Oncological and functional results of cryosurgical therapy of enchondromas and chondrosarcomas grade 1.

BACKGROUND AND OBJECTIVES: Cryosurgery using liquid nitrogen is used as adjuvant treatment after intralesional resection of bone tumours to induce cell death. It is applied to enlarge the oncological margins of resection and to reduce the local recurrence rate. The objective of this study is to analyze the oncological and functional results. METHODS: We studied the oncological and functional results of curettage and cryosurgery in 123 patients with 130 tumors. There were 75 enchondromas and 55 chondrosarcomas grade 1. The minimal follow-up period for all patients was 2 years (range 24-119 months). RESULTS: During follow-up one local recurrence of an active enchondroma and one local recurrence of an aggressive enchondroma occurred. They were treated with curettage and cryosurgery again. Both patients were disease-free at minimum of 3 years follow-up. No local recurrences after treatment of chondrosarcoma grade 1 were seen. Functional scores, according to the MSTS scoring system, showed an average score of 28 points (94%) at 2 years follow-up. Post-operative fractures were seen in 18 patients (14%). CONCLUSIONS: Curettage and cryosurgery for enchondroma and chondrosarcoma grade 1 has excellent oncological and functional results. The post-operative management has been adjusted to reduce the number of fractures.

Ring chromosomes in a malignant mesenchymoma.

We report, for the first time, the cytogenetic and molecular genetic constitution of a human mesenchymoma. As in several other soft tissue sarcomas, supernumerary ring and rod-shaped marker chromosomes were observed next to an otherwise normal diploid karyotype. Comparative genomic in situ hybridization and whole chromosome painting experiments revealed that chromosome 1q21-q25 and 12q14-q15 sequences were amplified, and that these sequences resided on the supernumerary marker chromosomes. We assume that, in this malignant mesenchymoma, the observed chromosomal anomalies may be associated with its well differentiated liposarcomatous component.

Ring melanoma of the iris and ciliary body.

The case is described of a tumour of the iris and ciliary body--a ring type melanoma--primarily diagnosed and treated as pigmentary glaucoma.

Malignant eccrine poroma with lymph node metastasis followed by multiple basal cell carcinomas in a young female patient.

A case of metastasizing malignant eccrine poroma is described. This case is unique because a relatively young patient presented later on with multiple basal cell carcinomas. Our observation is compared with a review of the literature.

Treatment of benign and low-grade malignant intramedullary chondroid tumours with curettage and cryosurgery.

AIMS: To shed light on the controversy surrounding the methods of evaluating, staging and final treatment of intramedullary chondroid lesions. Controversy particularly exists for enchondroma and low-grade chondrosarcoma located in the extremities, because their accurate distinction is hampered by their radiographical and histological similarity. METHODS: Since 1991 we have treated 22 patients (mean age: 39.6 years) with 26 lesions (three chondroblastomas, 14 enchondromas and nine grade 1 chondrosarcomas) with curettage, cryosurgery and bone grafting. RESULTS: After a mean follow-up of 26 months no recurrences were observed. Complications consisted of two post-operative fractures, one wound infection and one intraoperative venous gas embolism. All bone grafts incorporated, resulting in full weight-bearing capacity and excellent functional results. CONCLUSION: The usefulness of a combination of curettage and cryosurgery as adjuvant therapy is considered to be equal to marginal resection according to orthopaedic oncological principles. The pre-operative assessment of these lesions and cryosurgical technique is described in detail.

Synovial sarcoma: oncological and functional results.

AIM: To evaluate the oncological and functional result of the treatment of patients with a synovial sarcoma. This paper gives a retrospective review of 20 patients (15 male and five female) treated for synovial sarcoma at the Nijmegen University Hospital, The Netherlands. METHODS: The median age of the patients was 30 years (range: 14-71, mean 37 years). RESULTS: The tumour locations were: lower extremity in 12 patients; upper extremity in three; pelvic and groin region in four; and the retroperitoneal space in one. Surgical stages according to Enneking (Clin Orthop 1986; 204: 9-24) were IIA in five cases; IIB in seven; and IIIB in eight. The surgical margin was intralesional in three cases; marginal in three; wide in six; and radical in six. In one case the surgical margin could not be assessed and one patient was not operated. One patient developed a recurrent tumour and one developed nodal metastases. Eight patients who did not have metastases at the time of diagnosis developed metastase during follow-up. Fourteen patients died of metastatic disease; one patient died of diabetes; one is alive with disease; and four presently do not have evidence of disease. CONCLUSIONS: The outcome was poor, especially due to the occurrence of pulmonary metastases. The functional result according to the MSTS was 100% in four patients; in one the result was 93% because of the scar and venous insufficiency.

