Giant cell tumor of bone – Analysis of 213 cases involving extra-craniofacial bones

We elucidated clinicopathological characteristics of giant cell tumor of bone (GCTB) in Japan, and significant clinicopathological factors for predicting local recurrence. Clinicopathological profiles of 213 patients with GCTB (100 male, 113 female) involving extra-craniofacial bones were retrieved. Pathological slides obtained at the initial surgery were reviewed. Fourteen pathological and five clinical features were statistically analyzed to disclose prognostic significance. Patient age ranged from 12–80 years (Average 38.7). Long bones were most frequently affected (86.4%), especially around the knee (62.9%). Histological features are basically similar to those previously reported. Within a follow-up period (24–316 months, average 106.1 months), the local recurrence rate is 29.1%. Metastasis has occurred in 9 patients. Cox regression analysis of representative clinicopathological features shows that younger age, higher mitotic count, smaller zones of stromal hemorrhage, considerable vascular invasion and absence of ischemic necrosis are significant predictors for local recurrence. Initial operative method (curettage) is a significant risk factor in univariate analysis but not by multivariate analysis (P = 0.053). Denosumab administration increases risk but not significantly (P = 0.053). Histone 3.3 G34W immunopositivity is not significant for predicting local recurrence.     

Primary central chondrosarcoma of long bone,limb girdle and trunk: Analysis of 174 cases by numerical scoring on histology

The aims of this study were: (i) to elucidate clinicopathological characteristics of pcCHS of long bones (L), limb girdles (LG) and trunk (T) in Japan; (ii) to investigate predictive pathological findings for outcome of pcCHS of L, LG and T, objectively; and (iii) to elucidate a discrepancy of grade between biopsy and resected specimens. Clinicopathological profiles of 174 pcCHS (79 male, 95 female), of L, LG, and T were retrieved. For each case, a numerical score was given to 18 pathological findings. The average age was 50.5 years (15–80 years). Frequently involved sites were femur, humerus, pelvis and rib. The 5‐year and 10‐year disease‐specific survival (DSS) rates [follow‐up: 1–258 months (average 65.5)] were 87.0% and 80.4%, respectively. By Cox hazards analysis on pathological findings, age, sex and location, histologically higher grade and older age were unfavorable predictors, and calcification was a favorable predictor in DSS. The histological grade of resected specimen was higher than that of biopsy in 37.7% (26/69 cases). In conclusion, higher histological grade and older age were predictors for poor, but calcification was for good prognosis. Because there was a discrepancy in grade between biopsy and resected specimens, comprehensive evaluation is necessary before definitive operation for pcCHS.     

Impact of older age on clinicopathological and prognostic features of colon cancer in postmenopausal women

To clarify the clinicopathological features of colorectal cancer in older people, systematic studies considering age, sex, and the tumor locus is needed. We focused on colon cancer in postmenopausal women (<70 years, n = 68 vs. ≥70 years, n = 85), and examined the effect of age on clinicopathological features. Rates of medullary carcinoma /mucinous carcinoma were higher and pathological stages at diagnosis were less advanced in patients ≥70 years compared with <70 years. Matching pathological stages, no significant difference in disease‐free interval was observed according to age; however, disease‐specific survival (DSS) was poorer in patients ≥70 years than <70 years, being significantly different in stage IV cases. Regarding post‐metastasis/recurrence (met/rec) cases, chemotherapy and surgery for metastasis were less frequent in those aged ≥70 years than <70 years. Post‐met/rec DSS was poorer in ≥70 years, those with microsatellite instability, and those without surgery for met/rec than in each counterpart; however, post‐met/rec chemotherapy exhibited no effect. Multivariate analyses revealed that an older age and no surgery for metastasis were independent predictors of disease‐specific death. These findings remained after excluding stage IV cases. Older age was a potent risk factor of rapid disease‐specific death after met/rec.     

