Orbital malignant peripheral nerve sheath tumor: A case report and review.

Malignant peripheral nerve sheath tumor of the orbit is an exceedingly rare entity. These tumors exhibit locally aggressive behavior, recurrences, distant metastasis, and poor response to existing treatment protocols. Orbital nerve sheath tumors are often associated with neurofibromatosis 1, and malignant transformation of neurofibroma into malignant nerve sheath tumor has also been seen. The recommended treatment for localized disease is radical or wide surgical excision to achieve negative margins followed by chemoradiation. For extensive disease, chemotherapy and radiotherapy can be utilized to stabilize the disease. Due to poor response and outcomes with current regimens, the focus has been shifted to approaches utilizing molecular targets and immunological agents. Despite all the advancements, the outcomes still remain discouraging for moderate- to high-grade lesions and thus necessitate studies to design promising treatment modalities.

An Unusual Presentation of Angiolymphoid Hyperplasia with Eosinophilia as Postauricular Mass: A case Report.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare, benign, reactive vaso-proliferative condition in the dermal and subcutaneous tissues of the head and neck. A 28-year-old female presented with slow-growing painless swelling behind her left ear. FNAC revealed benign soft tissue neoplasm and histopathological examination after surgical excision revealed angiolymphoid hyperplasia with eosinophilia. ALHE origin has been variously attributed to prior trauma, hyperestrogenemia, infectious agents, atopy, reactive hyperplasia, and benign neoplasia. Retroauricular ALHE has been rarely reported. However, on the basis of our case report, it should be a viable differential diagnosis when large subcutaneous tumors of the head and neck are encountered. When big subcutaneous tumors of the head and neck are present, especially in females, a valid differential diagnosis for angiolymphoid hyperplasia with eosinophilia, a rare condition marked by dermal or subcutaneous endothelial cell proliferation, should also be considered.

Recurrent solitary fibrous tumor of eyelid: A rare entity.

Orbital and adnexal solitary fibrous tumors (SFT) are rare entities. The clinico-radiological and histologic features overlap with those of other spindle cell variants, and hence the use of immunohistochemical stains helps in making an accurate diagnosis. Furthermore, a thorough surgical resection is imperative to prevent tumor recurrences. We report a rare case of SFT arising primarily from the eyelid with multiple recurrences.

Magnitude of Neurogenic Tumor Burden in Pediatric Population: A Tertiary Care Center Study.

OBJECTIVE: Progress in the diagnosis and treatment of childhood neoplasm in the past few decades is one of the most gratifying achievements in the field of oncology. This study was aimed to ascertain the burden (incidence and prevalence) and histopathologic features of neurogenic tumors occurring in the pediatric population. MATERIALS AND METHODS: The study evaluated a total of 492 cases of pediatric tumors over a period of 8 years from 2007 to 2015, including patients of 0-12 years of age group; attending the out-patients and in-patients Department of Pediatrics and Pediatric Surgery at Jawaharlal Nehru Medical College, AMU, Aligarh, with the complaints of tumor or tumor associated sign and symptoms. Clinical profile, hematological and histopathological examination along with immunohistochemical analysis were implicated to attain a conclusive diagnosis. RESULT: Out of 492 pediatric tumor cases, 255 (52%) cases were benign and 237 (48%) cases were malignant. Neurogenic tumor (brain tumors) comprised 49 (10%) of the total case, being most common malignant solid neoplasm and second most common (next to leukemia) overall malignancy, constituting 49/237 (21%) cases. Astrocytoma 22/49 (45%) cases were the most common type brain tumor followed by medulloblastoma 15/49 (31%), ependymoma 9/49 (18%), and craniopharyngioma 3/49 (6%). CONCLUSION: Effective management of pediatric neurogenic tumor is a multipronged approach involving effort of good Pediatric neurosurgeon, Pathologist and a host of Oncology specialists with insight into childhood neoplasms.

Necrotizing periodontitis in a heavy smoker and tobacco chewer - A case report.

Necrotizing periodontitis is a distinct and specific disease characterized by rapidly progressing ulceration of the interdental gingiva and then spreading along the gingival margins and leading to acute destruction of periodontal tissues. Necrotizing ulcerative gingival lesions are common in developing countries because of poor nutritional status, poor oral hygiene and debilitating conditions. In the developed world it is mostly seen in patients with the HIV infections and other immune system dysfunctions. The exact etiology of the necrotizing lesions is still unknown; however a fuso-spirochaetal infection along with weakened host immune system seems to play a major role in the pathogenesis of these diseases. Presented is the case of acute necrotizing periodontitis in a 21 year old male patient with no systemic disease but a history of tobacco use (chewing and smoking) since 7 years. The patient was managed by conservative treatment followed by surgery for the correction of gingival defects.

Isolated cystic echinococcosis of kidney burlesquing as renal cell carcinoma: a diagnostic pitfall.

