Benign metastasizing leiomyoma: metastasis to rib and vertebra

Benign metastasizing leiomyoma is very rare and characterized by the presence of pelvic, peritoneal, nodal, or pulmonary nodules in women with a history of uterine leiomyomas. We report a case of benign metastasizing leiomyoma in a 30-year-old woman who had undergone a prior myomectomy due to uterine cellular leiomyoma 3 years earlier. The patient had a mass on the right sixth rib and 2 masses in the sixth thoracic vertebra. Pathologically, these masses were diagnosed as cellular leiomyomas. Estrogen and progesterone receptors were both positive in the metastatic tumors as well as in the uterine leiomyomas. The diagnosis of benign metastasizing leiomyoma can only be made after careful examination of the primary tumor to exclude small foci of malignant change.     

Analysis of allelic loss as an adjuvant tool in evaluation of malignancy in uterine smooth muscle tumors

Uterine smooth muscle tumors of uncertain malignant potential (STUMPs) are difficult both from the diagnostic and patient management standpoint because they cannot be classified as benign or malignant by conventional histologic criteria. This study's aim was to determine the diagnostic utility of allelic imbalance (AI) analysis in uterine smooth muscle tumors. Using microdissection and genotyping, we tested 5 leiomyomas, 6 STUMPs, and 10 leiomyosarcomas with follow-up for AI across a panel of seven tumor suppressor genes (p16, p21, p53, VHL, XRCC3, RB, and NM-23). None of the 6 patients with STUMP experienced recurrent disease, whereas 8 of the 10 patients diagnosed with leiomyosarcoma died of disease at follow-up. The mean frequency of allelic loss (FAL) for leiomyomas (18%) was not significantly different from that of STUMPs (21%) (P = 1), whereas leiomyosarcomas displayed a significantly higher FAL (52%) than both leiomyomas (P = 0.001) and STUMPs (P = 0.002). Loss of NM-23, a reported tumor metastasis suppressor gene, was found only in leiomyosarcomas (5 of 9, or 56%), and 4 of 5 (80%) of these were the only cases that demonstrated distant metastases (P = 0.04). Additionally, an FAL of >50% correlated with both NM-23 loss (P = 0.008) and distant metastatic disease (P = 0.04). In conclusion, leiomyomas and STUMPs displayed similar mean FALs and all were clinically benign, whereas uterine leiomyosarcomas had significantly higher frequencies of allelic loss than both leiomyomas and STUMPs. Molecular profiling may thus provide a valuable tool in assessment of malignancy in uterine smooth muscle tumors. Additionally, NM-23 is a promising candidate gene for determination of metastatic potential in these tumors.     

Treatment of Multiple Cutaneous Leiomyomas with CO2 Laser Ablation

BACKGROUND: Cutaneous leiomyomas are benign tumors that can be exquisitely painful. Symptomatic lesions often necessitate treatment, but many options are inadequate or create substantial morbidity. Without a definitive therapy of first choice, the search continues for alternative methods to alleviate discomfort in affected patients. OBJECTIVE: To describe the use of CO2 laser ablation for the symptomatic treatment of a 73-year-old woman with multiple cutaneous leiomyomas in whom pharmacologic therapy was contraindicated and surgical excision was impractical. METHODS: CO2 laser ablation of six symptomatic cutaneous leiomyomas was performed under local anesthesia. After initial success, all remaining symptomatic lesions were treated. RESULTS: There was complete pain relief in the originally treated lesions 9 months postoperatively, and in all remaining treated lesions 3 months postoperatively. CONCLUSION: CO2 laser ablation may be an effective alternative treatment for symptomatic cutaneous leiomyomas.     

Inguinal smooth muscle tumors in women-a dichotomous group consisting of Müllerian-type leiomyomas and soft tissue leiomyosarcomas: an analysis of 55 cases

