Solitary fibrous tumour of the face: a rare case report.

Solitary fibrous tumour is a rare mesenchymal neoplasm that most commonly involves the pleura. The diagnosis of solitary fibrous tumour is primarily histological. It consists of histological and positive immunohistochemical findings of CD-34 and vimentin. Recently, solitary fibrous tumour has been reported to occur in extrapleural soft tissues, such as the orbit, nasal cavity, abdominal cavity, parotid gland, scalp and neck. In an extensive review of the literature, we found no reports of solitary fibrous tumour arising in the facial soft tissue, other than in the parotid gland. This rare location of an uncommon lesion can lead to a confusing diagnosis. We report a case of solitary fibrous tumour originating in the temporal region of the face, and call for awareness of this tumour among plastic surgeons.     

A rare submucosal tumour of stomach-glomus tumour: A case report

IntroductionGlomus tumour (GT) of the stomach is a rare submucosal mesenchymal tumour. Gastric glomus tumours are clinically recognized as benign. Nevertheless, some show biological behaviour similar to that of malignant lesions and presurgical confirmation is often impossible.Presentation of caseA 32 year old female who presented with epigastric pain and was subsequently investigated for a antral tumour of the stomach and Wedge resection of tumour was done. Immunohistochemistry demonstrated strong positivity of smooth muscle actin and vimentin with low rate of mitosis studied by ki-67.DiscussionWe discuss the preoperative investigation, the diagnostic problems and the surgical treatment of the patient with gastric glomus tumour.ConclusionGlomus tumours should be considered as differential diagnosis for submucosal tumours of stomach.     

Elastofibroma dorsi: case series of a rare benign tumour of the back

We analyse this rare pseudotumour with few case reports in the orthopaedic literature. Elastofibroma dorsi is an uncommon benign soft tissue pseudotumour usually located at the lower pole of the scapula. This study is a clinical assessment of 5 patients. All the patients had their diagnosis based on clinical and MRI findings and had excision biopsy. The patients were followed for a mean period of 24 months. No patient had residual symptoms. We have only one recurrence. Surgical excision is recommended when it causes functional disability, compression symptoms, an asymmetric outline of the chest wall, or when it is more than 5 cm in diameter. Pre-operative tissue diagnosis is not necessary in most cases. Despite its rarity, the elastofibroma dorsi should always be suspected with the emergence of a swelling in the subscapularis, especially in females and in old age.     

Parosteal osteosarcoma of the distal ulna. A rare tumour at a rare location: a case report

We report a case of a histologically well differentiated, grade I, parosteal osteosarcoma of the distal ulna. The tumour's radiological and histological features are described, and the patient's course after wide en-bloc resection is detailed. Parosteal osteosarcoma is a rare low-grade malignant neoplasm. It belongs to the group of the osteosarcomas originating from the surface of the bone, representing a distinct tumour entity within this group, with defined histological and radiological features. We describe the radiological and histological characteristics of parosteal osteosarcoma and, based upon these features, present its classification. The differential diagnosis, including parosteal osteoma, myositis ossificans and osteochondroma, is also discussed. Finally, therapy and prognosis are outlined in accordance with the current literature. Wide en-bloc resection represents the mainstay of therapy offering an excellent prognosis with a 5-year survival rate of more than 90%. Incomplete resection, on the other hand, increases the risk of recurrence and dedifferentiation of the tumour. Dedifferentiation induces tumour spread and is associated with a poor prognosis equivalent to conventional osteosarcoma.     

Abrikossoff's tumour: report of a rare case in anal perianal region

The authors report a rare case of a 47-year old man suffered from Crohn's disease and depression with multiple prominent lesions in the anal and perianal region. The biopsy of these lesions showed the presence of Abrikossoff's tumour. This tumour is very rare in the anal region and usually the lesions are small (0.5-3 cm) and solitary. The authors report this case because they considered it an interesting case for the localization and the appearance of the lesions.     

Carcinosarcoma: a rare tumour of the breast

This report presents a case of carcinosarcoma, a rare tumour of the breast. The clinical and histological features and management are discussed.     

