the assessment of prognostic factors and treatment protocols.
Mahesh K Goenka
Full Text Available Context Spontaneous rupture of the bile duct, although rare, has been described as a known surgical cause of jaundice in infancy after biliary atresia. Case report This article describes a four-year-old girl who presented with severe abdominal pain and features suggestive of acute pancreatitis, who developed gradual distension of the abdomen, and was found to have a ruptured bile duct, producing biliary peritonitis. She was managed with laparoscopic drainage of the peritoneal cavity. However, in view of the persistent biliary drainage, an ERCP was performed followed by stent placement for a bile duct leak. She was subsequently diagnosed as having a choledochal cyst. Conclusion A high index of suspicion, appropriate investigation, such as MRCP, combined with early drainage can help in reaching an early diagnosis, and reduced morbidity and mortality in this rare disorder.
Ahn, Seong-Ki; Hur, Dong Gu; Jeon, Sea-Yuong; Park, Jung Je; Kang, Hung-Soo; Park, Ki-Jong
Ipsilateral facial palsy (FP) of the peripheral-type can result from lesions involving the inferomedial tegmentum of the pons. However, cases of a medullary lesion with peripheral-type FP have rarely been reported. The authors experienced an 83-year-old man with a pontomedullary infarction who presented with ipsilateral peripheral-type FP. Brain diffusion MRI revealed a hyper-intense signal on the left dorsolateral portion of the upper medulla and pontomedullary junction. This case suggests that clinicians should take into account the possibility of a central lesion and brainstem infarction, even when patients present with peripheral-type FP.
We present the case of a 15-year-old girl who presented with polycythemia. CT abdomen revealed an enhancing mass in the upper pole of her left kidney with features suggestive of renal cell carcinoma. She underwent a laparoscopic radical nephrectomy. Histology demonstrated a well circumscribed, focally encapsulated, round blue cell tumour showing areas of microcalcifications and numerous psammoma bodies. Imunostaining showed diffuse positive staining for CD 57. This was consistent with a diagnosis of metanephric adenoma a rare benign epithelial renal tumour.
Full Text Available We here report a rare presentation of bilateral anterior dislocation of shoulder with associated fracture of greater tuberosity in a 38 year old male due to minor trauma which he sustained secondary to hyponatremia induced irritability. There was no associ ated rotator cuff tear which is often associated with BADS which makes this presentation unique. Unilateral dislocation of shoulder is a common condition which is frequently encountered in emergency trauma department. Anterior dislocation is more common th an posterior dislocation. However, simultaneous bilateral shoulder dislocations are usually posterior. Bilateral anterior dislocations with fractures of the greater tuberosity are even rarer and are usually associated with trauma or seizures
Manzoor A Bhat
Full Text Available Sheehan′s syndrome occurs as a result of ischemic pituitary necrosis secondary to severe postpartum bleeding. It is one of the most common causes of hypopituitarism, characterized by variable clinical presentation. Acute kidney injury occurs rarely in Sheehan′s syndrome and most of the cases have been found to be precipitated by rhabdomyolysis. We here present a case of Sheehan′s syndrome with acute kidney injury where theprecipitating cause was chronic hypocortisolemia. We believe this is the first reported case of Sheehan′s syndrome in which acute kidney injury was precipitated by adrenal insufficiency.
Full Text Available Nocardiosis is an opportunistic infection caused by the Gram-positive weakly acid-fast, filamentous aerobic Actinomycetes. The lungs are the primary site of infection mainly affecting immunocompromised patients. In rare circumstances even immunocompetent hosts may also develop infection. Diagnosis of pulmonary nocardiosis is usually delayed due to nonspecific clinical and radiological presentations which mimic fungal, tuberculous, or neoplastic processes. The present report describes a rare bronchoscopic presentation of an endobronchial nocardial mass in a 55-year-old immunocompetent woman without underlying lung disease. The patient exhibited signs and symptoms of unresolving community-acquired pneumonia with a computed tomography (CT scan that showed a space-occupying lesion and enlarged paratracheal lymph node. This patient represents the unusual presentation of pulmonary Nocardia beijingensis as an endobronchial mass. Pathology obtained during bronchoscopy demonstrated polymerase chain reaction (PCR confirmation of nocardiosis. Symptoms and clinical findings improved with antibiotic treatment. This patient emphasizes the challenge in making the diagnosis of pulmonary nocardiosis, especially in a low risk host. A literature review presents the difficulties and pitfalls in the clinical assessment of such an individual.
