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Abstract Background Tenosynovial giant cell tumor is a rare soft tissue tumor of the synovium of joint, bursae, or tendon sheath. It is divided into localized or diffuse types on the basis of the growth pattern. Localized tenosynovial giant cell tumors are usually benign and treated successfully by excision. Diffuse tenosynovial giant cell tumors, in contrast to localized type, can destroy bone and cartilage and are associated with frequent local recurrences and distant metastasis. Localized type tenosynovial giant cell tumors rarely metastasize to distant organs. Here, we report a case of localized tenosynovial giant cell tumor presenting with lung metastases and systematically review literature. Case presentation A 55-year-old Asian male presented with a dry cough, right-sided chest pain and progressive dyspnea for 1 month. At 18 months before this presentation, he had undergone excision of a painless swelling on his right index finger. The swelling recurred within 3 months of excision, and a biopsy was then suggestive of a giant cell tumor. Given the suspicion of a giant cell tumor, a wide excision of the lesion was performed and the excisional biopsy was consistent with a diagnosis of tenosynovial giant cell tumor, localized type. At admission to our hospital, the patient had tachypnoea and absent breath sounds on the right side. A chest radiograph showed a right-sided pleural effusion with a homogenous opacity in the left mid-zone. A contrast-enhanced computed tomography of the chest and abdomen showed right massive pleural effusion and bilateral multiple lobulated heterogeneously enhancing pleural-based masses with areas of internal calcification. Pleural fluid analysis revealed an exudate with no malignant cells on cytology. A lung biopsy showed osteoclast-like giant cells and mononuclear spindle cells with areas of hemorrhage and necrosis, suggesting tenosynovial giant cell tumor metastasis. A final diagnosis of localized type tenosynovial giant cell tumor of the right index finger with metastases to the lungs and pleura was made. The patient passed away after receiving three cycles of denosumab injection owing to progressive disease. Conclusion Lung metastasis is extremely rare in patients with localized tenosynovial giant cell tumor. The survival is usually poor in patients with lung metastasis. A close follow-up of patients with localized type tenosynovial giant cell tumor is necessary for early detection of pleuropulmonary complications.

Sclerosing stromal tumor is a benign sex cord-stromal tumor, that commonly occurs in the second and third decades of age. Intraoperative diagnosis of this entity poses a great challenge because of the rare occurrence and can mimic malignant lesions. A 15-year-old female presented with a right ovarian mass. Serum markers were within normal limits. The radiological evaluation showed a large heterogeneously enhancing solid cystic abdominopelvic mass of size 16 × 14 × 9 cm with non-visualization of both ovaries separately and a few areas of calcification with mild ascites. An open cystectomy was performed. A part of the cyst wall was sent for an intraoperative frozen section. It was reported as sclerosing stromal tumor, and the post-operative specimen also confirmed the same. Areas of calcification and ossification were also identified as additional findings. We reported this case because of the uncommon occurrence, highlighting additional histological features, and also did a literature review, especially focussing on the intra-operative diagnosis.

Leiomyosarcomas (LMS) arise from smooth muscle cells with more predilection to the uterus, abdomen, retroperitoneum, and blood vessels. LMS of vagina is very rare and usually presents in the early stage as an asymptomatic mobile mass with no clinical features of malignancy and gives the appearance of a benign lesion which can easily be mistaken for a Bartholin’s cyst or a vaginal fibroid. The chances of metastasis in LMS are high with poor survival rates. Histopathology confirms the diagnosis and treatment still remains controversial due to less data on this rare malignancy. Even though there is no evidence that leiomyoma can transform into LMS, benign-looking vaginal fibroids need to be resected to avoid misdiagnosis of LMS. We present a case of vaginal LMS which was mistaken to be Bartholin’s cyst due to the lack of knowledge of this aggressive tumor and further management.

Aim The aim of this study was to evaluate the usefulness of high b-value diffusion-weighted imaging (DWI) to differentiate benign and malignant lung lesions in 3 Tesla magnetic resonance imaging (MRI).

Abstract Background The complications associated with abdominal testis include torsion, rupture, and malignant transformation. Case presentation A 40-year-old man presented with complaints of left-sided abdominal mass and abdominal pain. On contrast-enhanced computed tomography (CECT), there was a well-defined heterogeneously enhancing mass lesion in the lower abdomen with calcification showing fistulous communication to the adjacent ileal loops. The lesion was seen supplied by the left gonadal artery raising suspicion for testicular origin. On performing a scrotal ultrasound, the left testis was found absent. A radiological diagnosis of abdominal testicular neoplasm was made. Conclusions Histopathological examination proved the lesion to be a germ cell tumor, consistent with seminoma.