Gaint cell tumour of manubrium sterni - a rare case

Giant cell tumors of the manubrium sterni are extremely rare. There are no reports of gaint cell tumor involving the manubrium sterni in the literature till date. They typically arise in the metaphyseal-epiphyseal area most commonly in the distal femur, proximal tibia, and distal radius. They have rarely been reported in other sites, such as the chest wall or sternum. We report a case of an isolated giant cell tumor of the manubrium sterni in a 28-year old lady who presented with a slowly growing mass over the upper chest in the midline. X- Ray showed an expansile cystic lesion over the manubrium with intact cortex. Computed Tomography chest showed similar findings and Fine Needle Aspiration Cytology had features of giant cell tumor. The mass was resected and the manubrium was primarily reconstructed with bilateral pectoralis muscle advancement flaps with excellent functional and aesthetic result. The resected specimen showed microscopic features of benign giant cell tumor. The patient is well with stable stenum and no evidence of recurrence. At 2 months of follow up.     

Mediastinal ganglioneuroma: a rare and often asymptomatic tumour. A case report

Ganglioneuroma is a very rare tumour characterised by a slow evolution and arising from cells originating in the neural crest. This type of tumour occurs most commonly in the posterior mediastinum and is considered malignant in childhood and benign thereafter. It grows very slowly and can usually grow to a substantial size without giving rise to an evident clinical picture. We report the case of an 18-year-old asymptomatic patient, who had undergone a medical examination in a military hospital where a chest X-ray revealed a roundish mass in the right lung displacing the trachea to the left. Computed tomography showed that the mass was located in the posterior mediastinum. Surgical resection was performed. The tumour was encapsulated and adhered tightly to the pleura of the costovertebral angle. Histological examination revealed a ganglio-neuroma composed of Schwann cells and mature ganglion cells without any neuroblastomatous elements. This case of a mediastinal ganglioneuroma in a young male patient is a typical example of this rare pathological entity. The totally asymptomatic clinical picture could be explained by the very slow growth of this type of tumour which can displace the surrounding anatomical structures without infiltration.     

A rare case of solitary colonic fibromatosis mimicking a cystic tumour

Mesenteric fibromatosis or intrabdominal desmoid tumour is a rare proliferative disease that frequently involves the gastrointestinal tract. These tumours account for less than 3% of all tumours among soft tissue neoplasms. Most such lesions are large (10cm or more in diameter) at the time of the excision. Although grossly well-circumscribed, they lesions typically infiltrate the surrounding soft tissues including the bowel wall. The case described here concerns a 62-year-old woman with a partly cystic mesenteric fibromatosis incorporating the large bowel wall, which degenerated into an abscess. A right colectomy was undertaken, and the sample was sent for histological examination. Based on the morphological and immunohistochemical findings, a diagnosis of abdominal fibromatosis was established. The surgical margins were free of disease, and the examined lymph nodes showed cortical hyperplasia and histiocytosis of the lymph sinuses. The patient recovered from surgery with no complications; her fever subsided, and she was discharged from hospital 12 days later. Surgery is the primary therapy for extra-abdominal and intra-abdominal desmoid tumours. Considering the unique biology of desmoid tumours, conservative treatment strategies merit further investigation in both unresectable primary disease and resectable disease. The optimal criteria for choosing surgical or non-surgical treatment still need further evaluation with randomized controlled studies.     

Melanotic neuroectodermal tumour of infancy: a rare maxillary alveolar tumour

Melanotic neuroectodermal tumour of infancy is a rare, but usually benign, pigmented neoplasm occurring in the first few months of life. The most frequent site is anterior maxillary alveolar ridge. It should be differentiated from other head and neck neoplasms of infants. Computed tomography and fine-needle aspiration cytology are the choice of investigations while two-cell population of neuroblasts and melanocytes on histology confirms the diagnosis. Treatment is early conservative surgical excision with long-term follow-up to detect recurrence and malignant transformation. A classical case of a 7-month-old infant is described with a brief review of the literature.     

Intermediate nerve schwannoma: a rare tumour

The intermediate nerve (IN), also called the nerve of Wrisberg, is a relatively small nerve with a variable anatomical course. Currently, this nerve is not addressed well in the pertinent literature. Pre-operative diagnosis of IN tumours is clinically challenging due to the lack of related symptoms. Symptoms are typically caused by the functional deterioration of neighbouring anatomical structures. In this report, we describe a patient with a schwannoma that arose at the porus acusticus of the internal auditory canal; during surgery, this proved to originate from the IN. We discuss our findings in the context of previously reported cases of IN schwannomas and analyse the vague characteristics and symptoms of this rare tumour.     