Echinococcus granulosus infection affects worldwide population, including countries of South and Central America, Middle East, sub-Saharan region, Russia and China. In cystic echinococcosis diagnosis is related to the presence of single or multiple well-delineated spherical primary cyst, commonly seen involving liver followed by lungs. Renal hydatid cyst is rare, comprising of about 2-3% of all locations, isolated renal involvement is even rarer. We present a case of hydatid disease in a 45 year male patient who presented with the history of vague flank pain, mild fever and hematuria. IVU showed a filling defect while USG and CT scan displayed multiloculated cyst in the upper pole of right kidney, correlating the clinical and radiological findings a differential diagnosis of cystic renal cell carcinoma and cystic nephroma was derived. Total nephrectomy was done but the histopathological examination of the lesion revealed hydatid cysts and final diagnosis of renal cystic echinococcosis was made.

Cystic glioblastoma multiforme masquerading as a cerebral tuberculoma.

Glioblastoma multiforme (GBM) is by far the most common and most aggressive malignant neoplasm of the primary brain tumours. It arises from the astrocytes and classified as WHO grade 4 astrocytoma. Diagnosis of GBM is sometimes difficult as radiological picture sometimes mimic with cerebral tuberculoma. In both the cases contrast-enhanced CT may show similar finding of a mass lesion with a hypodense centre surrounded by a ring of enhancement and any cyst if present. In the present case, a 45-year-old male patient presented with seizures and headache, a provisional diagnosis of tuberculoma was made on the basis of clinical and CT findings. However, on grounds of suspicion the patient was operated and fluid from the cyst was sent peroperatively for cytopathological examination which suggested the diagnosis of cystic GBM. This helped the surgeon to do maximum debulking of the tumour. Diagnosis was further confirmed by histopathology.

Suspected hydatid cyst of liver harbouring an aggressive desmoplastic small round cell tumour.

Among the group of small round cell tumours, there is a distinct and rare tumour known as desmoplastic small round cell tumour (DSRCT). DSRCT presents as multiple, widespread masses in the abdomen and pelvis and may be accompanied by extensive tumour implants throughout the peritoneum as the tumour is known to spread diffusely along serosal surfaces. We discuss a case of DSRCT in a 16-year-old boy who presented with abdominal pain since 2 years, a non-tender mass was palpable on the right upper quadrant of the abdomen, ultrasonographic and CT findings suggested hydatid cyst of liver. Laparotomy revealed multiple small peritoneal deposits along with a single mass in the liver. On histopathology, the lesion was found to be neoplastic and composed of predominantly clusters of small round blue cells, in a desmoplastic stroma; tumour cells were diffusely positive for cytokeratin, vimentin and neuron-specific enolase, thus confirming the diagnosis of DSRCT.

Cytological diagnosis of chondroblastoma: diagnostic challenge for the cytopathologist.

Chondroblastoma is an uncommon osseous neoplasm that accounts for less than 1% of all bone tumours. It characteristically arises in the epiphysis or epimetaphyseal region of long bones and has been reported to affect people of all ages with slight male predilection. WHO has defined chondroblastoma as 'a benign, cartilage-producing neoplasm usually arising in the epiphyses of skeletally immature patients'. The authors document the cytological features on fine-needle aspiration cytology of a chondroblastoma which appeared as a lytic lesion in the upper end of the right fibula, an uncommon site, in an 18-year-old male patient. X-ray feature combined with fine-needle aspiration cytology favoured the diagnosis of chondroblastoma, which was further confirmed by histopathological examination.

Eosinophilic granuloma of the mandible: a diagnostic dilemma.

Eosinophilic granuloma (EG) is a rare histiocytic disorder resulting from clonal proliferation of Langerhans cells. It accounts for less than 1% of all osseous neoplasms and has a predilection for involving the axial skeleton. Although suspicion of the disease may arise from clinical features and radiographic demonstration of destructive bone lesions, it is still difficult to make a correct diagnosis without proper pathological evaluation. This is more evident when common differentials mimicking EG, both clinically and radiologically, need to be ruled out. This report describes a case of unifocal EG of the mandible occurring in a 4-year-old boy whose initial presentation led to confusion between osteomyelitis, primary bone tumour and lymphoma. A final diagnosis of EG was established after histopathological examination of the biopsy specimen.

Low Grade Appendiceal Mucinous Neoplasm with Pseudomyxoma Peritonei: An Enigma for Pathologist.

Malignant mucinous neoplasms of the appendix is an infrequently encountered entity. Extra-appendiceal spread of these tumor is one of the commonest etiology of pseudomyxoma peritonei, which demands a hightened vigilance in their early diagnosis. Although low-grade appendiceal mucinous neoplasms (LAMNs) largely stay confined to the appendix, but they can spread to the peritoneum as pseudomyxoma peritonei leading to an unpredictable outcome. Due to the rare occurrence of low-grade appendiceal neoplasm only tenuous and limited information is present in the medical literature. We report a case of LAMN with pseudomyxoma peritonei in a 45-year-old male, who presented with the complaints of abdominal distension associated with abdominal pain and constipation. Clinical examinations and computed tomography (CT) scan were suggestive of pseudomyxoma peritonei. Peroperative findings and histopathological examination rendered a conclusive diagnosis of low-grade appendiceal neoplasm. How to cite this article: Qadri S, Alam K, Alam F, Maheshwari V. Low Grade Appendiceal Muci-nous Neoplasm with Pseudomyxoma Peritonei: An Enigma for Pathologist. Euroasian J Hepato-Gastroenterol 2014;4(2):113-116.