Abstract Background Hydatid disease is an infectious disease that affects several organs. Isolated renal involvement is very rare. The treatment for renal hydatid cyst ranges from minimally invasive percutaneous aspiration techniques to laparoscopic and open techniques. We describe five cases of isolated renal hydatidosis with varied presentations who were treated successfully by various methods. Case presentation The presenting symptoms included flank pain (n = 5), mass abdomen (n = 2), and hydaturia (n = 1). In 4 patients, the diagnosis of a hydatid cyst was known preoperatively, but one patient with a preoperative diagnosis of a simple cyst was found to harbor hydatidosis intra-operatively. Eosinophilia as a marker for the active disease was present in 60% (3/5), while echinococcal serology was positive in only 25% (1/4). Two cases were approached laparoscopically, while three required an open approach. Two patients were treated with nephrectomy due to the high bulk of the disease, while the other three underwent renal preserving cyst excision. Conclusions The presence of eosinophilia in the preoperative workup may indicate an infective/active hydatid disease. Echinococcal serology is representative of past hydatid infection but cannot reveal about current disease status. Cysts with varied attenuations and residence in an endemic region may support a renal hydatid cyst diagnosis. A holistic approach including clinical history, laboratory parameters, and imaging is needed for diagnosis. Surgical treatment requires cyst excision, along with precautions to prevent spilling. Nephrectomy may be preferred in cases with minimal residual function.

Mature cystic teratoma of the ovary (MCT) is rare in pre and postmenopausal age patients. Among various types of malignant transformation in MCT, adenocarcinoma is a rare subtype. Dual type tumors arising from ovarian MCT have been described in the literature very rarely. A 47-year-old postmenopausal female patient presented with abdominal mass for ten years. The radiological opinion was a dermoid cyst. Grossly, a 22 × 20 × 10 cm, unilocular cystic left ovarian mass with intact capsular surface and focal thickened wall measured 3.0 cm. Microscopically, it showed components of all three germ cell layers. In addition, features of colonic type adenocarcinoma and well-differentiated neuroendocrine tumor (carcinoid) were noted and confirmed by immunohistochemistry (IHC). We report this rare case of synchronous malignancy arising from an ovarian MCT with a clinicopathological review.

A leukemic phase of anaplastic lymphoma kinase positive anaplastic large cell lymphoma (ALK+ ALCL) is rare. The leukemic cells morphologically appear as small to intermediate-sized cells with cerebriform and cloverleaf-like nuclei and are misdiagnosed as other T-Cell lymphomas/leukemia with similar morphology. We describe a case where the diagnosis of leukemic ALK+ ALCL was aided by immunohistochemistry performed on the cell blocks prepared from the peripheral blood buffy coat specimen. The diagnosis of ALK+ ALCL was further confirmed on the biopsy of a cutaneous nodule of this patient. We found the method of immunohistochemistry on peripheral blood buffy coat cell block very useful and suggest that it may be used as an alternative method to flowcytometry in low resource settings.

Astroblastomas are uncommon tumors of uncertain histogenesis. Recently tanycyte is postulated to be the cell of origin. These occur predominantly in the cerebral hemisphere of young adults and children. Astroblastoma constitutes approximately 0.45 to 2.8% of all neuroglial tumors. We are reporting a case of 28-year-old woman who presented with headache and double vision. Magnetic resonance imaging (MRI) of the brain revealed well-demarcated, ring-enhancing solid cystic mass in right temporoparietal lobe with significant mass effect. The patient underwent gross total resection of the lesion through right temporoparietal craniotomy. The histopathologic diagnosis was suggestive of astroblastoma. She was advised for postoperative radiotherapy, but she lost to follow-up and again presented 1 year later with recurrent tumor in the same location along with another lesion in infratentorial location. She was reoperated and gross total excision of supra- and infratentorial tumor was done. She also received radiotherapy. We are reporting this uncommon case of astroblastoma and also reviewing the existing literature.

Phyllodes tumors are uncommon fibroepithelial neoplasms of breast. Heterologous sarcomatous differentiation of malignant phyllodes tumor (MPT) is a rare phenomenon as shown in the literature. Herein we report a series of nine cases from a tertiary care centre in Eastern India. Patients demographic data and clinical details were obtained from the medical records. Histopathology and immunohistochemical studies were analysed and diagnosed accordingly. Out of 38 cases of PT, 13 were found to be MPT, of which 9 cases showed heterologous differentiation in the form of angiosarcoma, fibrosarcoma, undifferentiated sarcoma, extensive squamous differentiation and with lipomatous metaplasia. Proper diagnosis and subtyping of the sarcomatous component is essential for deciding the correct treatment modality and prognostication of the disease. However there is no clear cut treatment protocol is available because of paucity of data.

