Recurrent phyllodes tumours of the breast: pathological features and clinical implications

BACKGROUND: Phyllodes tumours (PT) of the breast are fibro-epithelial neoplasms that are known to recur locally in up to 19% of patients. The failure to achieve adequate surgical margins is an important risk factor for local recurrence. This, however, is a common problem as PT are clinically similar to the more common fibro-adenoma and are therefore often locally excised without any gross surgical margins. It is still debatable as to whether it is necessary to subject the patient to repeat surgery to obtain pathologically negative margins after a diagnosis of a benign or borderline PT is made. Although the majority of recurrences are histologically similar to the initial tumour, a malignant recurrence is possible. Malignant tumours can metastasize through the haematogenous route and metastases are associated with a poor prognosis as they are poorly responsive to conventional chemotherapy. METHODS: We retrospectively reviewed 37 women who presented with local recurrence over a 10-year period to the Singapore General Hospital. Data, including age at the time of diagnosis, clinical presentation, histological features, type of surgery carried out, clinical progression and characteristics of locally recurrent disease, were analysed. Comparisons were made between those with benign, borderline and malignant tumours, as well as between those who developed a malignant recurrence and those who did not. RESULTS: The mean age at the time of diagnosis was 39.6 +/- 7.4 years and the mean tumour size was 6.0 +/- 5.1 cm. A total of 22 patients were classified as having benign tumour, 9 as having borderline tumour and 6 as having malignant tumour. Tumour grade did not influence the tumour size, the adequacy of surgical margins or the time interval to local recurrence or the number of recurrences. Local recurrence occurred after a median interval of 20 months. Although malignant tumours tended to recur earlier, this was not found to be statistically significant. The majority of recurrent tumours were histologically similar to the initial tumour; however, seven patients (19%) developed a malignant recurrence from an initially benign or borderline tumour. Although these tumours were larger, recurred more frequently and within a shorter interval, no significant predictive factor was found on multivariate analysis. Distant metastasis developed only in patients with malignant tumours and accounted for all three mortalities in the study. CONCLUSIONS: It may be acceptable to use an expectant management towards benign and borderline tumours that are excised without adequate surgical margins. However, surgery for locally recurrent tumours, as well as malignant tumours, should aim to achieve adequate surgical margins to reduce the risk of local recurrence, particularly that of a malignant recurrence.     

Malignant peripheral nerve sheath tumours--report of 8 cases and review of the literature.

BACKGROUND: Though Malignant peripheral nerve sheath tumours (MPNST) are a rare entity accounting for 5-10% of soft-tissue sarcomas they are an important differential diagnosis to benign tumours of the peripheral nervous system regarding treatment and prognosis. METHOD: We present our experience with eight patients who underwent surgery for MPNST at the Department of Neurosurgery between 10/1990 and 9/1999. The median age was 37 years [range: 13-64], the male/female ratio was 1:1. Two patients suffered from Neurofibromatosis type 1. Paraffin embedded tumour specimens were immunohistochemically stained for S-100, p53 and Ki67/MIB-1. FINDINGS: The most frequent initial symptoms were local swelling and pain followed by irritation of cranial nerves and spinal ataxia. Four tumours were localised at the head & neck region, three were found in the extremities and one tumour was located on the trunk. All patients underwent surgery with curative intent, but total resection, defined by negative surgical margins, was achieved in only 3 cases. All of these developed local recurrence with a mean disease free survival time of 10.6 months. In five cases, adjuvant radiation was given. During follow up, three patients developed distant metastases located in the lung, liver and subcutaneous tissue. Five out of eight patients died during follow-up with a mean survival time of 11.6 months after diagnosis. Results of immunohistochemical staining were as followed: S-100 (7/8), p53 (7/8). The Ki67/MIB-1 proliferation index was detectable in all tumour samples, it differed from 10-30%. INTERPRETATION: MPNST is a rare and fatal diagnosis in neurosurgery with high risk of local recurrence and occurence of distant metastases. Though mulitimodal therapy including surgical resection and adjuvant radiotherapy including brachytherapy is available, the prognosis remains dismal. Modern clinical studies and the development of effective chemotherapy is needed in order to gain control of the disease.     

