Aggressive angiomyxoma presenting as a vulval polyp

Aggressive angiomyxoma is a rare, locally invasive mesenchymal tumor, occurring predominantly in the pelvic-perineal region of adults and carries a high risk for local relapse and hence the need to differentiate it from the other mesenchymal tumors occurring in this region. Presentation as a pedunculated polyp, like in our case, is unusual for this rare tumor. Except for positive surgical margins, there are no clinical or histological means for predicting the tumor recurrence. A diligent long-term follow-up is mandatory. Though rare, this tumor needs to be considered in the differential diagnosis of vulval polyps.     

Atypical teratoid rhabdoid tumor located in the pineal region following prophylactic irradiation for acute lymphoblastic leukemia

Atypical teratoid rhabdoid tumor (AT/RT) is a rare entity. In the central nervous system, AT/RT generally arises from the posterior fossa of infants and behaves aggressively. AT/RT is reported to arise from the infratentorial region (63%) and other sites, such as the suprasellar region, cerebellopontine angle, and spinal cord. The pineal region is rare (6%) as a site of origin. Radiation-induced brain tumors are well known. In this report, we present a case of a pineal region tumor causing acute hydrocephalus that could be pathologically diagnosed as AT/RT following prophylactic cranial irradiation for acute lymphoblastic leukemia.     

Atypical angiofibroma of larynx — a case report

Primary extra nasopharyngeal angiofibroma of larynx is a very rare tumour. We here by present a case of angiofibroma of larynx affecting the anterior commissure & subglottic region in larynx-a rare site of involvement.     

Rare sellar region tumors

We present three cases of rare intracranial midline tumor in the sellar region, often mimicking pituitary adenomas clinically. We describe their symptoms, radiological and pathomorphological features. The first case is a pituitary adenoma producing growth hormone with ganglion cell differentiation. In addition, a rare intracranial granular cell tumor of sellar region and germinoma of pituitary fossa are also presented. All tumors were resected and histologically analyzed. Their biological behaviour was favorable with a 10-year follow-up demonstrating no recurrent tumor mass.     

An encephalocele replacing nose completely

Congenital encephalocele presenting in the nasal region is extremely rare. We present here a case ot tncephaloce lecompletely replacing the nose of a five year old boy and duscuss about similar swellings in this region.     

Schwannoma of the conus medullaris: a rare case

Intradural schwannoma of the conus medullaris is a rare form of spinal neoplasm, which commonly occurs in the lumbar region. Conus medullaris level is unusual for schwannomas. A 49-year-old woman presented with chronic sciatica, mild bladder dysfunction, and paresthesia in the buttocks. Magnetic resonance imaging of the spine showed a mass lesion in the conus medullaris region with nerve compression. The tumor was completely resected and diagnosed histologically as schwannoma. The patient recovered after surgery. Clinical and radiologic features of this rare tumor are reviewed and are accompanied by literature findings.     

Plexiform fibrohistiocytic tumor in the submandibular region: A rare diagnosis

Plexiform fibrohistiocytic tumor (PFHT) is a rare mesenchymal neoplasm of intermediate malignancy, first reported by Enzinger and Zhang in 1988 [1]. It mainly affects children and young adults and preferentially involves the upper extremity [1, 2]. We report a rare case in the submandibular region which was diagnosed on histopathology.     

A case of myxofibrosarcoma of the maxilla with difficulty in preoperative diagnosis

Myxofibrosarcoma (MFS) is a very rare fibroblast-derived sarcoma that occurs in the head and neck region. Here, we report the case of a 52-year-old man in whom MFS generated from the maxilla and whose beginning of treatment was considerably delayed because he was initially diagnosed as having a benign inflammatory lesion. Because a definite diagnosis was not obtained via 2 independent biopsies, total maxillectomy was used for both diagnosis and treatment. Histopathological and immunohistochemical analyses suggested that the tumor was a low-grade MFS. Because soft tissue tumors in the head and neck region are rare and a definite diagnosis is relatively difficult, surgical excision is indispensable if malignancy of the tumor is suspected.     

De novo osteogenic sarcoma of mastoid bone

The most common primary malignant tumor of the bone is osteosarcoma. Primary involvement of the craniofacial bones in osteosarcoma is relatively rare. The mandible and the maxillae are the most commonly affected bones of the head. Here, we report a rare case of de novo high-grade osteogenic sarcoma of the mastoid region of the temporal bone and discuss thediagnostic and therapeutic properties.     

Salivary gland cyst in extra-parotid region

A rare case of a simple salivary serous cyst arising from the extra-parotid region is described, with a brief review of the literature and histogenesis.     