Group G streptococcus--a rare cause of osteomyelitis simulating bone tumour: a case report

We report a case of osteomyelitis of the proximal femur caused by Lancefield group G streptococcus in a 71-year-old otherwise healthy man. The organism has rarely been identified as the cause of osteomyelitis. The subacute nature of the symptoms and the radiological appearance of the femur in this patient mimic bone tumour. The patient was successfully treated with conservative methods, including a prolonged period of oral antibiotics. We stress the importance of histological and bacteriological evidence in avoiding misdiagnosing patients with equivocal clinical and radiological presentation.     

Multifocal metachronous giant cell tumour in bilateral upper limb: a rare case presentation

Giant cell tumour (GCT) of bone is a benign but locally aggressive tumour and accounts for 20 % of all benign bone tumours and 5 % of all bone tumours. Multicentric GCT of bone is a rare entity and has increased prevalence of involvement of the small bones of hands and feet in multicentric GCT. The clinical behaviour in multicentric GCTs tends to be aggressive as in recurrent GCTs. En-bloc resection remains the most successful surgical technique for treating both multicentric and solitary lesions. We report a 14-year-old female patient presenting with metachronous benign GCT located at the right proximal humerus and subsequent lesions in left hand and left proximal humerus. The case was treated with multimodality therapy including en-bloc resection along with bisphosphonate therapy over a period of 5 years.     

Primary Leydig cell tumour of epididymis: a rare case report with review of literature

Leydig cell tumour (LCT) is an uncommon tumour that typically occurs in the testis. Primary epididymal LCT is extremely rare. To the best of our knowledge, only two cases have been reported in the world literature. Herein, we report a case of primary epididymal LCT in a 41‐year‐old Chinese male. The patient presented with right epididymal swelling for 3 months without endocrine manifestations, including gynaecomastia and decreased libido. Scrotal ultrasound demonstrated a mass about 1.5 cm in diameter entirely in the cephalic region of right epididymis. No abnormality was found in his bilateral testes. The patient underwent total mass resection without post‐operative therapy. Histological examination revealed that the well‐circumscribed tumour was separated by conspicuous hyalinised fibrous stroma; the tumour cells were large and polygonal with round nuclei and abundant eosinophilic cytoplasm. Immunophenotypically, the tumour cells expressed four markers of sex cord differentiation (calretinin, melanA, CD99 and inhibin). There was no recurrence at 2‐year follow‐up. Our observation once again confirms that LCT could primarily occur in the epididymis, and we suppose that it probably originates from the ectopic Leydig cells. As little is known about the pathogenesis and prognosis for such a rare disease, accumulation of more pathological and clinical data can help to better interpret this tumour.     

Syringotropic mycosis fungoides: a rare variant of the disease with peculiar clinicopathologic features

A rare variant of mycosis fungoides (MF) characterized by prominent involvement of the eccrine glands with syringometaplasia has been reported in the past as "syringolymphoid hyperplasia with alopecia," "syringotropic cutaneous T-cell lymphoma," "adnexotropic T-cell lymphoma," or "syringotropic MF." The clinicopathologic features of this variant are not well understood, and only a few case reports or small series have been published to date. We reviewed the clinicopathologic features of 14 patients with syringotropic MF (male:female=10:4; median age, 59 years; mean age, 57.8; age range, 33 to 83 y). Six patients had variably large, solitary patches or plaques, located on the thigh (n=3), arm, trunk, or eyebrow (1 each). The other 8 patients had multiple, mostly generalized lesions. A history of MF was known in 4 of these 8 patients. With the exception of 1 biopsy specimen that was too superficial and did include the eccrine secretory coils but not the eccrine glands, all cases showed prominent involvement of the eccrine glands. Variable degrees of syringometaplasia ranging from small to large epithelial complexes were present in all specimens. The eccrine glands and syringometaplastic structures were surrounded by dense lymphoid infiltrates with prominent epitheliotropism. Concomitant involvement of the epidermis and of the hair follicles was observed in 13 and 8 biopsies, respectively. This is the largest series of syringotropic MF, showing that this is a rare variant of the disease with peculiar clinicopathologic features. Dermatologists and dermatopathologists should be aware of this rare variant of MF to avoid delayed diagnosis and treatment.