Surgical strategy in primary retroperitoneal tumours

Sixty-nine patients with primary retroperitoneal tumours (17 benign, 52 malignant including 4 malignant tumours of uncertain origin) were reviewed to determine the best form of surgical strategy. Total resection was performed in 88 per cent of benign cases and in 65 per cent of malignant cases. In 62 per cent of the total resections for malignant tumours, en bloc excision included adjacent organs or anatomical structures. Operative mortality rate (in terms of the total number of operations performed) was 5 per cent. Postoperative complications occurred in 14 per cent and recurrences in 35 per cent. The overall 5-year survival rate was 67 per cent in patients with totally resected tumours and zero in patients whose tumours were treated by partial resection or biopsy. An aggressive surgical approach aimed at total excision of the tumour is the best form of therapy currently available. In the totally resected retroperitoneal tumour, the use of adjuvant radiotherapy and/or chemotherapy depends on the grade of the malignancy and clearance as assessed histologically. Careful follow-up based on the use of computerized axial tomography and ultrasound allows early identification of recurrence at a stage when the recurrence is amenable to total resection.     

Definitive surgical management for soft-tissue sarcomas

The surgical management of soft-tissue sarcomas, a seemingly heterogeneous group of malignant tumours, depends on the circumstances (e.g., untreated primary tumour, inadequately resected primary tumour, local recurrence, metastasis) and site. The basic steps in managing a primary tumour include using the correct method to establish a diagnosis, obtaining adequate tumour-free resection margins and giving consideration to adjuvant radiotherapy or chemotherapy, or both. Local recurrences and metastatic lesions require a multidisciplinary approach. The relative rarity of this group of sarcomas and the low survival rate associated with them make it mandatory that such patients be treated in centres able to provide specialized care from the beginning.     

Primary amelanotic anorectal melanoma--a case report

BACKGROUND: The amelanotic melanoma of the rectal mucosa is very rare with an unfavourable prognosis. The surgical approach is still discussed controversially. Therefore transrectal ultrasound is of major importance in the preoperative staging and postoperative follow-up especially in diagnosis of local recurrence by using the ultrasound-guided, transrectal aspiration. METHODS: In literature 5 cases of amelanotic malignant melanoma were reported. The overall survival time is 10 months after diagnosis. All patients were female. RESULTS: We report about a 55-year-old female patient with an amelanotic melanoma of rectal mucosa. 7 months after a wide local excision of the tumour and interferon therapy in case of the absence of pararectal, inguinal metastases and other metastases the patient developed pararectal metastasis. An abdominoperineal resection and resection of inguinal lymph nodes was performed. Two months later paraaortal lymph nodes were detected. We started chemotherapy with Dacarbazin and with regard of the tumour progress the chemotherapy was changed to Vindesin 25 months after first operation supported by a radiotherapy with 40 Gray. The patient died 36 months after diagnosis. CONCLUSION: The prognosis of primary malignant anorectal melanoma is poor, irrespective of surgical treatment. Wide local resection is the first choice for primary anorectal melanoma. Abdominoperineal resection should be reserved for cases were complete tumour resection is impossible. Chemotherapy, radiotherapy and immunotherapy should be considered in the treatment of anorectal melanoma to influence the overall survival.     

Spinal teratoma: is there a place for adjuvant treatment? Two cases and a review of the literature