Retained foreign body in sino-orbital region

It is rare to find foreign bodies in nose and paranasal sinuses. The further rarity is to encounter impacted foreign body in this region and route of entry being through the orbit. We are here by presenting a case, where a metallic foreign body was retained for duration of 4 months in sino — orbital region.     

Pineal parenchymal tumor of intermediate differentiation with papillary features: a continuum of primary pineal tumors?

Pineal parenchymal tumors comprise a rare group of primary neoplasms of the pineal gland. We describe a case involving a 29-year-old woman who presented with signs and symptoms of hydrocephalus secondary to a pineal region tumor obstructing the third ventricle. Surgical resection was performed and pathological analysis revealed a novel diagnosis consistent with a pineal parenchymal tumor of intermediate differentiation (PPTID) with transition to a papillary tumor of the pineal region (PTPR). To our knowledge, this particular pineal region tumor pathology has not yet been reported in the literature and highlights the continuum with which primary pineal tumors exist. We provide a review of the existing literature on pineal region tumors, specifically PTPR and PPTID, and offer insight into the management of these rare neoplasms.     

Mesenchymal chondrosarcoma of the paraspinal region: a case report.

Mesenchymal chondrosarcoma is a very uncommon tumor. An extremely rare presentation of the tumor i.e. arising from the paraspinal region is being presented.     

Paratesticular desmoplastic small round cell tumor: case report and review of the literature.

Desmoplastic small round cell tumor (DSRCT) is a rare neoplasm mainly affecting young males and typically located in the abdomen. Prognosis is generally very poor. We report a rare case of paratesticular DSRCT in a 17-year-old boy, presenting with an isolated left scrotal mass. The patient had an excellent outcome after complete surgical resection of the tumor and adjuvant multi-agent chemotherapy. DSRCT should be included in the differential diagnosis of small round cell tumors of the paratesticular region in adolescents and young adults. Tumor resection and chemotherapy may be beneficial for these patients. Our experience and a review of the literature suggest that DSRCT located in the paratesticular region may have a better prognosis than its more frequent abdominal counterpart.     

Paraganglioma of the retropharyngeal region

A rare case of paraganglioma of the retropharyngeal region is reported.     

Schwannoma of submandibular region

Tumours of the nerve sheath origin in the head and neck are not common. Schwannomas are rare. Only a few cases have been reported so far. We report a rare case of Schwannoma of Submandibular region arising from the lingual nerve.     

Ewing sarcoma of the thoracic wall in a 54-year-old man

Ewing tumor is the second most common bone tumor in children. Its variant, malignant small cell tumor of the thoracopulmonary region, is infrequent in children and extremely rare in adults. A multimodal treatment approach is preferred for this tumor. We describe a rare case of primitive neuroectodermal tumor/Ewing sarcoma arising from the thoracic wall in a 54-year-old man. He underwent extensive resection of the tumor followed by adjuvant chemotherapy and radiotherapy.     

Peripheral primitive neuroectodermal tumor of head-neck region: our experience

To present four rare cases of peripheral primitive neuroectodermal tumors of different sites of head and neck region. Four cases of different age (range 8–40 years) and sex (three female, one male) with rare primitive neuroectodermal tumor of sinonasal region and neck are presented. Treatment options, biological behavior and prognostic outcome are discussed herewith. Two patients succumbed to the disease within four to six months of treatment; other two patients are still under follow-up depicting the aggressive nature of the tumor. Primitive neuroectodermal tumor belongs to the class of malignant round cell tumor. Immunohistochemistry plays a pivotal role in differentiating this tumor entity. Chemoradiation was tried, but local and systemic spread occurs early and holds poor prognosis. This case series is an attempt to describe the aggressive behavior of this rare tumor with high mortality.     

Nasal hemangiopericytoma

Haemangiopericytoma is rare tumour of vascular tissue. It’s pathological behaviour is variable. It arises from pericytes of Zimmermann. Although one fourth of cases are reported from head and neck region, nasal haemangiopericytoma is very rare. Because of rarity of condition, one case of nasal haemangiopericytoma arising from nasal septum is reported.     

Schwannome mammaire bénin : à propos de deux cas

Schwannoma is a rare nervous tumor developed on schwan cells. It occurs usually in the extremities, the trunk and the head region. Its occurrence in the breast is rare with only a few cases being reported. It usually appears as a breast lump having clinical and radiological characteristics suggestive of benign lesions. Its diagnosis is histological. Its treatment is surgical. Through two observations and a literature review we would try to remind the characteristics of this tumor and its management.