Full Text Available Nasopharyngeal carcinoma (NPC has a diverse incidence pattern but remains a rare tumour in the pediatric age group. Childhood nasopharyngeal carcinoma usually presents in the second decade of life. Paediatric NPC cases present a diagnostic challenge due to the high prevalence of common NPC presenting symptoms of nasal obstruction, serous otitis media and hearing problems among children. Furthermore, adenoid tissue is most often present, and a biopsy in an outpatient setup would be difficult. However, trismus on initial presentation is uncommon, and even in adults is a therapy related complication. As such, surgeons should have a high index of suspicion in NPC endemic areas, and a lower threshold for imaging in these cases as overall outcome appeared to be similar between adult and childhood NPC.
Full Text Available Hereditary Haemorrhagic Telangiectasia (Osler-Rendu-Weber disease is a rare autosomal dominant disorder commonly a ecting small vessels of skin and mucosa. It is usually misdiagnosed because of its atypical presentation. This disease frequently presents as epistaxis, GI bleed and visceral arterio venous malformations. Patient may present with stroke or migraine but presentation due to predominant respiratory symptoms may occur in the presence of pulmonary A-V stula. We present a 9 yr old male with dyspnoea on exertion and cyanosis and clubbing. He had a past history of intracerebral bleed with thrombocytopenia and he was being treated as ITP. Contrast echo revealed ndings suggestive of pulmonary arterio venous stula which led to subsequent investigations and retrospective evaluation and hence achieving the final diagnosis.
Full Text Available Background: Otalgia and Bell’s palsy are rare manifestations of metastasis and the most common presentation of an inflammatory process in the temporal bone.Case presentation: This article explains a 34-year-old woman with breast cancer who presented with cranial nerve palsy symptoms. The 7th and 8th cranial nerves were involved in the metastatic phase and then hoarseness was added to her symptoms. Brain MRI showed a petrous lesion in the temporal bone due to metastasis, which was the first clue to cancer. Her metastatic workup showed multiple bone lesions. On chest CT scan, multiple lung lesions were noted. Also, a breast mass was discovered on her chest CT scan. On breast examination an irregular mass fixed to the pectoralis muscle was found. Pathologic evaluation of samples obtained through ultrasound-guided core needle biopsy confirmed the diagnosis of invasive ductal carcinoma.Conclusion: Temporal bone metastases are rare and may be asymptomatic, or with mild symptoms mimicking mastoid infections. Physicians should consider metastatic cancer on the list of differential diagnoses in patients presenting with prolonged otologic symptoms or facial nerve disorders.
Birinchi Kumar Saikia
Full Text Available Dermatofibrosarcoma protuberans is a cutaneous soft tissue neoplasm with potential for local recurrence but distant metastasis is rare. Trunk and extremities are most commonly involved. This case presented as left-sided breast lump in a male patient. The patient underwent left-sided modified radical mastectomy. Tissues were subjected to histopathological and immunohistochemical test subsequently. The tumor cells showed storiform arrangement with nuclear pleomorphism and increased mitotic figures at places. They were reactive to CD34 and non-reactive to S-100, smooth muscle actin, desmin, cytokeratin and epithelial membrane antigen. The diagnosis of dermatofibrosarcoma protuberans with areas of fibrosarcomatous change was given. Though trunk is a common site for this tumor but its presentation as male breast lump has made the case unique.