Spinal teratomas are rare intradural spinal tumours, commonly of benign histological profile. The main treatment remains surgical excision which often is incomplete as the tumour is frequently intimately related to surrounding nerve roots and adjacent spinal cord. The value of adjuvant treatment in the form of radiotherapy has not been clearly identified. We report two patients with spinal teratomas in the lower thoracic and conus region, who were treated with surgical excision and did not receive radiotherapy. One of the tumours recurred at 38 months following surgical excision and required a second operation. We review in addition another 20 patients reported in the literature with such tumours. With a mean follow-up of 25 months a local recurrence rate of 10% was observed, the same in both total and subtotal excision groups. No deaths were reported. Analysis of cumulative survival from surgery to treatment failure demonstrated that recurrence occurs late, verifying a slow biological progression of this tumour type. Although no reliable information of the effect of radiotherapy can be obtained, it is difficult to see how radiotherapy could have an impact on such a slow growing tumour. Initial treatment of choice remains surgical excision. If the teratoma shows any malignant histological features or germ cell elements radiotherapy should be employed after surgical excision, even if apparently totally excised. In the presence of benign histology it would seem appropriate to avoid radiotherapy after initial surgery. In case of tumour recurrence or progression to explore the possibility of further surgery first, before considering radiotherapy which even then may have doubtful efficacy.     

Surgical management of thoracic malignancy in childhood: eight years' experience in Leeds

During the period 1978 to 1986, 13 children aged 2-15 years underwent surgical resection of malignant thoracic tumours. Five children with neuroblastomas presented with chest pain and infections, pleural effusions, dysphagia, lymphadenopathy and paraplegia. Chemo- and radiotherapy were given preoperatively to previously diagnosed cases and postoperatively to all survivors. At operation, complete tumour clearance was possible in only two cases. Two children remain alive with no sign of recurrence at 6 and 7 1/2 years. Eight children with pulmonary metastases had undergone resection of the primary tumour and systemic chemotherapy. All were asymptomatic and were detected by chest radiographs. Wedge resection or lobectomy was performed. Two required contralateral resections at 4 months. Two children remain alive with no evidence of recurrence at 2 and 6 1/2 years. We conclude that aggressive surgical resection of childhood thoracic malignancy is worthwhile, but cooperation with a paediatric oncology team is essential.     

Surgical treatment of patients with stenosis of the central airway due to tracheal tumours

This study retrospectively evaluated bronchoscopic and surgical treatments for patients with central airway stenosis due to tracheal tumours. Seven patients treated by resection and reconstruction of the trachea for tracheal tumours between 1994 and 2008 were retrospectively reviewed. The most common histological finding was thyroid carcinoma (n = 3), followed by adenoid cystic carcinoma (n = 2), a metastatic thyroid tumour (n = 1), and a benign granular cell tumour (n = 1). Three of the patients required preoperative laser treatment (Nd:YAG) for recanalization. Five patients underwent end-to-end anastomosis for reconstruction. There was no postoperative mortality or morbidity such as anastomotic insufficiency of the reconstructed trachea. Three patients with a microscopic residual tumour required postoperative external radiotherapy. Surgical resection of malignant tracheal tumours is recommended not only for curative purposes, but also for reduction of the risk of smothering.     

Primary tumours of the ribs

Thirty-four primary rib tumours (24 benign, 10 malignant) were surgically treated in 1966-1985. The mean age was higher and the tumour diameter was greater in the patients with malignant, than in those with benign neoplasm. The benign tumours were excised without operative death. At follow-up after a mean of 12.3 years there was no recurrence of benign growth, but in two cases with initial diagnosis of chondroma a regrowth at the same site proved to be chondrosarcoma. Among the cases of malignant tumour there was one operative death from pulmonary embolism, after radical resection of sarcoma. None of the four patients with chondrosarcoma had recurrence 6-13 years after surgery. There was no long-term survival among the patients with other forms of sarcoma or malignant tumour of the reticuloendothelial system.     