Different subtypes of epithelioid sarcoma and their clinical implication: long‐term multi‐institutional experience with a rare sarcoma

Epithelioid sarcoma (ES) is a very rare soft‐tissue sarcoma with a high tendency of recurrence and metastasis. We analyzed clinical features of ES and aimed to identify the potential role of radio‐ and chemotherapy in ES. Fifty‐five patients diagnosed with ES between 1997 and 2014 were enrolled from seven tertiary hospitals in Korean Cancer Research Group. The clinical variables were retrospectively reviewed and analyzed. Forty‐six (84%) patients underwent surgical resection of ES, and among them, 27 experienced recurrence. In these patients, resection margin status and adjuvant radiotherapy were independent prognostic factors for longer recurrence‐free survival (RFS), while adjuvant chemotherapy did not influence RFS. Twenty‐two (40%) patients received palliative chemotherapy for metastatic or recurrent ES, and in these patients, palliative chemotherapy was the only independent prognostic factor for longer overall survival. Intriguingly, the clinical benefit of radio‐ and chemotherapy was observable only in proximal ES, but not in extremity ES, indicating that subtypes of ES might respond to radio‐ or chemotherapy differently. Proximal ES seems to benefits more from active anticancer treatment than conventional extremity ES. The aggressive characteristics of proximal ES could be overcome with an optimal multimodal treatment.     

Dedifferentiated leiomyosarcoma: clinicopathological analysis of 18 cases

Chen E, O’Connell F & Fletcher C D M
(2011) Histopathology  59 , 1135–1143
Dedifferentiated leiomyosarcoma: clinicopathological analysis of 18 cases Aims: To clinicopathologically characterize the dedifferentiated variant of leiomyosarcoma in a series of 18 cases. Methods and results: Dedifferentiated leiomyosarcoma was defined as showing features of low‐grade leiomyosarcoma associated with a discrete undifferentiated component lacking morphological or immunophenotypic features of myogenic differentiation. Tumours developed in 11 women and seven men, with an age range of 16–84 years (median, 64 years). Sites were retroperitoneum (eight cases), limbs (four), trunk (two) uterus (two), and paratesticular and prostate (one each). In 17 cases, dedifferentiation occurred de novo in the primary tumour. Tumour size ranged from 50 to 280 mm (median: 120 mm). Histologically, most showed discrete transition from well‐differentiated smooth muscle morphology to high‐grade pleomorphic morphology with no smooth muscle differentiation. Unusual features in the dedifferentiated component (epithelioid and rhabdomyoblast‐like morphology) were present in three cases. Heterologous osseous or chondro‐osseous elements were present in two cases. Dedifferentiated areas were negative for myogenic markers in all cases. Follow‐up for 13 cases (median, 36 months) showed local recurrence in 38% (5/13). So far, three patients have died of disease (median survival, 8 months), and metastasis developed in five of 13 cases. Conclusions: Dedifferentiated leiomyosarcoma has morphological parallels with other types of dedifferentiated sarcoma, and is clinically aggressive.     

浅表性平滑肌肉瘤临床病理分析

目的:探讨浅表性平滑肌肉瘤(superficial leiomyosarcoma,SLMS)的临床病理特点、诊断、鉴别诊断及治疗和预后.方法:回顾性分析13例SLMS的临床病理特征,并复习相关文献讨论.结果:男7例,女6例,平均年龄59岁.肿瘤位于四肢7例,躯干3例,头面部2例,阴囊1例,其中位于真皮的9例,皮下4例.肿瘤直径1~8(平均4.3)cm.局部复发5例,无远处转移病例.镜下:肿瘤呈结节状或弥漫状生长,根据肿瘤细胞不同分化分为三型:中分化(最多见,9例)、高分化和低分化型(较少,各2例).免疫组织化学:肿瘤细胞desmin,MSA,a-SMA和h-caldesmon阳性.结论:SLMS多发生于中老年人,预后较好,易发生局部复发,极少发生远处转移.治疗采用局部扩大切除.     

Differential diagnosis of complex renal cysts based on lesion size along with the Bosniak renal cyst classification

Purpose

To identify size criteria for complex cystic renal masses that can distinguish renal cell carcinoma from benign cysts supplementing the Bosniak classification.