Full Text Available Pituitary thyrotroph hyperplasia with hyperprolactinemia has been described as a rare presentation of primary hypothyroidism. Premenopausal females with this disorder can present with features of hypothyroidism, menstrual disturbances, galactorrhea, and visual field defects because of enlarged pituitary. Here we describe a 32-year-old female presenting to her gynecologist primarily with galactorrhea and secondary amenorrhea. She was found to have raised serum prolactin, and MRI brain showed enlarged pituitary. She was referred for pituitary surgery when she came to us. Clinical examination and biochemistry were suggestive of primary hypothyroidism. She was prescribed levothyroxine replacement. At 6 weeks follow-up, serum prolactin came down to normal, galactorrhea subsided, and spontaneous menstrual cycles resumed. In 12 weeks, pituitary enlargement completely regressed and in another month after that, she conceived. Hence, primary hypothyroidism can present with thyrotroph hyperplasia, where correct diagnosis and levothyroxine therapy can prevent unnecessary pituitary surgery. Hyperprolactinemia in this setting is of no clinical significance.
Uğraş, Nesrin; Tolunay, Şahsine; Öz Atalay, Fatma; Gökgöz, Şehsuvar
Phyllodes tumors are uncommon biphasic fibroepithelial neoplasms of breast, comprising less than 1% of all breast neoplasms. We therefore aimed to present the case with its microscopic findings. In this article, we report a 59-year-old female admitted to the general surgery department with a rapidly, enlarging, palpable mass in right breast. After histopathological examination, it was diagnosed as borderline phyllodes tumor with extensive squamous metaplasia. Metaplastic changes are infrequent in the stromal and epithelial component of these tumors. Extensive squamous metaplasia within phyllodes tumor is rare and may occur in benign, borderline and malign subtypes.
Patterson-Fortin, Jeffrey; Kohli, Anita; Suarez, Maria J; Miller, P Elliott
Ocular Lyme borreliosis is a rare manifestation of Lyme disease. We describe a case of an 80-year-old woman who presented with a 1-month history of unilateral painless central vision loss. Based on a temporal artery biopsy, she was initially diagnosed with giant cell arteritis and treated with a 3-day course of high-dose intravenous steroids. A more detailed history uncovered multiple previous treatments for Lyme disease and residence in an endemic Lyme area. The patient was subsequently diagnosed with ocular Lyme borreliosis and treated with intravenous antibiotics. After 5 weeks of treatment, unilateral vision loss did not progress and optic disc oedema resolved.
Kilincaslan, Huseyin; Gedik, Ahmet Hakan; Bilici, Mustafa; Cakir, Secil
Torsion of the vermiform appendix is a rarely observed acute abdominal emergency. The clinical manifestations are similar to acute appendicitis. It should be included in the differential diagnosis if a patient presents with intermittent abdominal pain and an acute abdominal emergency. Here, we describe a 4-year-old male patient with a mass located in the right lower abdominal quadrant, whose clinical findings were consistent with an acute abdomen. The gangrenous appendix was found in the pelvic cavity and its stump was twisted clockwise. A 1.5-cm fecalith was detected in the appendix lumen and appendectomy was performed. © 2013 The Authors. Pediatrics International © 2013 Japan Pediatric Society.
Mareddy, Aswani Srinivas; Rangaswamy, Dharshan; Vankalakunti, Mahesha; Attur, Ravindra Prabhu; Nagaraju, Shankar Prasad; Koti, Neeraja
Hepatitis B virus (HBV) infection presenting as crescentic glomerulonephritis in the absence of cryoglobulinemia is an extremely rare phenomenon. We report a case of a 44-year-old male with HBV infection, who underwent kidney biopsy for rapidly progressive renal failure and nephrotic range proteinuria. Histopathological evaluation of the kidney biopsy was consistent with immune complex mediated crescentic membranoproliferative glomerulonephritis (MPGN). The patient achieved complete renal and virological remission with steroids, plasmapheresis and antiviral therapy. This case report summarises the importance of early initiation of immunosuppression and plasmapheresis under antiviral coverage for improved clinical outcomes.
Aswani Srinivas Mareddy
Full Text Available Hepatitis B virus (HBV infection presenting as crescentic glomerulonephritis in the absence of cryoglobulinemia is an extremely rare phenomenon. We report a case of a 44-year-old male with HBV infection, who underwent kidney biopsy for rapidly progressive renal failure and nephrotic range proteinuria. Histopathological evaluation of the kidney biopsy was consistent with immune complex mediated crescentic membranoproliferative glomerulonephritis (MPGN. The patient achieved complete renal and virological remission with steroids, plasmapheresis and antiviral therapy. This case report summarises the importance of early initiation of immunosuppression and plasmapheresis under antiviral coverage for improved clinical outcomes.