Malignant Peripheral Nerve Sheath Tumours – Report of 8 Cases and Review of the Literature

Summary  Background. Though Malignant peripheral nerve sheath tumours (MPNST) are a rare entity accounting for 5–10% of soft-tissue sarcomas they are an important differential diagnosis to benign tumours of the peripheral nervous system regarding treatment and prognosis.  Method. We present our experience with eight patients who underwent surgery for MPNST at the Department of Neurosurgery between 10/1990 and 9/1999. The median age was 37 years [range: 13–64], the male/female ratio was 1:1. Two patients suffered from Neurofibromatosis type 1. Paraffin embedded tumour specimens were immunohistochemically stained for S-100, p53 and Ki67/MIB-1.  Findings. The most frequent initial symptoms were local swelling and pain followed by irritation of cranial nerves and spinal ataxia. Four tumours were localised at the head & neck region, three were found in the extremities and one tumour was located on the trunk. All patients underwent surgery with curative intent, but total resection, defined by negative surgical margins, was achieved in only 3 cases. All of these developed local recurrence with a mean disease free survival time of 10,6 months. In five cases, adjuvant radiation was given. During follow up, three patients developed distant metastases located in the lung, liver and subcutaneous tissue. Five out of eight patients died during follow-up with a mean survival time of 11,6 months after diagnosis.  Results of immunohistochemical staining were as followed: S-100 (7/8), p53 (7/8). The Ki67/MIB-1 proliferation index was detectable in all tumour samples, it differed from 10–30%.  Interpretation. MPNST is a rare and fatal diagnosis in neurosurgery with high risk of local recurrence and occurence of distant metastases. Though mulitimodal therapy including surgical resection and adjuvant radiotherapy including brachytherapy is available, the prognosis remains dismal. Modern clinical studies and the development of effective chemotherapy is needed in order to gain control of the disease.     

Treatment and outcome of cystosarcoma phyllodes in Brunei: a 13-year experience

Cystosarcoma phyllodes is a rare tumour of the breast whose clinical behaviour does not correlate well with histological findings. The optimal treatment of this tumour remains controversial. A retrospective study on the treatment and outcome of women diagnosed with cystosarcoma phyllodes between 1986 and 1998 in Brunei was undertaken. Twenty-seven women were diagnosed over the 13-year study period. Follow-up was complete in 26 cases. The mean age at diagnosis was 35 years. There were 19 (73%) histologically benign lesions, 3 (12%) borderline lesions and 4 (15%) malignant lesions. The mean follow-up period was 37 months. Four patients (16%) had recurrences after surgery (1 benign, 1 borderline and 2 malignant lesions). Mean time to recurrence was 9 months. Breast-conserving surgery with adequate resection margin is advocated in benign and borderline lesions. For malignant lesions, simple mastectomy without routine axillary dissection is recommended. More research is required to determine the role of adjuvant chemotherapy and radiotherapy in the management of malignant cystosarcoma phyllodes [corrected].     

Functioning adrenocortical neoplasms in children

OBJECTIVE: To describe the clinical features, treatment and outcome of functioning adrenocortical tumours in children, and to assess the value of tumour size as a marker for malignant potential. PATIENTS AND METHODS: Twenty-one children (mean age at presentation 4.9 years, range 0.6-11; 12 girls and nine boys) who presented with a functioning adrenal mass over a 29-year period (1972-2000) were assessed. Clinicopathological features and outcome information were obtained from a structured review of the case-notes. Before 1980 the diagnosis and staging was mainly obtained by intravenous pyelography and plain X-ray. Since 1980, ultrasonography, computed tomography and more recently magnetic resonance imaging were the methods of choice. RESULTS: The mean (range) duration of symptoms before diagnosis was 31 (2-108) months. The symptoms and signs at presentation comprised virilization (18 children), Cushingoid features (three), precocious puberty (three) and hypertension (four). Three children had malignant neoplasms, one presented with metastasis and of the other two, one died from the disease within 6 months, despite adjuvant chemotherapy and radiotherapy. The third girl is disease-free 11 years after complete excision of the neoplasm. Eighteen children had a benign neoplasm and all are alive and free of recurrence. In most of the children surgical extirpation was relatively simple, blood loss minimal, recovery uneventful and the hospital stay short (mean 5 days). The virilizing effects of all the neoplasms gradually resolved after surgery within a median (range) of 27 (6-108) months. CONCLUSIONS: Functioning adrenocortical neoplasms in children are rare, with a peak incidence in the first decade, are predominantly unilateral and more common in girls. Most are benign, but both benign and malignant lesions can present with virilizing and Cushingoid features. The malignant neoplasms have an extremely poor prognosis, especially if excision is incomplete, despite adjuvant chemotherapy and radiotherapy. Although there is an association between tumour size and malignancy, this cannot be used as a reliable individual discriminator.     