Materials and Methods

We reviewed the records of 97 patients who underwent surgery for complex cystic renal masses from January 2001 to April 2010. The pathological results were compared with the lesion sizes measured by preoperative computed tomography and other radiological features (contrast enhancement, irregularities of cyst walls and septa, and calcification) were also obtained for categorization according to the Bosniak renal cyst classification.

Results

Malignancy was significantly associated with cyst size (>2 cm), male gender, and younger patient age (<50 years). According to the Bosniak classification, there was no category I cyst, and all 8 category II cysts were benign. However, 3 of 18 (17%) category IIF cysts, 21 of 39 (54%) category III cysts, and 29 of 32 (90%) category IV cysts were malignant. All category IIF cysts were benign in patients older than 50 years of age.

Conclusion

Many complex cystic renal masses smaller than 2 cm were benign. We suggest that lesion size should be taken into account when formulating treatment plans for complex cystic renal masses.     

Eosinophilia in bronchoalveolar lavage fluid and architectural destruction are features of desquamative interstitial pneumonia

Aims:  Desquamative interstitial pneumonia (DIP) is a rare pattern of diffuse parenchymal lung disease known to overlap with respiratory bronchiolitis-interstitial lung disease (RB-ILD). The aim was to review biopsy-proven cases of DIP to investigate further the clinical, imaging and histological features of this disease.
Methods and results:  Twenty patients fulfilled the pathological criteria: 19 men and one woman with a mean age of 54 years. Clinical features, bronchoalveolar lavage (BAL) data, radiological findings, pathological findings other than criteria, effect of therapy and outcome were examined. The BAL data for 17 cases revealed marked eosinophilia (mean 18%) and moderate neutrophilia (mean 11%). Computed tomography in 17 patients showed peripheral involvement in all cases with a clear margin in 64% and thin-walled cysts in 35% of cases. Additional pathological features were a distinct lobular distribution (70%) and architectural destruction (70%) with cyst formation (55%). Eighteen of the 19 patients (95%) improved under steroid pulse and/or oral therapy. Sixteen subjects (80%) are alive, three died of other diseases and one died of DIP 74 months after the diagnosis. Percent vital capacity increased significantly and new thin-walled cysts appeared in one case.
Conclusions:  BAL eosinophilia, lobular distribution and architectural destruction with cyst formation are characteristic features of DIP.     

Centrally necrotizing carcinoma of the breast: clinicopathological analysis of 33 cases indicating its basal‐like phenotype and poor prognosis

Yu L, Yang W, Cai X, Shi D, Fan Y & Lu H
(2010) Histopathology 57 , 193–201 Centrally necrotizing carcinoma of the breast: clinicopathological analysis of 33 cases indicating its basal‐like phenotype and poor prognosis Aims: To investigate the clinicopathological features and immunophenotype of centrally necrotizing carcinoma (CNC) of the breast to ascertain its relationship to basal‐like phenotype and its prognosis. Methods and results: The clinical and pathological characteristics of 33 CNCs were reviewed. Immunohis‐tochemical study of oestrogen receptor, progesterone receptor, HER2, cytokeratin (CK) 8/18, high‐molecular‐weight CK (34βE12), CK5/6, CK14, CK17, smooth muscle antigen, p63, vimentin and epidermal growth factor receptor was performed. The striking feature of CNC was a central, necrotic or acellular zone surrounded by a ring‐like area of viable tumour cells. The central zone showed three morphological types: predominance of coagulative necrosis (21 cases), predominance of fibrosis and scar tissue (nine cases) and infarction (three cases). Tumour cells displayed invasive ductal carcinoma of high grade. The expression rate of basal‐like markers was higher than that of myoepithelial markers (87.9% versus 46.2%). Basal‐like subtype was shown by 63.6% of cases. The expression rate of CK5/6 (90.5%) was highest among basal‐like markers. Follow‐up data of 19 patients were available. Median progression‐free survival was 15.5 months. In 12 patients (63.2%), local recurrence and/or distant metastasis developed (median time to recurrence and/or metastasis, 14.0 months). Conclusions: CNC has distinctive morphological features, which mostly exhibit a basal‐like immunophenotype and poor prognosis. CNC is a typical representative of basal‐like breast cancer.     

