Two Liberal Fallacies in the Hate Crimes Debate

Possible Cold Autoinflammatory Syndrome

Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. Prevalence is unknown. The main symptom is predominantly bilateral lower limb edema. Edema may be moderate to severe with anasarca and includes pleural effusion, pericarditis or chylous ascites. Fatigue, abdominal pain, weight loss, inability to gain weight, moderate diarrhea or fat-soluble vitamin deficiencies due to malabsorption may also be present. In some patients, limb lymphedema is associated with PIL and is difficult to distinguish lymphedema from edema. Exsudative enteropathy is confirmed by the elevated 24-h stool α1-antitrypsin clearance. Etiology remains unknown. Very rare familial cases of PIL have been reported. Diagnosis is confirmed by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of intestinal biopsy specimens. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Differential diagnosis includes constrictive pericarditis, intestinal lymphoma, Whipple's disease, Crohn's disease, intestinal tuberculosis, sarcoidosis or systemic sclerosis. Several B-cell lymphomas confined to the gastrointestinal tract (stomach, jejunum, midgut, ileum) or with extra-intestinal localizations were reported in PIL patients. A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL medical management. The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with its ensuing lymph loss. Medium-chain triglycerides are absorbed directly into the portal venous circulation and avoid lacteal overloading. Other inconsistently effective treatments have been proposed for PIL patients, such as antiplasmin, octreotide or corticosteroids. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. The need for dietary control appears to be permanent, because clinical and biochemical findings reappear after low-fat diet withdrawal. PIL outcome may be severe even life-threatening when malignant complications or serous effusion(s) occur.

Porocarcinoma is a rare cancer of the developing sweat glands. It often occurs in older adults and frequently affects the head, neck, and extremities. We report a rare case of metastatic porocarcinoma with intriguing approach of the diagnosis and management due to limited resource setting. A 60-year-old man with a history of type 2 diabetes mellitus presented with nodules on the left foot with no palpable lymph node. A chest radiograph revealed multiple coin lesions and histopathological findings were suggestive for porocarcinoma. We assessed the case as a metastatic porocarcinoma in a patient with uncontrolled type 2 diabetes mellitus. Surgery was performed in the initial phase of treatment, consisting of below-knee amputation. The patient refused the planned chemotherapy in the referral hospital and then underwent other modalities of palliative care. He passed away eleven months after the initial diagnosis. Metastatic porocarcinoma is a rare oncological case with a challenging approach of the diagnosis and management. High awareness of clinical clues in rare cancer is needed for early diagnosis and prompt treatment, especially in limited resource settings.

Oral focal mucinosis (OFM) is a rare mucosal lesion of unknown etiopathogenesis. It is considered the oral counterpart of cutaneous focal mucinosis. From the anatomo-pathological point of view it is characterised by a focal degeneration of myxoid type of connective tissue. A literature survey revealed 50 reports of OFM cases worldwide. Here, we present an even more rare case with tongue involvement. Particular emphasis is placed on diagnostic-differential aspects of this kind of lesion, both from the clinical and the histopathological point of view, in respect to other manifestations of tongue mucosa.

10053 Background: Rare cancers (RC) are a challenge in terms of quality of care, access to health resources and clinical research. The Italian Rare Cancer Network (RTR: “Rete Tumori Rari”) is a clinical collaborative effort to improve quality of care in adult rare solid cancers in Italy. RTR enables institutions to share clinical cases and to rationalize access to distant reference centers minimizing patient migration. It indirectly promotes collaborative clinical research by encouraging accrual into clinical trials and supporting observational studies. Methods: RTR includes 150 oncology institutions across Italy. Clinical cases are shared asynchronously over a secure Web resource. Data, images and transactions are stored in an online clinical record. Patients are shared: 1. "logically”, when they are dealt with following common clinical practice guidelines; 2. "virtually”, when they are discussed over the network between two or more centers; 3: "physically", when they are referred to an excellence center for a specific treatment modality. Pathology review is arranged through transferal of paraffin-embedded specimens and upload of consultations. While it was chosen not to implement telepathology facilities, a teleradiology resource is now available. Results: From 2003 to 2011, more than 5,000 rare cancers cases (mostly sarcomas) have been uploaded. More than 1,300 teleconsultations have been delivered, while more than 1,000 patients moved across the network during their experience of disease. 700 cases were reviewed pathologically: amongst 365 cases originally diagnosed as soft tissues sarcomas up to 2010, treatment-relevant discordances were recorded in more than one third. An observational prospective study on gastrointestinal stromal tumors was done, enrolling 800 patients. An original paper documenting the activity of a drug in a highly specific sarcoma subgroup was published. Conclusions: Clinical asynchronous online collaboration on RC is feasible through a Web-based secure environment and proved the most practical way of clinical distant sharing. Pathologic review was a crucial network service, with a special added value in RC. Retrospective and prospective observational studies, and unplanned observations in very rare cases, were an interesting by-product.

Multifocal epithelial hyperplasia (MEH), also known as verrucae of the oral cavity, focal epithelial hyperplasia, Heck's disease, and multifocal papillomavirus epithelial hyperplasia, is a rare benign oral condition. It is most typically seen in the labial mucosa of younger people and rarely seen in middle-aged patients. Multiple small nodules or papules in the oral cavity, notably on the labial mucosa, buccal mucosa, and tongue, are clinically defined by this lesion. For precise diagnosis needs, clinical history, examination, and histopathologic findings. After months or years, spontaneous regression was commonly recorded; therefore, no treatment was required. Conservative surgical excision can be used for diagnostic and aesthetic reasons and recurrent trauma lesions. We present a very rare oral multifocal epithelial hyperplasia case involving the generalized attached gingiva of the upper and lower jaw in a fully edentulous patient in the Oral Specialized Clinic, Tehran, Iran.