Recurrence of olfactory groove meningiomas

OBJECTIVE: Despite apparent gross total resection, olfactory groove meningiomas have a high rate of late recurrence (average, 23%). In this retrospective study, we confirmed that the sites of these recurrences are the cranial base and paranasal sinuses. We postulated that these recurrences stem from conservative handling of the underlying invaded bone. Therefore, we analyzed patient outcomes according to the radicality of surgical resection. METHODS: Fifteen consecutive patients with a diagnosis of olfactory groove meningioma were treated surgically between 1992 and 2001 (nine new cases, six recurrent). Only patients with benign meningiomas were included; atypical and malignant meningiomas were excluded. Surgical resection included the dura and drilling of the underlying bone and resection of involved mucosa. We reviewed each patient's clinical records, radiological studies, sites of recurrence, grade of previous resection, and complications. RESULTS: Olfactory groove meningiomas invaded the underlying bone in 13 cases. All patients with recurrence had previously undergone a surgical resection corresponding only to Simpson Grade 2, which does not include the removal of underlying invaded bone. The sites of recurrence were in the cranial base or adjacent paranasal sinuses. The time to recurrence varied from 1 to 12 years (average, 7 yr; mean, 8 yr). Three patients had undergone one previous resection, two had undergone two previous resections, and one had undergone four previous operations. The ethmoid sinus was involved in all cases of recurrence, either with the sphenoid sinus or with an intracranial recurrence. Thirteen patients underwent complete resection of underlying bone and the invaded paranasal sinuses, then reconstruction of the anterior fossa. No patient died. There were three instances of cerebrospinal fluid leakage (one requiring operative repair), one case of delayed worsening vision after initial improvement, and two cases of transient cranial nerve palsy (Cranial Nerves III and IV). There was no recurrence at follow-up (average, 3.7 yr; range, 1-7.3 yr). CONCLUSION: The cranial base and paranasal sinuses are sites of predilection for recurrence of olfactory groove meningiomas. Recurrence is the result of a direct extension attributable to incomplete resection of involved bone and regrowth at the edge of a previous surgical field. Extensive resection of all suspicious underlying bone is a complement to radical removal of these lesions. Reconstruction with a vascularized pericranial flap to prevent cerebrospinal fluid leakage is crucial.     

Factors leading to local recurrence or death after surgical resection of phyllodes tumours of the breast

BACKGROUND: Local recurrence and death from metastases are occasional, but consistent, themes in reports of patients with phyllodes tumours. Factors that might contribute to these outcomes were sought. METHODS: Data from 38 patients with a phyllodes breast tumour were reviewed retrospectively, reclassifying the pathological material using the Pietruszka and Barnes criteria. RESULTS: At a median of 12 months, nine patients had developed a local recurrence and four had died from metastases. Following local excision in 24 patients (for diagnosis in 13, for 'fibroadenoma' in nine and for phyllodes tumour in two patients), 13 had no further surgery and five had local recurrence (three of eight benign tumours, two of two malignant tumours). Wide local excision or mastectomy in 18 patients was followed by four recurrences (one of eight borderline tumours, three of ten malignant tumours). All patients with recurrence had margin involvement on histological examination, but not all patients with margin involvement developed recurrence. Lack of statistical correlation between local recurrence and age, delay, size, grade or type of surgery was confounded by selection bias for more extensive surgery for malignant tumours. Death correlated with size (P = 0.05) and grade (P = 0.03) of tumour. CONCLUSION: Inadequate preoperative diagnosis ('fibroadenoma' or failure of triple assessment) frequently led to local excision with positive margins. Without revision this often resulted in local recurrence. Local recurrence of any grade was usually followed by further recurrence. Death was related to tumour size and histological grade, confirming these as prognostic factors.     