KiSS‐1 expression in oral squamous cell carcinoma and its prognostic significance

Downregulated expression of KiSS‐1 has been correlated with tumor progression, metastasis, and patient prognosis in various human malignancies. However, there is no information regarding the expression of KiSS‐1 in oral squamous cell carcinoma (OSCC). Our aims were to examine KiSS‐1 expression in OSCC tissue samples and cell lines and to determine its prognostic significance. KiSS‐1 expression was significantly lower in lymph node (LN) metastases than in primary tumor tissues. Five of six OSCC cell lines showed absence or relatively low expression of KiSS‐1. Correlations between KiSS‐1 expression and clinicopathological parameters were statistically assessed. There were significant correlations between KiSS‐1 expression and LN metastasis (p = 0.007), TNM stage (p = 0.024), and local recurrence (p = 0.012). In the Kaplan–Meier survival analysis, negative KiSS‐1 expression significantly correlated with poorer overall survival (OS) and disease‐free survival (DFS) (p = 0.000 and 0.000, respectively). Multivariate analysis using Cox regression modeling revealed that KiSS‐1 expression was an independent prognostic factor for both OS and DFS (p = 0.001 and 0.000, respectively). Our findings suggested that KiSS‐1 downregulation may play a role in tumor progression and metastasis of OSCC and may be a reliable biomarker for predicting clinical outcome in OSCC.     

Surgical treatment of recurrent proximal tibio-fibular joint ganglion cysts

BackgroundComplex symptomatic ganglion cysts arising from the proximal tibio-fibular joint (PTJ) are not an uncommon presentation in specialist knee clinics and can be managed by aspiration or excision. There is, however, a high rate of recurrence and often there is progressive involvement of the common peroneal nerve (CPN) and its branches, and permanent nerve damage may result.MethodsThis study is a review of the outcome of recalcitrant and recurrent cyst disease with CPN involvement treated by proximal fibulectomy. Nine patients with clinical and radiological diagnosis of a ganglion cyst involving the proximal tibio-fibular joint were treated by proximal fibulectomy. Average age was 47.2 years (19 to 75). Patients were followed up clinically and radiologically. Medical notes were reviewed to assess clinical/pathological characteristics, surgical outcome, recurrence rate and the symptoms of instability and nerve function.ResultsNone of the patients were lost to follow-up. After an average follow-up of 83 months (15 to 150), none of the patients had clinical or radiological evidence of recurrence. All patients were pain-free and had a complete resolution of nerve symptoms and no evidence of CPN injury. None of the patients complained of localised pain or knee instability and there were no wound healing problems.ConclusionsMRI now confirms TFJ-ganglion cysts to be more common than previously recognised. Where there is refractory disease with progressive nerve symptoms and evidence of nerve sheath involvement, joint excision by proximal fibulectomy gives a satisfactory functional result in controlling disease and preventing further nerve damage.Level of evidenceIV     

Fine‐needle aspiration of dermatofibrosarcoma protuberans metastasizing to hemithorax with superior vena cava compression: Case report and literature review