Abstract Gastrointestinal stromal tumors (GISTs), the commonest mesenchymal tumors of gastrointestinal tract are often described to take origin from the interstitial cells of Cajal (ICC) or its precursor cells. Rarely these tumors do arise in structures other than the alimentary tract like omentum, mesentery, retroperitoneum, etc., of varying malignant potential and are known as extra-gastrointestinal stromal tumors (eGISTs). This is a case report of a 70-year-old female with multicentric malignant eGISTs arising in the mesentery of ileum. On laparotomy, a large mass of 20 × 15 cm was found in the small bowel mesentery without involvement of the adjacent ileum, with multiple other small nodules resembling lymph nodes, present adjacent to it. Histopathological study of the excised lump, confirmed the mass to be malignant eGIST without involvement of the adjacent ileum, with cluster differentiation (CD)117 positive and of high-risk stratification. The mesenteric nodule was confirmed on histopathology to be malignant eGIST, similar to that of that of the primary, without any lymphoid tissue. Adjuvant imatinib mesylate treatment was started immediately postoperation with the patient doing well at 1 year of follow-up. We report this case, due to the rare occurrence of multifocal malignant eGISTS of small bowel mesentery, which is yet to be reported. The existing literature is unclear regarding the clinicopathology and management of multifocal malignant stromal tumors of the mesentery.

Abstract Giant schwannoma of the conus medullaris is a rare form of spinal neoplasm. A 22-year-old woman presented with sciatica. Magnetic resonance imaging of the spine showed a mass lesion in the conus medullaris region with nerve compression. Subtotal resection was done and histologic analysis confirmed the diagnosis as Schwannoma. The patient improved in symptoms after surgery. This case report of a very rare cystic tumor is presented with review of the literature.

Lipoleiomyoma of the uterus is a rare variant of leiomyoma, and lipoleiomyoma of the broad ligament is still rarer, with only a handful of cases being reported. The present case was a perimenopausal woman who presented with a huge lower abdominal mass. Ultrasonography and computed tomography showed a heterogeneous solid mass in the left adnexa. The histopathological findings confirmed the nature of the lesions as a benign lipoleiomyoma with dermoid cyst of the left ovary and its other associated benign lesions, were the interesting features seen in this case which were not suspected clinically and radiologically.

Filariasis is a global social health problem of tropical and sub tropical countries like India. W.bancrofti accounts for 95% of cases of lymphatic filariasis. Microfilaria in cytosmears are a rare finding. We report a case of 55 year old female presented with right axillary swelling with ipsilateral breast lump. Cytosmears from the lymph node aspirate showed metastatic adenocarcinomatous deposits and a bunch of microfilariae surrounding the tumor cells and the aspirate from the breast shows ductal carcinoma. We report an additional case of a rare association of microfilaria co-existing with carcinomatous deposit in the lymph node.

Introduction: Primary extranodal lymphoma (pENL) refers to group of disorders arising from tissues other than lymph nodes and even from sites, which normally do not contain lymphoid tissue. This study was undertaken to ascertain the prevalence, anatomical distribution, and histological subtypes of extranodal non-Hodgkin lymphoma (NHL) from a tertiary care institute in Southern India. Materials and Methods: This was a cross-sectional study conducted in the Department of Pathology over a period of 3-years. Detailed clinical history, routine complete blood count, microbiological status was obtained from the medical records. Hamatoxylin and eosin slides were reviewed and immunohistochemistry was performed using a panel of antibodies depending on the morphology. All cases were classified based upon morphologic and immunophenotypic criteria according to World Health Organization 2008 classification. Results: Primary extranodal NHL constituting 22.6% (68/300) of all NHL and the majority of patients were from higher age group with peak incidence seen in fourth to fifth decade of life. In the pediatric population, the most common site is ileo-caecal region (42.8%) and the most common morphology is lymphoblastic lymphoma (42.8%). Similarly in the adult population head and neck was the most common site constituting 36% and diffuse large B-cell lymphoma is the most common morphological subtype comprising 54% of all extranodal lymphomas. Conclusion: Although the reported incidence of pENL is low in India compared to other parts of the world, the possibility of pENL should always be kept in mind even though it arises in an extranodal site.

Handful cases of invasive Cystic Hypersecretory Ductal Carcinoma (CHC) have been reported so far in literature. Cystic hypersecretory lesions of breast have a spectrum of morphological features ranging from Cystic Hypersecretory Hyperplasia (CHH), CHH with atypia, in situ to invasive CHC. We are reporting a case of a 32-year-old female who had nipple discharge and lump in her right breast for one month. A modified radical mastectomy was done and morphological diagnosis of invasive CHC with axillary node metastasis was made. The postoperative events were uneventful. Invasive CHC being a rare entity, our understanding of its biological behavior, prognostic factors and genetic basis is limited. The authors are aware of only 15 similar cases being reported in the English literature.

