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Introduction: Solitary fibrous tumor (SFT) is a rare neoplasm of mesenchymal origin that occurs most often in the visceral pleura. It rarely occurs in the central nervous system (CNS). If they occur intracranially, they are extra-axially located and develop from the meninges., Case Report: Herein, we present a case of SFT in a 45 years old female. MR imaging showed isointense to hypointense signal intensity, and marked enhancement. Microscopically, the tumors showed characteristic "patternless-pattern" architecture. Elongated tumor cells formed fascicles alternating with hypocellular densely collagenous stroma. Immunohistochemistry for STAT6, CD34, BCL2 and CD99 favours the definitive diagnosis of SFT., Conclusion: In general, complete surgical resection may offer the best chance of a favourable clinical outcome. Their biological features remain largely unknown. Hence, the clinical management and prognosis is often challenging due to the lack of comprehensive data., (Copyright © 2018. Published by Elsevier Ltd.)

Introduction: Leukemia is the most common malignancy of childhood but myeloid sarcoma is a rare presentation of underlying leukemic disorder. Myeloid sarcoma (MS) is a rare tumor composed of proliferation of myeloid precursors at extramedullary sites., Presentation of Case: We report an unusual case of myeloid sarcoma involving the temporal bone in a young male child who presented with a large mass involving the left temporal region. This lesion was the initial presentation which led to further diagnosis of acute myeloid leukemia in our case. This case report brings awareness to the diverse extramedullary manifestations of isolated myeloid sarcoma, as well as the importance and difficulties that are associated with establishing a rapid diagnosis and initiating treatment., Discussion: They can arise de novo or in association with hematological malignancies, most commonly acute myeloid leukemia (AML-M2). Clinically, it can masquerade as an abscess, cutaneous ulcer, or as a mass lesion. Morphologically, MS can mimic a variety of small round cell tumors including lymphomas, neuroblatoma and rhabdomyosarcoma. The occurrence of this tumor usually heralds AML or the onset of the blastic phase of chronic myeloid leukemia. Early recognition of this rare entity is important, because early aggressive chemotherapy and focal irradiation can cause regression of the tumor and thus improve patient longevity., Conclusion: The possibility of MS should be considered when dealing with unusual lymphoma like neoplasms that cannot be categorized as any of the Non-Hodgkin lymphoma subtypes and small blue round cell tumors., (Copyright © 2019. Published by Elsevier Ltd.)

Introduction: Adenoid cystic carcinoma of the breast (breast-ACC) is a rare tumor with a favorable prognosis, despite its triple-negative status and special type of basal-like tumor for which scant population-based descriptive data exist. We sought to provide new population-based information on breast-ACC in India. Due to the paucity of the number of cases, the natural history of the disease is not fully understood. This study was undertaken to examine the clinico-pathological characteristics of the disease and to evaluate the outcome of surgical intervention in a tertiary referral care centre., Materials and Methods: A retrospective analysis of all patients diagnosed and treated for ACC Breast in our hospital over the past 10 years was carried out (2005-2015). A database of the characteristics of these patients was developed. In all, 14 patients were identified. The investigations performed included routine blood investigations, chest X-ray, bone scan and either an ultrasound or a CT scan., Results: During the time period of 10 years, of 2347 with breast malignancy admitted to our department, only 14 were diagnosed as having ACC (3.15%). All patients were women (100%). The patients had a median age of 60.7 years (range 37-81). The most common symptom was lump in the breast. Two patients (14.2%) presented with nipple and skin retraction and two patients (14.2%) were asymptomatic with the diagnosis made by an incidental finding on routine examination. The CT and/or magnetic resonance imaging (MRI) showed the typical features of carcinoma breast. All the 14 patients were taken up for surgery. Nine patients underwent Modified radical mastectomy and five patients underwent Breast conservation surgery. Axillary lymph node dissection was carried out in seven patients and sentinel lymph node biopsy in the remaining. Tumor cells had a characteristic histologic pattern of ACC of the breast. Perineural invasion was present in six cases., Discussion: ACC of the breast is a very rare malignancy, accounting for less than 0.1% of all breast neoplasms. It affects the left and right breasts equally and tumors arise irrespective of the breast quadrants. However, in about 50 percent of patients, lesions are found in subareolar region. Pain or tenderness described in the minority of cases has not been correlated with histologically-confirmed perineural invasion. ACC is categorized as a basal-like subtype of breast carcinoma. Most cases are macroscopically well-circumscribed. Occasionally, pink, tan, or gray microcysts are evident. A tumor typically consists of a dual-cell population of luminal and myoepithelial-basal cells which may be arranged in one or more of three architectural patterns: tubular-trabecular, cribriform, and solid-basaloid. There is no consensus on the optimal management for patients with ACC of the breast. Based on its indolent clinical course and favorable outcome, ACC of the breast is generally cured by breast-conserving surgery, such as wide excision or quadrantectomy with or without radiotherapy., Conclusion: Breast-ACC among women is characterized by ER-negative/PR-negative expression, rare regional lymph node involvement, a favorable prognosis with excellent survival, and absence of associated cancers. These findings reinforce the importance of tailored treatments for breast-ACC and the apparent heterogeneity of basal-like breast cancers., (Copyright © 2018. Published by Elsevier Ltd.)