Hussain, Shabbir; Solanki, Fanindra Singh; Sharma, Deepti B; Sharma, Dhananjay
We report herein a clinical case of a patient with femur fracture due to metastasis from penile squamous cell carcinoma. A young man, who was treated for carcinoma penis, presented with pathological fracture of femur and lung metastasis from metastatic carcinoma penis after 18 months. Long bone metastasis from penile cancer is extremely rare, to the best of our knowledge; this is the first report of a patient with penile cancer spread to the femur from primary squamous cell carcinoma of the penis.
Tamer Ahmed EL-Sobky
Full Text Available Primary hyperparathyroidism (PHPT is rare in children and adolescents, but has greater morbidity in this age group. Most of these patients show predominantly skeletal pathology and to a lesser extent renal involvement. Osteopenia, osteoporosis and subperiosteal resorption are frequently encountered radiographic skeletal signs. This study describes the orthopedic manifestations of PHPT in a child. PHPT in this child exhibited a late presentation with significant clinical morbidity and extensive radiographic manifestations. The characteristic radiographic pattern of PHPT in childhood is an important contributor to the diagnosis. The radioclinical and biochemical correlations augment diagnostic accuracy and delineate extent of skeletal pathology.
Subasinghe, Duminda; Keppetiyagama, Chathuranga Tisara; Sudasinghe, Hemantha; Perera, Niranthi; Skandarajah, Thurairajah; Sivaganesh, Sivasuriya
Pancreatic desmoplastic small round cell tumour (DSRCT) is an extremely rare malignancy of which very few reports exist. It follows an aggressive course and has a dismal prognosis. A twenty-four-year-old male presented with a one-month history of rapidly progressive obstructive jaundice associated with abdominal pain suggestive of a biliary colic. Contrast-enhanced CT (CECT) of the abdomen revealed a pancreatic head mass. He underwent a pancreaticoduodenectomy and adjuvant chemotherapy and is disease free one year after surgery. This is the first reported case of a pancreatic head DSRCT, discovered in a young male investigated for a short history of painful obstructive jaundice.
Jaffe, Stephen L; Glabus, Michael F; Kelley, Roger E; Minagar, Alireza
Cortical speech disorders rarely occur in multiple sclerosis (MS). We report a patient with relapsing-remitting MS, who presented with acute verbal dyspraxia. Magnetic resonance imaging (MRI) demonstrated an acute T2/Flair hyperintense, primarily white matter lesion underlying the middle third of the inferior frontal gyrus. The verbal dyspraxia cleared beginning 48 hours after the initiation of iv dexamethasone. Follow-up MRI demonstrated qualitative and quantitative diminution of the hyperintensity. This is the first report of a clinically definite MS patient with acute verbal dyspraxia. Moreover, there was a suggestive localization of verbal praxis to Brodmann areas 44/45.
Full Text Available Carbon monoxide poisoning during pregnancy is a rare and potentially serious condition. Fetal complications are uncommon, related to anoxic lesions. The severity of these complications does not depend on the level of maternal COHb. We report the case of a 22-year-old pregnant woman who at 30 weeks of gestation had carbon monoxide poisoning secondary to a fire in her home, complicated by cardiac arrest and severe fetal damage. The child had not brain damage, but presented bladder lesions not previously described, with urinary ascites complicating megacystis.
Nag, Hira-L; Kancherla, Ramprasad; Malpura, Aftab
Thorn prick injuries are generally conceded frivolous and rarely demand medical attention. Howbeit deep seated injuries are well described in the literature. We presented a case of thorn prick injury to the knee that manifested as Brodie's abscess of the medial distal femoral condyle and synovitis. Magnetic resonance imaging (MRI) and ultrasonography could only construe the affliction but not spot the thorn. Arthrotomy was undertaken for exploration and debridement. Empirical therapy with initial systemic Cefotaxime and subsequent Ofloxacin worked well after the surgery. Deep seated injuries by a thorn prick may take a protracted and torpid course. Thorough curettage of the bony lesion and debridement are vital for proper manage- ment.