Biology and technology in the surgical treatment of malignant bone tumours in children and adolescents,with a special note on the very young

PurposeThe main challenge in reconstruction after malignant bone tumour resection in young children remains how and when growth-plates can be preserved and which options remain if impossible.MethodsWe describe different strategies to assure best possible long-term function for young children undergoing resection of malignant bone tumours.ResultsDifferent resources are available to treat children with malignant bones tumours: a) preoperative planning simulates scenarios for tumour resection and limb reconstruction, facilitating decision-making for surgical and reconstructive techniques in individual patients; b) allograft reconstruction offers bone-stock preservation for future needs. Most allografts are intact at long-term follow-up, but limb-length inequalities and corrective/revision surgery are common in young patients; c) free vascularized fibula can be used as stand-alone reconstruction, vascularized augmentation of structural allograft or devitalized autograft. Longitudinal growth and joint remodelling potential can be preserved, if transferred with vascularized proximal physis; d) epiphysiolysis before resection with continuous physeal distraction provides safe resection margins and maintains growth-plate and epiphysis; e) 3D printing may facilitate joint salvage by reconstruction with patient-specific instruments. Very short stems can be created for fixation in (epi-)metaphysis, preserving native joints; f) growing endoprosthesis can provide for remaining growth after resection of epi-metaphyseal tumours. At ten-year follow-up, limb survival was 89%, but multiple surgeries are often required; g) rotationplasty and amputation should be considered if limb salvage is impossible and/or would result in decreased function and quality of life.ConclusionSeveral biological and technological reconstruction options must be merged and used to yield best outcomes when treating young children with malignant bone tumours.Level of EvidenceLevel V Expert opinion     

Phyllodes tumours of the breast--a retrospective study from 1982-2000 of 50 cases in Portsmouth

INTRODUCTION: This study is a retrospective analysis of 50 phyllodes tumours to determine the optimal surgical procedure for these types of tumours. We have also reviewed rates of recurrence, metastases and mortality based on choice of procedure and histological type. PATIENTS AND METHODS: Cases were ascertained from pathology databases and clinical details extracted from the hospital records. Fifty patients with phyllodes tumours were identified. These comprised 29 benign, 12 borderline and 9 malignant phyllodes tumours. RESULTS: All benign phyllodes tumours were treated with breast-conserving surgery, these included 16 tumours over 40 mm. Borderline and malignant lesions were treated by breast-conserving surgery or mastectomy. The median follow-up period was 35 months (range, 4-96 months). The recurrence rate for all tumours was 14%. Malignant and borderline phyllodes tumours had a recurrence rate of 28%. Tumours excised with a wide margin did not seem to recur. Breast-conserving surgery appeared to be as effective as mastectomy. The choice of procedure was less important than the width of the excision margin. Recurrence occurred in 1/29 benign tumours. Excision margin width did not influence rate of recurrence. One patient died of metastases after mastectomy. CONCLUSIONS: Breast-conserving surgery is the treatment of choice for all benign lesions. For borderline and malignant lesions, excision with a wide margin reduces the rate of recurrence. If a diagnostic local excision biopsy or enucleation is performed, it should be followed by a definitive wider excision.     

Uncommitted gastrointestinal stromal tumour. Case report

Gastrointestinal Stromal Tumours (GIST) are mesenchymal tumours with uncertain prognosis. Malignant variety represents about 2.0% of malignant gastroenteric tumours. The Authors report a clinical case of malignant gastric and duodenojejunal GIST, in which the only surgical treatment seems to be definitive. R. S., a 69-year-old female, was admitted for asthenia and fever in January 1997. Endoscopic exploration, ultrasonography and CT-scan of the abdomen demonstrated an exophytic tumour in the greater gastric curvature and one tumour of 5.5 cm of diameter in the Treitz's angle. We performed a resection of the gastric tumour and the duodenojejunal angle. Postoperative course was uneventful and the patient was discharged after 14 postoperative hospital days. Histological analysis showed two spindle cells stromal tumours with mitotic rate > 20/10 HPF. The immunohistochemistry demonstrated the uncommitted origin of tumour cells. The patient refused the chemotherapy treatment. There was no local recurrence or metastasis at a follow up of 47 months, in spite the high malignancy degree. For this reason and because of the uncertain behaviour of benign GIST, the authors propose a lifelong follow up of the patients managed with potentially curative surgical resection.     