Introduction: Multiple myeloma (MM) is characterized by malignant proliferation of plasma cells, monoclonal bone marrow plasmacytosis, the presence of M-protein in serum and/or in urine and osteolytic bone lesions., Presentation of Case: We report a case of a 28-years old female, who was diagnosed to have relapsing extra-skeletal and extra-nodal plasmablastic myeloma, an atypical variant of MM with a poor prognosis. In addition to bone marrow plasmacytosis and the presence of M protein in the serum, the patient had an extramedullary lesion affecting the hard palate., Discussion: There is a strong correlation with age. The peak incidence is seen in 6th-7th decade. The clinical course in adolescents and young individuals is generally indolent and the survival is longer. Extramedullary myelomas are rare tumors accounting for 0.4% of all head and neck malignancies., Conclusion: A case of extraskeletal plasmablastic myeloma presenting as a hard palate growth and that too in young female patient is an extremely rare and requires a multidisciplinary approach for management.

Transverse testicular ectopia is an extremely rare anomaly, in which both the testis migrate towards the same hemiscrotum through the same inguinal canal. It is usually associated with other abnormalities such as persistent Mullerian duct syndrome, true hermaphroditism, inguinal hernia, pseudohermaphroditism, and scrotal anomalies; the association with inguinal hernia being the commonest. We, here, report a case of transverse testicular ectopia in a 12 years old boy having the ectopic testis in contralateral hemiscrotum without any inguinal hernia but having scrotal hypospadias with severe chordee. The diagnosis was made on clinical examination and confirmed on ultrasonography. Trans-septal orchiopexy was done for ectopic testis and hypospadias and chordee were managed by staged repair., (Copyright © 2017. Published by Elsevier Ltd.)

Introduction: Trichobezoars are concretions formed by accumulation of hair in stomach. Usually, trichobezoar is confined to the stomach, but rarely it may extend from the stomach to the small intestine and even colon. This is an unusual form called Rapunzel syndrome. Our experience with this rare entity of Rapunzel syndrome and interesting entity of trichobezoar is being presented with review of literature., Presentation of Cases: We, at our institute, encountered four cases of trichobezoar in last five years, out of which two were found to be of Rapunzel syndrome. All of these cases were managed successfully by open surgical intervention in view of the very large size of the mass in all the cases., Discussion: The clinical presentation is highly variable ranging from asymptomatic cases diagnosed incidently to serious gastrointestinal symptoms and complications. Cases of trichobezoar have been reported in literature very infrequently but Rapunzel syndrome is extremely rare and less than 50 cases have been reported in medical literature till date., Conclusion: Trichobezoar leading to Rapunzel syndrome is an extremely rare entity. The clinical presentation is usually vague and non-specific. Treatment is mainly surgical because of delayed presentation in majority of the cases. Psychiatric illness is the usual association., (Copyright © 2017. Published by Elsevier Ltd.)