Dermatofibrosarcoma protuberans (DFSP) is a low‐grade spindle cell tumor of the skin commonly arising on the trunk and extremities which tends to be slow growing yet locally aggressive. DFSPs are associated with a good prognosis when surgical excision with negative margins is achieved. Although local recurrences occur up to 50% of incompletely resected cases, distant metastases are very rare. Here, we report a case of DFSP metastasizing to the right hemithorax diagnosed by an endobronchial ultrasound‐guided fine‐needle aspiration (FNA) 9 years after initial presentation. The aspirate showed a bland spindle cell proliferation that was morphologically similar to the original skin excision; the storiform pattern was particularly prominent in tumor‐tissue fragments in the cellblock. Immunostaining showed strong, diffuse positivity for CD34. Molecular studies demonstrated a characteristic COL1A1/PDGFB fusion in both original and metastatic specimens. A review of the literature revealed that metastatic DFSP most often involves the lungs, occurs usually in cases with fibrosarcomatous transformation and after a local recurrence, and presents on average 4.5 years after the original diagnosis. This case did not show fibrosarcomatous transformation or local recurrence prior to metastasis 9 years later. In summary, it is important to consider the potential for metastases years after a nonrecurring primary DFSP, despite its rarity. Cytologic features when complemented by ancillary studies and awareness of the patient's prior clinical history permit a confident diagnosis of metastatic DFSP by FNA. In addition, by confirming the characteristic translocation, tyrosine‐kinase inhibitor imatinib can provide additional treatment options for unresectable metastatic DFSP.     

Mitotic Index is an Independent Predictor of Recurrence‐Free Survival in Meningioma

While World Health Organization (WHO) grading of meningioma stratifies patients according to recurrence risk overall, there is substantial within‐grade heterogeneity with respect to recurrence‐free survival (RFS). Most meningiomas are graded according to mitotic counts per unit area on hematoxylin and eosin sections, a method potentially confounded by tumor cellularity, as well as potential limitations of accurate mitotic figure detection on routine histology. To refine mitotic figure assessment, we evaluated 363 meningiomas with phospho‐histone H3 (Ser10) and determined the mitotic index (number of mitoses per 1000 tumor cells). The median mitotic indices among WHO grade I (n = 268), grade II (n = 84) and grade III (n = 11) tumors were 1, 4 and 12. Classification and regression tree analysis to categorize cut‐offs identified three subgroups defined by mitotic indices of 0–2, 3–4 and ≥5, which on univariate analysis were associated with RFS (P < 0.01). In multivariate analysis, mitotic index subgrouped in this manner was significantly associated with RFS (P < 0.01) after adjustment for Simpson grade, WHO grade and MIB‐1 index. Mitotic index was then examined within individual WHO grade, showing that for grade I and grade II meningiomas, mitotic index can add additional information to RFS risk. The results suggest that the use of a robust mitotic marker in meningioma could refine risk stratification.     

Defining the width of the normal tibial plateau relative to the distal femur: Critical normative data for identifying pathologic widening in tibial plateau fractures

Tibial plateau widening in the setting of fracture is an indication for surgical treatment, and restoring width is an important goal of surgery. In order to identify and correct pathological widening, the width of the normal tibial plateau must first be defined. The aim of this study was to establish normative data for the width of the tibial plateau relative to the distal femur to enable surgeons to identify and correct pathological widening in the setting of tibial plateau fracture. Fifty‐one uninjured anteroposterior (AP) knee radiographs and 11 XR and CT scans of lateral tibial plateau fractures were retrospectively reviewed. The distances measured included maximal distal femoral width, femoral articular width, tibial articular width, and lateral plateau widening. On average, lateral plateau widening was +0.02 ± 2.03 mm, indicating that the most lateral aspect of the tibial plateau is collinear with the most lateral aspect of the lateral epicondyle of the femur. In the fracture population, average widening was 7.13 ± 3.59 mm on XR and 6.57 ± 3.34 mm on CT, with an absolute difference between XR and CT of 1.19 ± 0.66 mm. In conclusion, this study is the first to define the radiographic anatomy of the proximal tibia quantitatively. In the setting of tibial plateau fracture, residual widening of 2.1 mm could be within normal variation. However, the authors consider widening >2.1 mm pathological. These values can be used for assessing pathological widening of tibial plateau fractures. Clin. Anat. 31:688–692, 2018. © 2018 Wiley Periodicals, Inc.     

Clinical Strategy for the Management of Solid Pseudopapillary Tumor of the Pancreas: Aggressive or Less?