Adult cystic teratoma of the neck: A rare case report and a review of literature

Supernumerary teeth or hyperdontia are defined as the teeth that exceeds the normal dental formula, regardless of their location and morphology. A supernumerary tooth is a developmental anomaly and it has been argued to arise from multiple aetiologies. Supernumerary teeth with orthodox shapes and sizes, that resemble the normal dentition are called 'supplemental teeth'. The supplemental teeth are often overlooked because of their normal shapes and sizes. Supplemental lateral incisors are rare and bilateral cases are even rarer, with only two cases being reported in the literature till date. Therefore, we are describing a rare case of non-syndromic, bilateral, supplemental, maxillary, lateral incisors and we have discussed the management of supernumerary teeth.

Interstitial lung disease in Indonesian adult with systemic sclerotic: A rare case

Cribriform morular thyroid carcinoma has been added under tumors of uncertain histogenesis. Its peculiar clinical, histomorphological pattern, and immunohistochemical profile have been proved different from papillary thyroid carcinoma. A 59-year-old female patient had a lesion in the left lobe of the thyroid. Fine needle aspiration cytology was reported as medullary thyroid carcinoma. The total thyroidectomy specimen showed a predominantly solid tumor of size 9.5 cm in the left lobe. Microscopy showed a mixed growth pattern with the dominant cribriform and solid morular area. Nuclear features of papillary carcinoma were not seen. Squamoid morules had nuclear clearing. Marked stromal hyalinization and calcification were noted. Extrathyroidal extension, lymphovascular invasion, and lymph node metastasis were not identified. Immunohistochemically the tumor cells were diffuse and strong nuclear positive for β-catenin, TTF1, PAX8, estrogen receptor, focal, and weak positivity for CD5. Synaptophysin, calcitonin, thyroglobulin, and CDX2 were negative. We report this rare cribriform morular thyroid carcinoma case with its associated uncommon histological and immunohistochemical features.

Pentalogy of Cantrell (PC) is a rare congenital syndrome comprising five characteristic abnormalities that include midline anterior abdominal wall defect, sternal defect, diaphragmatic defect, defect in apical pericardium, and structural heart defect. The exact etiology of this syndrome is still unknown. However, many causative factors and association with other syndromes/aneuploidies (trisomy 18/trisomy 13) have been described in literature. Prognosis is often poor in cases with complete ectopia cordis with large defect. The postnatal surgical correction demands expert care in good resource settings. We are describing a rare case of severe form of PC with complete ectopia cordis and omphalocele, detected at 32 weeks of gestation. The patient had vaginal delivery of live female baby but unfortunately, the baby succumbed on day 2 of life. We are reporting this rare and fatal case to make health-care professionals more aware about importance of timely detection of such anomalies by anomaly scan in the second trimester before the period of viability so that the parents can make informed choice about termination of pregnancy in such lethal cases. Furthermore, the corrective surgeries can be planned beforehand with multidisciplinary approach in cases where affordability is not an issue for parents.

Learning with Rare Cases and Small Disjuncts

Systemic lupus erythematosus (SLE) is an immune-mediated, lifelong disease characterized by quite heterogeneous neuropsychiatric manifestations. Herewith, we report the first rare co-incidental case with posterior reversible encephalopathy syndrome (PRES), Guillain Barre Syndrome (GBS), and (SLE). The coexistence of these neurological conditions in SLE patients could lead to delayed diagnosis and treatment due to this rare coalescence and clinical diversity. Currently, there are no specific, diagnostic radiological or laboratory biomarkers for neurological involvement in SLE. Awareness and, early recognition of neuropsychiatric involvements of the disease are important for timely appropriate treatment. Delayed treatment may cause permanent damage, poor prognosis, long term morbidity, and even death.

Odontomas are the most common types of Odontogenic tumors.Their prevalence exceeds that of all other Odontogenic tumors combined.Odontomas represent a hamartomatous malformation; the term refers to tumors of Odontogenic origin [1], [2] .They are usually asymptomatic and are diagnosed on routine radiological examination as an incidental finding [1], [3] .Odontomas are further subdivided into compound and complex types depending on the extent of morpho differentiation or on the resemblance to normal teeth [4] .The compound odontome is composed of multiple small tooth like structures, the complex odontome consist of a conglomerate mass of enamel and dentine, which bears no anatomical resemblance to the tooth [3] .The eruption and infection of odontoma are uncommon, only few cases of erupted odontome are reported in the literature.We report a rare unusual case of an infected complex odontoma with concomitant eruption of odontoma in the oral cavity involving perforation of the mucosa with an impacted third molar which makes the presentation unusual.We also highlighted the significance of proper diagnosis as most of these rare tumors go unnoticed with incorrect treatment to the patient.The dentist should be familiar with common and unusual appearances of odontomas for proper diagnosis and management.

Duplicated appendix is a rare congenital anomaly with incidence of 0.004-0.009% and its mostly an incidental finding on table, when one of them is acutely inflamed and very rarely both of them can be inflamed as in this case. Report a case of young girl who presented with complain of pain abdomen and vomiting in the last 2 days. Clinically patient was diagnosed to have acute appendicitis and on laparoscopy, patient had duplicated appendix one at the ileocaecal valve and the other 2cm away near the caecum with pus and faecolith. Histopathology confirmed appendicitis in the both appendices. Reported about this interesting rare case because even though the incidence of duplicated appendix is too low (0.004-0.009%), should always search for the missed appendix in patients who underwent appendicectomy earlier and complains of severe pain in right iliac fossa. Aim was to report such a rare interesting case and give a small gentle reminder to the surgeons as duplicated appendix even though rare but still a possibility.