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Congenital smooth muscle hamartoma of the skin.

A 2-month-old white girl had a congenital, tan-colored, slightly elevated, 3 x 2-cm plaque on the left midback. Clinically, the lesion was suspected to be a solitary mastocytoma. Rubbing the lesion produced a transient erythema and edema that was similar to Darier sign seen in mastocytoma. Analysis of a skin biopsy specimen revealed a smooth muscle hamartoma; special stains did not show evidence of increased numbers of mast cells. This relatively uncommon condition can be confused with a variety of other cutaneous diseases. Light microscopic examination of a skin biopsy specimen establishes the diagnosis. A review of the approximately 50 cases reported in the literature showed that there is no known associated systemic involvement or malignant transformation. The clinical lesions usually become less prominent with time.

Biopsy↗

Fine structure of granular cell tumor of Abrikosov.

The case report illustrates the microscopical and ultrastructural findings obtained in a case of Abrikossoff's tumor. On the basis of the light and ultrastructural data a neural histogenesis is suggested.

Adult↗

Inflammatory myofibroblastic tumour of paranasal sinuses with fatal outcome: reactive lesion or tumour?

Inflammatory myofibroblastic tumours (IMTs) are clinicopathologically distinctive but biologically controversial entities, which have been described in the lungs, abdomen, retroperitoneum, and extremities, but rarely affect the head and neck region. IMT usually follows a benign clinical course after radical excision, but invasive, locally recurrent, and metastatic forms of abdominal and mediastinal IMT have also been described. This report describes a case of IMT of the paranasal sinuses with a fatal outcome. A 22 year old woman was admitted to hospital as a result of epistaxis. Computed tomography scan and magnetic resonance imaging showed an expansive process in the paranasal sinuses, extending into the nasal cavity, orbita, and endocranium. The tumour progressed despite several surgical procedures. Radiotherapy, corticosteroids, and chemotherapy were unsuccessful, and the patient died four years after diagnosis, as a result of extensive intracranial spread of the tumour. This is the first known case of an IMT of the head and neck region with a fatal outcome. It shows that the aggressive behaviour of IMTs is not limited to abdominal and mediastinal locations, and supports recent observations that at least a subset of IMTs represents true neoplasia rather than reactive myofibroblastic proliferation.

Adult↗

Multicentric tracheobronchial and oesophageal granular cell myoblastoma.

Two patients with multiple intrathorcic granular cell myoblastomas are described. In one case multiple tumours were present in the major airways and oesophagus. The patient presented with recurrent pulmonary infections and stridor due to airway obstruction. In the other case dysphagia caused by multiple oesophageal granular cell myoblastomas was the major symptom. Granular cell myoblastoma is a rare tumour of neurogenic origin with a characteristic histological appearance. The pattern of multiple tracheobranchial and oesophageal tumours is uncommon and forms the basis of this report.

Adult↗

Granular cell tumor of the trachea.

Granular cell tumors are rare neoplasms that arise in different regions of the body. Their cell of origin is still debated among many pathologists, but recent authors consider them to be neurogenic. These tumors are believed to be benign; however, local recurrences after excision and multifocality arouse suspicions for malignancy. In the head and neck, granular cell tumors occur most frequently in the tongue. In the laryngotracheobronchial tree, most tumors reported have been in the larynx and the next most in the bronchi. Only six cases of true tracheal occurrences have been reported previously; two additional cases are reported in this paper. The literature of the cell of origin of these tumors is reviewed, the different theories are presented, and diagnosis, treatment, and follow-up are discussed.

Adult↗

Acute bleeding from leiomyoblastoma of the greater omentum. A case report.

Extragastric leiomyoblastomas are extremely rare. This is the sixth reported case of leiomyoblastoma of the greater omentum, and the only one with acute major bleeding which required an emergency operation. Leiomyoblastomas are low-grade malignant tumors, and especially the gastric ones have an excellent prognosis after surgical removal. Extragastric leiomyoblastomas have a higher rate of dissemination. However, none of the previously reported omental leiomyoblastomas showed signs of metastatic spread at the time of operation. The present patient is alive and well 18 months post-operatively.

Adult↗

Inflammatory myofibroblastic tumor with bone marrow involvement. A case report and review of the literature.

Inflammatory myofibroblastic tumor, also referred to as inflammatory fibrosarcoma, is a rare tumor composed of myofibroblastic spindle cells of uncertain etiology and disputed nosology. We report a case of inflammatory myofibroblastic tumor of the omentum with involvement of the bone marrow in an 18-year-old man. Histologic and immunohistochemical studies of the abdominal mass and bone marrow were consistent with inflammatory myofibroblastic tumor. Additionally, fluorescence in situ hybridization using a probe specific for the ALK gene showed disruption of the gene. The literature is reviewed with emphasis on the ability of inflammatory myofibroblastic tumor to recur, metastasize, and cause mortality.

Adolescent↗