Immunohistochemical and ultrastructural studies of a primary aortic intimal sarcoma.

An intimal sarcoma of the abdominal aorta in a 63-year-old woman is reported. The clinical symptoms consisted of chronic arterial hypertension, vomiting and epigastric pain. Treatment was operative, but the patient died 20 hours after surgery. The studies were performed on a surgical specimen and on autopsy material. The aortic tumour consisted of pleomorphic spindle-shaped and giant cells. In the vertebral metastases a storiform pattern of the tumour cells was found. No specific features characteristic for leiomyogenic, lipogenic or an endothelial nature of the tumour giant cells was disclosed in electron microscopy and the picture rather indicated their histiocytic character. Of the 18 cellular markers studied, the immunostainings for vimentin and alpha-1-antichymotrypsin were evidently positive. The tumour was classified as a pleomorphic intimal aortic sarcoma probably a malignant fibrous histiocytoma (MFH). The literature on 26 previously published aortal tumours is reviewed with emphasis on their topographical distribution and histological classification. In only 4 previous cases was the final diagnosis supported by electron microscopical or immunopathological findings. The role of marker studies in the classification of aortal tumours is discussed.

Intramural oesophageal cyst as a cause of acute respiratory distress.

A case is reported of an intramural oesophageal cyst. Acute dyspnoea, due to compression of the trachea, was an indication for exploratory sternotomy.

Lipomatous hypertrophy of the interatrial septum: indication for surgery?

A fortuitous finding during open heart surgery of lipomatous hypertrophy of the interatrial septum is described in a 65-year old man with ischaemic heart complaints due to coronary artery disease and with premature ventricular contractions. An incision biopsy confirmed the diagnosis. The choice of treatment of lipomatous hypertrophy of the interatrial septum is controversial. Indications for surgery and surgical techniques are discussed.

Unusual intracytoplasmic inclusions in metastatic carcinoma. Discussion of their possible significance.

Unusual filamentous inclusion bodies in the cytoplasm of metastatic tumor cells are described. Their presence (intermingled with zymogen granules) seems rather restricted to cells of primary or metastatic acinar cell carcinoma of the pancreas, acinic carcinoma of salivary gland (parotid gland) and Paneth cells (neoplastic or in zinc deficiency state). For the time being, the real nature of these inclusions (deranged zymogen granules?) is unclear. This case also emphasizes the value of electron microscopy in solving the problem of the occult primary tumor and avoiding the misdiagnosis of an endocrine tumor (e.g. islet cell tumor or carcinoid), or a duct cell tumor with eosinophilic granular cytoplasm or in recognizing the foci of acinar cells in a mixed variant of carcinoma of the pancreas.

An autoradiographic study of the NK/Ly tumor transplanted into the brain of the mouse.

The NK/Ly ascites tumor transplanted into the brain of mice is well accepted and the growth is similar to that of malignant lymphomas in the human brain. The authors studied the incorporation of the tritiated thymidine in the malignant lymphoma cells transplanted into brain of the mouse by autoradiographic method.

Aneurysmal bone cysts treated by curettage, cryotherapy and bone grafting.

We treated 26 patients with 27 aneurysmal bone cysts by curettage and cryotherapy and evaluated local tumour control, complications and functional outcome. The mean follow-up time was 47 months (19 to 154). There was local recurrence in one patient. Two patients developed deep wound infections and one had a postoperative fracture. We compared our results with previous reports in which several different methods of treatment had been used and concluded that curettage with adjuvant cryotherapy had similar results to those of marginal resection, and that no major bony reconstruction was required. We recommend the use of cryotherapy as an adjuvant to the surgical treatment of aneurysmal bone cysts. It provides local tumour control. Combination with bone grafting achieved consolidation of the lesion in all our patients.

Malignant fibrous histiocytoma of the stomach during pregnancy: a case report.