Objective: To further delineate the clinicopathological and radiological features of solid pseudopapillary tumor (SPT) of the pancreas and summarize the surgical therapy strategy for this tumor. Methods: A retrospective review of 18 pathologically confirmed cases of SPT was performed and the clinical and pathological features, radiological findings and surgical interventions were analyzed. Results: The patients included 17 females and 1 male with a median age of 23 years. The median diameter of the lesions was 8.0 cm. Abdominal pain was the predominant complaint (8/18). The rest of the patients were asymptomatic and presented with a pancreatic mass detected incidentally. Radiological study revealed a well-demarcated mass which was composed of a solid-cystic portion. On post-contrast CT, the solid portions could be enhanced whereas the cystic parts remained unenhanced. With the preoperative diagnosis of SPT in 11 patients and pancreatic cyst, benign or malignant pancreatic tumor in the rest, pancreatic tumor resection was successfully completed. Surgical exploration findings, pathological characteristics and good prognosis of the patients with SPT, indicated its low-grade malignant potential. Conclusion: In combination with clinical findings, radiological features of SPT may help to make the correct diagnosis and differentiation from other pancreatic neoplasms. Once diagnosed, given the excellent prognosis and low-grade malignancy, less aggressive surgical resection of the primary lesion is proposed.     

背阔肌皮瓣联合假体在乳腺癌改良根治术后乳房重建中的应用

目的 研究背阔肌皮瓣联合假体在乳腺癌改良根治术后乳房重建中的应用。方法 选取2013年6月~2017年6月本院收治的100例乳腺癌患者作为研究对象,通过抓阄法随机分为对照组和观察组,每组50例,其中对照组采用传统治疗方式,观察组患者采用背阔肌皮瓣联合假体治疗方式,使用焦虑评价量表（ASA）评价患者焦虑情况,比较两组患者对体型、社交方面的满意度,复发、转移和生存情况。结果 观察组对术后焦虑情况、体型、社交方面的满意度分别为24.00%、50.00%、90.00%均优于对照组的86.00%、6.00%、2     

Fixation of femoral shaft osteotomy with an intramedullary composite rod: an experimental study on dogs with a two-year follow-up

A novel composite material with an ultra-high strength and a low elastic modulus called carbon fibre-reinforced liquid crystalline polymer (LCP/CF) has been developed. An experimental diaphyseal osteotomy of the proximal femur in fourteen Beagle dogs was fixed with an intramedullary LCP/CF rod of 4.5 mm in diameter and 80 mm in length. The radiological follow-up intervals were 1, 3, 6, 12, 24, 52, and 104 weeks. Five dogs were killed at 1 year and three dogs at 2 years for histological studies; six dogs were retained for longer follow-up. Radiographs showed an uncomplicated healing of the diaphyseal osteotomy with an external callus formation in all dogs in 12 weeks. Histological analysis revealed a benign host tissue response with few inflammatory cells. Both bone and fibrous tissue were seen at the LCP/CF-host tissue interface. The cross-sectional cortical area of the operated femur was slightly greater than that of the control femur in the 2-year follow-up. LCP/CF showed promising properties for high-load applications.     

15例原发性胃肠外来源胃肠间质瘤的临床病理特征及预后分析并文献复习

目的　探讨原发性胃肠外来源胃肠间质瘤（extragastrointestinal stromal tumors,EGISTs）的临床病理特征及预后影响因素,并复习相关文献。 方法　回顾性收集2006年1月~2017年8月广东省人民医院收治的原发性EGISTs临床资料,并进行描述性分析。 结果　共收集到15例原发性EGISTs,所有病例经病理确诊。其中男性11例,女性4例;中位发病年龄为45岁（33~70岁）。首发症状多为腹盆腔包块或腹痛。原发部位中,有4例位于腹膜后,4例位于结肠系膜,3例位于小肠系膜,1例位于盆腔,1例位于左侧胸膜腔,1例位于胰腺被膜,1例位于小网膜囊。中位随访时间为53个月（1~102个月）。免疫组化显示,14例CD117表达阳性（14/15）,10例CD34表达阳性（10/15）,6例患者DOG-1表达阳性（6/8）。有7例患者接受基因检测,2例c-KIT基因外显子9突变,2例c-KIT基因外显子11突变,1例PDGFRa基因外显子12同义突变,2例未检测到c-KIT基因及PDGFRa基因突变。依据改良NIH（National Institutes of Health）标准进行危险度分级,5例高危病例行R0术后接受伊马替尼靶向治疗,平均生存期为67个月;高危患者中8例未行辅助靶向治疗,平均生存期为36.58个月。有8例在随访过程中出现复发转移,8例出现肿瘤相关性死亡。 结论　EGISTs发病率低,症状多为腹盆腔包块,预后差;其病理特征与常见的胃肠来源的GISTs相仿。R0手术切除是治疗首选,伊马替尼辅助治疗有利于改善预后并预防复发转移。     