Assessment of the biological potential of smooth muscle tumors can be difficult and depends primarily on tumor site, stage, and histologic parameters. In this study, we examined the clinicopathologic and immunohistochemical features of 55 noncutaneous inguinal smooth muscle tumors of women (age range, 20 to 82 y; median, 57 y). Histologically, 23 tumors were considered as leiomyomas. They showed low mitotic activity (range, 0 to 6 mitoses/10 high-power fields, without atypical mitotic figures), minimal cytologic atypia, and absence of coagulative necrosis. Fifteen of these tumors histologically resembled conventional uterine leiomyomas and 8 resembled their variants: lipoleiomyomas (n = 2) and epithelioid variants (n = 6). The mean size was 7.8 cm, and half of the tumors with specified location arose in association with the round ligament. Immunohistochemical expression of estrogen receptor (ER) and/or Wilms tumor protein (WT1) was detected in most cases (83%), supporting Müllerian derivation. Follow-up data (range, 10 to 29 y; median, 13 y) on 11 patients showed that all were alive without disease or death from unrelated causes. The second group, classified as leiomyosarcomas, consisted of 32 mitotically active smooth muscle tumors, almost invariably with atypical mitotic figures, and exhibiting significant cytologic atypia. These patients were older than those with leiomyomas, and their tumors were mostly subcutaneous with a mean tumor size of 5.4 cm. Two leiomyosarcomas showed a femoral vein origin, but none were associated with the round ligament. All but 3 leiomyosarcomas were negative for ER. Follow-up data on 13 patients (range, 2 mo to 30 y; median, 4.5 y) showed that 5 died of metastatic sarcoma. Six individuals were alive without disease (median, 16 y), and 2 died of unrelated causes. In conclusion, inguinal smooth muscle tumors in women are a dichotomous group. They consist of ER/WT1-positive Müllerian-type leiomyomas resembling uterine leiomyomas with an excellent prognosis and conventional LMSs that are usually ER/WT1-negative and show a variable malignant course. Separation of these 2 categories is important for prognostication and optimal patient management, and is aided by immunohistochemical studies for ER and WT1.     

A case report of an intracaval extrathoracic solitary fibrous tumour

Solitary fibrous tumours are infrequent neoplasms based in the pleura that are predominantly benign with malignant pathology and behaviour described in 10–36% of cases. Extrathoracic solitary fibrous tumours (ESFTs) have been considered separately to their intrathoracic counterparts and comprise a third of all solitary fibrous tumours. The extrathoracic location was identified as an adverse prognostic factor for local recurrence but not for metastatic disease. So far, there have not been any reports of solitary fibrous tumours demonstrating caval infiltration. We present a case of a benign ESFT infiltrating into the perirenal inferior vena cava. Together with extrauterine leiomyomas, ESFTs should also be considered as a differential diagnosis for the rare benign lesions invading the inferior vena cava.     

Morphologic features of uterine leiomyomas associated with hereditary leiomyomatosis and renal cell carcinoma syndrome: a case report

Patients with hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome are prone to develop smooth muscle tumors of the uterus and skin and also renal carcinomas. The morphologic features of renal tumors that arise in the setting of HLRCC are well described, the hallmark feature being the presence of prominent eosinophilic nucleoli surrounded by a clear halo. Renal tumors associated with HLRCC are aggressive and often present late with high-stage disease. Early detection of patients with HLRCC could lead to surveillance for renal carcinomas. Women with HLRCC often present at a young age with uterine leiomyomas and frequently undergo hysterectomy as a result. HLRCC associated uterine leiomyomas show nuclear features similar to those described in HLRCC associated renal tumors. Therefore, presence of these nuclear features in uterine smooth muscle tumors may aid in early detection of HLRCC, resulting in surveillance for renal carcinoma. We present a case of a 32-year-old woman who underwent hysterectomy for uterine fibroids and subsequently presented 5 years later with high-stage renal carcinoma. Histologic evaluation of uterine leiomyomas and renal carcinoma revealed identical nuclear features, that is, prominent nucleoli with perinucleolar halos, raising the possibility of HLRCC. Subsequent testing confirmed a fumarate hydratase mutation. The presence of above-described nuclear features in uterine leiomyomas should raise the possibility of HLRCC.     

Leiomyoma of the kidney. Differential diagnostic aspects of renal cell carcinoma with increasing clinical relevance

Renal leiomyoma is a rare benign tumor which has its origin in smooth muscle cells of different structures of the kidney. The clinical incidence of renal leiomyoma is much lower than the frequency described in large autopsy studies. Renal leiomyomas are mainly located in the renal capsula and pelvis or next to those structures. Clinical symptoms are nonspecific (e.g., flank pain or flank tumor) or completely absent, making most of the leiomyomas an incidental finding during routine diagnostics. A differential diagnosis between renal leiomyoma and renal cell carcinoma on the basis of medical imaging is extremely difficult. Therefore, laparotomy and nephrectomy are performed in most cases of suspected renal cell carcinoma. The definitive diagnosis of a leiomyoma is only possible after histological examination of the tumor. Due to the rising number of diagnoses resulting from improved medical imaging, renal leiomyomas are gaining more importance in the differential diagnosis of renal cell carcinoma, especially with respect to kidney-sparing surgery.     

Bilateral synchronous leiomyomas of the testicular tunica albuginea. A case report and review of the literature

Genitourinary leiomyomas are uncommon but may arise from any structure or organ containing smooth muscle [1]. The kidney capsule is the most common location for genitourinary leiomyomas [2]. We report a case of bilateral synchronous leiomyomas of the testicular tunica albuginea.     