Gelatinous marrow transformation (GMT) or serous atrophy of bone marrow (BM) is a rare disease characterised by focal marrow hypoplasia, fat atrophy, and accumulation of extracellular mucopolysaccharides abundant in hyaluronic acid. This study reviews 11 cases of GMT from South India. Clinical and haematological parameters, BM aspirate, and biopsies of all patients diagnosed with GMT over a period of 7 years were studied. GMT was diagnosed in BM biopsy based on characteristic morphological appearance and was confirmed by alcian blue positive staining pattern at pH levels of 2.5 and 0.5. Eleven patients were diagnosed with GMT. All were males within the age range of 15 to 50 years. The underlying clinical diagnosis was human immunodeficiency virus positivity in 5 cases, 2 with coexistent disseminated tuberculosis, 1 with cryptococcal meningitis, and 1 with oral candidiasis; disseminated tuberculosis in 1 case; pyrexia of unknown origin in 2 cases; Hodgkin's lymphoma in 1 case; acute lymphoblastic lymphoma with maintenance chemotherapy in 1 case; and alcoholic pancreatitis in 1 case. BM aspirates showed gelatinous metachromatic seromucinous material in 3 cases. BM biopsies were hypocellular in 7 and normocellular in 4 cases and showed focal GMT in 5 and diffuse GMT in 6 cases. Reactive changes were seen in 4 cases and haemophagocytosis in addition to GMT in 1 case. GMT is a relatively uncommon condition and an indicator of severe illness. It should be differentiated from myelonecrosis, amyloidosis, and marrow oedema. A high index of suspicion is required to diagnose this condition.

Serous psammocarcinoma is a rare variant of serous carcinoma arising from either ovary or peritoneum, characterized by massive psammoma body formation, low grade cytologic features, and invasiveness. Its clinical behavior is similar to serous borderline tumors with relatively favorable prognosis. We report herein a case of a 60-year-old postmenopausal woman who presented with abdominal distension. Contrast enhanced computed tomography (CECT) revealed calcified pelvic masses with ascites. Elevated serum CA-125 (970 U/mL) suggested malignant ovarian neoplasm. Patient underwent exploratory laparotomy with primary debulking surgery. Histopathology showed bilateral serous psammocarcinoma of ovary with invasive implants on omentum. Adjuvant chemotherapy was advised in view of advanced stage disease, although its benefits are poorly defined due to rarity of the tumor. However, patient opted out of it and is now on follow-up.

Ancient schwannoma is an uncommon variant of schwannoma, a benign tumor arising from the nerve sheath. It is reported to arise from any nerves except optic and olfactory. However, only six cases of ancient schwannomas arising from ansa cervicalis nerve have been reported to date in English literature. Proper preoperative evaluation is necessary to rule out other causes of neck mass such as thyroid lesions, lymphadenopathy, and carotid body tumor. We report a case of ancient schwannoma arising from the ansa cervicalis nerve. The origin of the lesion from ansa cervicalis was confirmed by intraoperative finding. Postoperative histopathological examination revealed degenerative changes including pleomorphism, cellular atypia, large nuclei with prominent nucleoli, and paucity of mitotic figures. Periphery of the mass showed nuclear palisading with characteristic verocay bodies. Immunohistochemical evaluation for S-100 showed diffuse positivity of the tumor cells, thereby confirming the diagnosis of schwannoma. We consider that schwannoma of cervical region can have origin from any nerve and should try to identify the origin pre- and intraoperatively. The postoperative complications depend on the nerve of origin and the precision of the surgery performed.

Silicosis is an occupational lung disease caused by inhalation of crystalline silica. People working in occupations like sandblasting, surface drilling, tunneling, silica flour milling, ceramic making, and so forth are predisposed to develop silicosis. Crystalline forms of silica are more fibrogenic than the amorphous forms, highlighting the importance of the physical form in pathogenesis. Lung biopsy is rarely performed for the diagnosis of silicosis as it can easily be detected by occupational history and radiological features. Patients with silicosis can develop complications like tuberculosis, lung cancer, progressive massive fibrosis, cor pulmonale, broncholithiasis, or tracheobronchial compression by lymph nodes. Pleural involvement in silicosis is rare. Spontaneous pneumothorax is a pleural complication that can develop in such patients. Usually in silicosis pneumothorax is unilateral. We hereby report the lung biopsy findings and discuss the mechanism of pneumothorax development in a case of chronic silicosis who, later on died during the course of the disease.