In this case report we describe a patient with a primary malignant fibrous histiocytoma of the stomach, diagnosed and operated upon during the sixth month of pregnancy. We stress the importance of a thorough diagnostic examination in cases of severe anaemia during pregnancy. Although very rare, malignancy of the gastrointestinal tract should be taken in consideration. No holding back is justified in diagnostic and therapeutic measurements because of pregnancy. Radiologic examination of the gastrointestinal tract should be replaced by endoscopy.

Was isolation of Veillonella from spinal osteomyelitis possible due to poor tissue perfusion?

From a needle biopsy of the body of the 12th thoracic vertebra in a 74-year-old man with spinal osteomyelitis a Veillonella parvula was isolated. The significance of this bacterium as lactic acid indicator has been considered and discussed. Since this strictly anaerobic bacterium mainly uses lactic acid for energy generation, lactic acid must continuously and sufficiently have been produced in or near to the vertebrate body to permit outgrowth of this bacterium. Since neither microbial infections nor tumours could be demonstrated, we finally hypothesised that in this patient poor tissue perfusion has been the primary cause of lactic acid production.

Atypical mycobacterial infection of the parotid gland.

A localized atypical mycobacterial infection of the major salivary gland is a rare disease. In this report the cases of three patients with this lesion are presented. The diagnosis was based on the clinical picture, skin testing with specific antigens, bacteriologic culture, and histopathologic findings. The patients were successfully treated by total parotidectomy with facial nerve preservation, which in our opinion is the therapy of choice in localized atypical mycobacterial infections.

The midline cervical cleft.

The midline cervical cleft is a rare congenital anomaly of the ventral neck. With reference to two patients with a midline cervical cleft, we discuss the clinical picture, the embryology, and the surgical treatment.

Unusual haemostasis for an unusual tumour. Catastrophic bleeding from a tactile neurofibroma affecting the thoracic vertebrae. Case report.

The symptomatology of a typical acute descending thoracic aorta dissection was imitated by profuse haemorrhage caused by a benign tumour composed almost exclusively of Wagner-Meissner like tactile corpuscles and fatty tissue. The tumour caused extensive destruction of the bodies of the fifth and sixth thoracic vertebrae at the level of the vertebro-costal articulation. Emergency cross clamping of the descending aorta and haemostasis of the bleeding from osteal defects by tamponade with bone polymethyl methacrylate appeared the only way to control the life threatening haemorrhage. It seems that an intrathoracic tactile neurofibroma with a similar case history has not been reported till now.

Unilateral hearing loss due to a rhabdomyoma in a six-year-old child.

A case report of a six-year-old child is presented, who had had a unilateral sensorineural hearing loss for several years. Because of impairment in the ABR as well as in the caloric testing a MRI and CT scan were performed. A 17 mm tumour in the cerebellopontine angle (CPA) was detected, which after suboccipital surgery proved to be a rhabdomyoma. This tumour has not been described before in the CPA. Unilateral sensorineural hearing loss should, at all ages, be an indication for further (radiodiagnostic) investigations.

Eosinophilic granuloma of bone: results of treatment with curettage, cryosurgery, and bone grafting.

Intralesional instillation of steroids is currently the first choice of treatment for eosinophilic granuloma of bone. However, some lesions fail to respond or are unsuitable for injection therapy due location, pending pathologic fracture, and soft tissue intrusion. The authors treated six patients with these lesion characteristics using curettage, cryosurgery, and bone grafting. After a mean follow-up of 34.3 months, all lesions healed completely, although one femoral fracture occurred 8 months after the operation. In selected cases of eosinophilic granuloma of bone, a primary surgical treatment seems feasible. The use of cryosurgery as adjuvant treatment extends the surgical margin and is of value in averting local recurrence.

[Chronic recurrent multifocal osteomyelitis]. / Chronische recidiverende multifocale osteomyelitis.

In a 9-year-old girl chronic recurrent multifocal osteomyelitis was diagnosed, a rare disease mostly affecting children of which the aetiology remains obscure. It is usually diagnosed around the age of II years. The patient suffers from exacerbations and remissions for an average of 2.9 years. Awareness and recognition of the disease is of importance to avoid unnecessary diagnostic procedures and treatment. The prognosis is good.