Waad-Allah S. Mula-Abed
Full Text Available A 50-year-old Omani woman presented to the Outpatient Clinic, Royal Hospital, Oman with right upper abdominal pain and backache that had lasted 10 days. She had no palpitation, sweating, or hypertension (blood pressure 122/78mmHg. The patient’s history revealed that she had a similar incidence of abdominal pain two months prior, which was a "dull ache" in nature and somewhat associated with headache. The pain was relieved using a mild analgesic drug. Abdominal ultrasonography showed a right adrenal mass, and both computed tomography and magnetic resonance imaging of the adrenal glands confirmed a right adrenal mass consistent with adrenal pheochromocytoma. However, clinical biochemistry tests revealed normal levels of plasma catecholamines (dopamine, norepinephrine, and epinephrine and metanephrine, which are unusual findings in adrenal pheochromocytoma. Meanwhile, the patient had markedly raised plasma normetanephrine (10-fold which, together with the normal metanephrine, constitutes a metabolic profile that is compatible with extra-adrenal pheochromocytoma. The patient also had markedly raised chromogranin A (16-fold, consistent with the presence of a neuroendocrine tumor. Laparoscopic right adrenalectomy was done and the adrenal tumor was excised and retrieved in total. Histopathology and immunohistochemistry confirmed the diagnosis of adrenal pheochromocytoma; the tumor cells being positive for chromogranin, synaptophysin, and S-100 protein. Following surgery, the patient did well and showed full recovery at follow-up after three months. Molecular genetic testing showed no pathogenic mutation in pheochromocytoma genes: MAX, SDHA, SDHAF2, SDHB, SDHC, SDHD, VHL, and PRKAR1A. A review of the literature was conducted to identify the pathophysiology and any previous reports of such case. To our knowledge, this is the first report in Oman of the extremely rare entity of pheochromocytoma with an unusual clinical and biochemical scenario.
Full Text Available Cutis verticis gyrata is a rare skin condition characterized by swelling of scalp resembling the surface of the brain. Various conditions, like cerebriform intradermal nevus (CIN, may give rise to this clinical entity. Moreover, its association with cellular blue nevus is extremely rare and has not been reported so far. Here, we report a 28-year-old male with a huge cerebriform swelling covering the occipital lobe along with multiple nodules all over the body. Histology of the scalp swelling showed solitary or clusters of nevus cells in the dermis and from the body lesions showed features of cellular blue nevus. The diagnosis of CIN with cellular blue nevus was confirmed
Edwards, Paul C; Graham, Jasmine; Oling, Rebecca; Frantz, Kate E
The aim of this study was to determine whether a patient educator presentation (PEP) on pemphigus vulgaris would increase second-year dental students' awareness of the importance of learning about rare conditions and improve their retention of rare disease knowledge. The study involved students' subjective assessments of a PEP experience at two U.S. dental schools. In this mixed methods study, cross-sectional data were obtained by surveys and in-depth interviews. Questions focused on students' assessment of the messages acquired from the PEP and its likely impact on their future clinical care. At University 1, students completed paper surveys with open-ended questions and participated in a focus group. At University 2, students completed an online survey consisting of rating scale and open-ended questions. Responses to open-ended questions were categorized into themes. At University 1, 79 students (out of a possible 102; response rate 77.5%) completed the survey, and an additional ten students participated in a focus group. At University 2, 30 students (out of a possible 104; response rate 28.8%) completed the survey. At Universities 1 and 2, 88% and 100%, respectively, of respondents stated the PEP would influence their future clinical decision making. The vast majority of respondents (94% and 100% at University 1 and University 2, respectively) were of the opinion that the personal testimonial from a patient would help them recall information about pemphigus vulgaris in five years' time. Respondents from both universities commented that the PEP emphasized the importance of not dismissing a patient's concerns. These results suggest that a presentation by a patient with a rare condition can be an effective educational tool for preclinical dental students.