An approach to malignant parotid tumours

A series of 30 patients with malignant parotid tumours selected for surgical treatment is presented. There were 18 males and 12 females, with an age range of 12-80 years. All presented with a parotid lump, one had an associated painful facial nerve palsy and one had clinically involved neck nodes. Four had previous surgery, surgical biopsy or radiotherapy. Pre-operative fine needle aspiration was performed on one patient. Postoperative radiotherapy was administered to 14 patients. Follow-up was for a mean of 5.5 years. The primary tumour was controlled by surgery alone in 15 of the 16 patients. There was one local recurrence and two patients in this group died from distant metastases. Of the group receiving postoperative radiotherapy, there was one local recurrence, one local and neck recurrence and one death from distant metastases. Mobile, discrete tumours can be treated by parotidectomy with preservation of the facial nerve and the selective use of radiotherapy. The malignant tumour with restricted mobility but no facial palsy is treated by parotidectomy, sacrificing only involved branches and postoperative radiotherapy. The clinically fixed lesions with facial palsy demand total parotidectomy in operable cases, nerve sacrifice and postoperative radiotherapy.     

Surgical treatment possibilities in tumors of the spine. Indications, technics, experiences

Widely normal stability of the vertebral column with adequate load-bearing capacity should be achieved, following osseous reconstruction of a defect area from which benign tumours or tumour-like lesions had been removed by radical surgery. In malignant processes, surgical tumour removal may add to the effectiveness of chemotherapy or radiotherapy. Postoperative restoration of the vertebral column will also improve the psychic, physical, and social quality of survival.     

Association between intracranial plasmacytoma and multiple myeloma: clinicopathological outcome study

OBJECTIVE: Intracranial plasmacytomas are rare lesions that can arise from the calvarium, dura, or cranial base and exhibit a benign course unless associated with myeloma. Attention has recently been focused on the role of the cell adhesion molecules CD56 and CD31 in the pathogenesis of myeloma. No such information is available for intracranial plasmacytomas and myeloma-associated lesions. METHODS: We investigated the relationship between CD56 and CD31 expression, intracranial location, and progression to myeloma for a series of nine intracranial plasmacytomas (three dural, one calvarial, and five cranial base lesions). These parameters were also correlated with proliferation indices, as assessed by MIB-1 immunostaining of the histological sections. A single pathologist (AO) performed immunohistochemical analyses and reviewed all slides. RESULTS: Intracranial plasmacytomas presented more commonly in female patients (89%). The three dural lesions were CD56- and CD31-negative and exhibited MIB-1 staining of less than 10%; no patient developed myeloma or recurrence. Of the five cranial base lesions, three were CD56-positive, none was CD31-positive, and two exhibited MIB-1 labeling of more than 45%, with plasmablastic morphological features. Compared with other intracranial plasmacytomas, five of five patients with cranial base lesions developed bone marrow biopsy-proven myeloma (P < 0.05) within 8 months. The calvarial lesion was CD56- and CD31-positive, and the patient developed myeloma soon after diagnosis. Both of the two highly proliferative plasmablastic lesions recurred, one after gross total resection without radiotherapy and the other after a biopsy and 2000-cGy radiotherapy. CONCLUSION: Among intracranial plasmacytomas, cranial base location was the strongest predictor of the development of multiple myeloma. Expression of the cell adhesion molecules CD31 and CD56 was not predictive of outcome. Extramedullary dural-based lesions were CD56-negative and were not associated with myeloma. A high proliferation index and plasmablastic morphological features were predictive of a short time to recurrence and aggressive behavior. We recommend 4050- to 5040-cGy fractionated radiotherapy for all intracranial plasma cell neoplasms and gross total resection for non-cranial base lesions.