Introduction: Melanoma is a malignant tumor that can affect any area of the anatomical economy. Its occurance in the female urethra is extremely rare. We report a case of primary malignant urethral melanoma developed in an elderly female patient., Presentation of Case: A 70 years old female presented with dysuria, poor stream, gross haematuria, intermittent blood spots, and a painful mass. On physical examination, there were no suspicious lesions on the skin. On external genital examination, a lesion at the level of the urethral meatus was observed. The mass was removed by wide local excision under spinal anaesthesia. The pathological diagnosis was malignant melanoma of the urethra., Discussion: The common presentations include bleeding and/or discharge per urethra, voiding dysfunction and the presence of tumor mass. Survival depends on the stage, location and size of the neoplasm at the time of diagnosis. Despite major surgery, radiotherapy or immunotherapy; malignant melanoma usually has a poor prognosis., Conclusion: Melanoma of the female urethra is an extremely uncommon pathology leading to paucity of literature and any definite recommendations regarding management. The histological and immunohistochemical findings can be helpful in making an early and accurate diagnosis of malignant melanoma in the urogenital region., (Copyright © 2017. Published by Elsevier Ltd.)

Introduction: Solid pseudopapillary tumor (SPT) of the pancreas is rare, accounting for 0.13-2.7% of all pancreatic tumors. It is unique, has low malignant potential and predominantly affects young women. Radiological and pathological studies have revealed that the tumor is quite different from other pancreatic tumors. But the cell origin of SPT and tumorigenesis are still enigmatic. Abdominal mass is the most common presenting symptom. Due to the paucity of the number of cases, the natural history of the disease is not fully understood. This study was undertaken to examine the clinico-pathological characteristics of the disease and to evaluate the outcome of surgical intervention in a tertiary referral care centre., Materials and Methods: A retrospective analysis of all patients diagnosed and treated for SPN in our hospital over a period of 10 years (2005-2015) was carried out. A database of the characteristics of these patients was developed. In all, 11 patients were identified. A CT scan of the abdomen was performed in all the patients and the findings revealed a mass in the pancreas. The investigations performed included routine blood investigations, chest X-ray, CA-19-9 level and either an ultrasound or a CT Scan of the abdomen., Results: During the time period of 10 years, of 349 patients with pancreatic malignancy admitted to our department, only 11 were diagnosed as having SPN (3.15%). Ten patients were women (90%) and one patient was a man (10%). The patients had a median age of 27.6 years (range 17-41). The most common symptoms were abdominal pain and dullness. Eight patients (72.7%) presented with abdominal pain or abdominal dullness and three patient (27%) were asymptomatic. All the 11 patients were taken up for surgery. Three patients underwent distal pancreatectomy with splenectomy, three patients underwent the total mass excision and one patient underwent total pancreatic resection. Three required extended distal pancreatectomy with splenectomy. One underwent spleen-preserving distal pancreatectomy., Conclusion: SPT is rare, but treatable pancreatic tumor. While clinical signs and symptoms are relatively nonspecific, characteristic findings on imaging and histology separate these tumors from the more malignant pancreatic tumors. The prognosis is favorable even in the presence of distant metastasis. Although surgical resection is generally curative, a close follow-up is advised in order to diagnose a local recurrence or distant metastasis., (Copyright © 2017. Published by Elsevier Ltd.)

Introduction: Cutaneous metastasis from primary visceral malignancy is a relatively uncommon clinical entity, with a reported incidence of 0.22%-10% among various series. However, the presence of cutaneous metastasis as the first sign of a clinically silent visceral cancer is exceedingly rare., Presentation of Case: We describe here a case of an asymptomatic male patient who presented with a solitary scalp metastasis as the initial manifestation of an underlying lung cancer. Diagnostic evaluation revealed advanced disease., Discussion: The report emphasizes that physicians should be aware of this rare clinical entity, and appropriate investigation should be arranged for early diagnosis and initiation of the appropriate treatment. The occurrence of skin lesions in lung cancer announces an ominous prognosis., Conclusion: We conclude that the possibility of metastatic skin disease should always be considered in the differential diagnosis in patients with a history of smoking or lung cancer presenting with cutaneous nodules., (Copyright © 2017. Published by Elsevier Ltd.)

Introduction: Congenital diaphragmatic hernia (CDH) is a defect in diaphragm which usually presents with severe respiratory distress in neonatal period., Presentation of Case: We present a case of congenital diaphragmatic hernia presenting at an age of 2.5 years in a male child. It was misdiagnosed as a case of pyothorax for which chest tube was attempted on left side resulting in iatrogenic gastric perforation. The patient was managed by early and prompt surgery., Discussion: Late presentation is usually rare with vast array of respiratory and gastrointestinal symptoms. It often leads to clinical and radiological misdiagnosis., Conclusion: Surgical intervention in misdiagnosed cases can lead to catastrophic iatrogenic complications., (Copyright © 2017. Published by Elsevier Ltd.)