Srivastava, Madhur Kumar; Govindarajan, Krishna Kumar; Chakkalakkoombil, Sunitha Vellathussery; Halanaik, Dhanapathi
Renal masses account for 55% of cases presenting as palpable abdominal mass in children. An eight year male presented with palpable abdominal mass and pain. The patient underwent renal dynamic scan, which raised possibility of left duplex kidney with non-functioning moiety, as the size of left kidney was smaller than seen on Ultrasonography (USG). Magnetic resonance (MR)urography confirmed the findings with patient undergoing left hemi-nephrectomy and is doing well. In case of discrepancy in size of kidney on USG and renal scan, duplex kidney should be considered as differential, other causes being, renal cyst, benign/malignant mass and renal calculi. Gross hydro-ureter presenting as palpable abdominal mass is very rare with few reported cases..
Avascular necrosis (AVN) is a well-recognized complication of patients on high-dose steroids for a long time. Exogenous hypercortisolism is a well known cause of AVN and a number of cases have been reported. Cushing\\'s syndrome describes hypercortisolism of any cause endogenous or exogenous. A variety of traumatic and non-traumatic factors contribute to the aetiology of AVN although exogenous glucocorticoids administration and alcoholism are among the most common non-traumatic causes. AVN secondary to endogenous hypercortisolism is rare and very few case reports are available describing this complication. No literature is available on AVN presenting post-adrenal surgery. Here we present a young woman who presented with avascular necrosis of both hips 1 year after adrenalectomy for Cushing\\'s syndrome.
Ramnarayan, Bk; Maligi, Pm; Smitha, T; Patil, Us
Amalgam or its components may cause Type IV hypersensitivity reactions on the oral mucosa. These amalgam contact hypersensitivity lesions (ACHL) present as white striae and plaques, erythematous, erosive, atrophic, or ulcerative lesions. Postinflammatory pigmentation in such lesions and pigmentation due to amalgam incorporation in the soft tissue have been reported in the literature. However, ACHL presenting primarily as a black pigmented lesion is extremely rare if not reported. The clinician should be aware of one such presentation of ACHL; we report a unique case of ACHL in a 30-year-old female with such a pigmented lesion in close contact with amalgam restorations. The lesion regressed considerably in a year after replacement of the restoration with posterior composites.
Background Tumefactive demyelinating lesions are a rare manifestation of multiple sclerosis (MS). Differential diagnosis of such space occupying lesions may not be straightforward and sometimes necessitate brain biopsy. Impaired cognition is the second most common clinical manifestation of tumefactive MS; however complex cognitive syndromes are unusual. Case presentation We report the case of a 30 year old woman who presented with Gerstmann’s syndrome. MRI revealed a large heterogeneous contrast enhancing lesion in the left cerebral hemisphere. Intravenous corticosteroids did not stop disease progression. A tumour or cerebral lymphoma was suspected, however brain biopsy confirmed inflammatory demyelination. Following diagnosis of tumefactive MS treatment with natalizumab effectively suppressed disease activity. Conclusions The case highlights the need for clinicians, radiologists and surgeons to appreciate the heterogeneous presentation of tumefactive MS. Early brain biopsy facilitates rapid diagnosis and management. Treatment with natalizumab may be useful in cases of tumefactive demyelination where additional evidence supports a diagnosis of relapsing MS. PMID:24694183
Full Text Available Abstract Introduction Appendiceal duplication is a rare anomaly that can manifest as right lower quadrant pain. There are several variations described for this condition. We recommend aggressive operative management should this anatomical variation present in the presence of acute appendicitis. Case presentation We report the case of a 15-year-old African American girl who presented to our hospital with right lower quadrant pain and was subsequently found to have appendiceal duplication. Conclusion There are two categorical systems that have described and stratified appendiceal duplication. Both classification systems have been outlined and referenced in this case report. A computed tomography scan has been included to provide a visual aid to help identify true vermiform appendiceal duplication. The presence of this anatomical abnormality is not a reason for surgical intervention; however, should this be found in the setting of acute appendicitis, aggressive resection of both appendices is mandatory.