Chondroblastoma of bone--not a " benign disease": clinico-pathologic observations on sixty cases

Chondroblastomas (CBL) account for less than 1% of all bone tumors. A 46 year review (1955-2000) of bone tumors yielded 60 cases of CBL. This paper reviews their histology, radiology, clinical presentation and emphasizes that this seemingly benign lesion is capable of aggressive behaviour. Histopathology slides and radiographs of sixty patients were studied and clinical details and follow-up were obtained. There were 44 males and 16 females, aged between 10 to 30 years. Long bones were involved in 81.4% of the cases, tibia being the commonest site. Symptoms comprised pain (60%), local swelling (25%) and joint symptoms (5%) or a combination of these. Radiologically, an oval sharply demarcated lytic lesion predominantly in the epiphysis was noted in 50% of the cases. On microscopy all lesions revealed chondroblasts having irregular grooved nuclei and osteoclasts with 80% of the tumors showing chondroid differentiation and calcification. Treatment comprised curetting (34 cases), wide excision (21 cases), radical excision (1 case) and was unknown in 4 cases. Follow-up ranging from 2 to 26 years was available in 83.3% of the patients; six (10%) had local recurrence (at 6 months, 8 months, 1 year, 2 years, 3 years and 5 years), two (3.3%) developed lung metastasis (synchronous in one and at 18 months in the other) and spontaneous malignant transformation occurred in one (1.4%) patient after 9 years; subsequent to three local recurrences. CBL, though seemingly benign, can behave in an aggressive manner. Since there are no reliable histological features to predict biologic behaviour, regular long term follow-up is recommended.     

Cotyledonoid dissecting leiomyoma of the uterus: a review of clinical, pathological, and radiological features

Cotyledonoid dissecting leiomyoma is a rare benign uterine tumor whose gross and radiological appearance may raise the possibility of a malignancy. The authors summarize herein the clinical, radiological, and pathological features of the 41 previously reported cases. The patients typically presented with menorrhagia or symptoms that were ultimately relatable to the presence of a pelvic mass. The median patient age was 46 years (range 23-73). The average tumor size was 15.4 cm (range 4-41); most were exophytic, multinodular, occasionally cystic masses with a congested, spleen or placenta-like color, protruding over the uterine serosa and, variably, the broad ligaments and adjacent organs. In most cases, the exophytic component was contiguous with the intramural dissecting leiomyomatous components. Histological features suggestive of malignancy, such as cytological atypia, necrosis, or increased mitotic activity were absent. Intravascular growth, as assessed histologically, was present in 20% of reported cases, and this finding is apparently devoid of clinical significance based on limited data. Reported information on radiological features is limited. However, these lesions are typically isointense to myometrium on T1-weighted MRI and are in general less heterogeneous on T2 and postcontrast-imaging than sarcomatous lesions. The tumor may display extension to but not frank invasion of surrounding organs. Ultrasound features are nonspecific. Follow-up information was available in 25 (61%) of 41 reported patients: none experienced a tumor recurrence or metastases during the follow-up period, which ranged from 1 month to 41 years. This affirms the benign nature of this tumor, its alarming gross appearance and growth patterns notwithstanding.