Renal leiomyoma: Case report and literature review

Renal leiomyomas are rare benign tumours of the kidney originating from muscle cells. They are usually found by an autopsy, whether the patient is asymptomatic or has symptoms (i.e., abdominal/flank pain, hematuria, palpable mass). Today the widespread use of ultrasonography and computed tomography has increased the detection of clinically asymptomatic renal leiomyomas. The differential diagnosis between leiomyomas and other malignant lesions (above all renal cell carcinoma or leiomyosarcoma) is still possible by histological examination. Radiological examinations are not sufficient for the differential diagnosis. Renal leiomyomas have no aggressive behaviour and they usually do not metastasize. The prognosis, after surgery, is excellent without recurrence. We report a case of leiomyoma in a 31-year-old man who presented hematuria and flank pain. We also review the literature and provide a summary of clinical, radiological and histological features of renal leiomyomas.     

An immunohistochemical analysis of endometrial stromal and smooth muscle tumors of the uterus: a study of 54 cases emphasizing the importance of using a panel because of overlap in immunoreactivity for individual antibodies

The expression of desmin, h-caldesmon, calponin, CD10, CD34, CD99, inhibin, and keratin (AE1/3-Cam 5.2) was studied in 10 conventional leiomyomas, 9 highly cellular leiomyomas, 9 epithelioid smooth muscle tumors, 9 leiomyosarcomas, 10 endometrial stromal tumors (4 with smooth muscle metaplasia), and 7 uterine tumors resembling ovarian sex cord tumors (UTROSCTs). c-kit expression was tested in 10 endometrial stromal tumors, 7 UTROSCTs, and 9 leiomyosarcomas. Desmin was positive in almost all smooth muscle tumors except those of epithelioid type, which were positive in only about half of the cases. It also stained areas of smooth muscle differentiation in endometrial stromal tumors and five of seven UTROSCTs. h-caldesmon was positive in almost all nonepithelioid smooth muscle tumors and in areas of smooth muscle differentiation in endometrial stromal tumors; it was positive in only about half of the epithelioid smooth muscle tumors and negative in all UTROSCTs. Calponin was positive in most tumor types. CD10 was positive in nine of 10 endometrial stromal tumors and five of seven UTROSCTs, although very focally in the latter group. It was also expressed, however, in almost all leiomyosarcomas, almost 50% of highly cellular leiomyomas, and rarely in the other smooth muscle tumors. CD34 was negative in the tested tumors with rare exceptions. CD99 and inhibin were positive in four of seven and one of seven UTROSCTs. Keratin positivity was found in most (five of seven) UTROSCTs and occasionally in smooth muscle tumors (seven of 37). c-kit was negative in all endometrial stromal tumors, UTROSCTs, and leiomyosarcomas. The major conclusions of this study are as follows: 1) Pure endometrial stromal tumors are usually desmin negative. 2) In contrast to some previous studies, CD10 expression was often seen in smooth muscle tumors, including most leiomyosarcomas and almost half of highly cellular leiomyomas. As a result, a panel of CD10, h-caldesmon, and desmin should be used and will distinguish endometrial stromal tumors from highly cellular leiomyomas in most cases. 3) In contrast to a previous study, no significant differences in immunoreactivity were seen between h-caldesmon and desmin in tumors with smooth muscle differentiation. 4) The absence of h-caldesmon in UTROSCTs helps separate them from epithelioid smooth muscle tumors. 5) UTROSCTs may express epithelial, stromal, and smooth muscle markers, suggesting divergent differentiation. 6) Our study shows less frequent inhibin expression in the sex cord-like elements of the UTROSCTs than in other studies. 7) c-kit may help distinguish metastatic endometrial stromal tumors of the uterus (c-kit negative) from gastrointestinal stromal tumors (c-kit positive). 8) CD34, CD99, and keratin have no or minimal role in this area, but keratin positivity in smooth muscle tumors should not lead to their confusion with epithelial tumors.     

Leiomyosarcoma of the small intestine associated with von Recklinghausen's disease: report of a case.

A 54-year-old woman with intestinal multiple smooth muscle tumors including leiomyosarcoma, epithelioid leiomyoma, and leiomyomas in association with von Recklinghausen's disease is reported. Thirteen years after the excision of an intestinal leiomyosarcoma, another leiomyosarcoma arose in a different area of the jejunum and was also completely resected. Although leiomyomas are occasionally recognized in patients with von Recklinghausen's disease and a gastrointestinal neoplasm, there have been no other reports of leiomyosarcomas. Careful observation of the tumor is necessary and, if a rapid increase in tumor size is recognized, malignant tumor may have arisen and early surgical treatment is required.     

Diffuse, perinodular, and other patterns of hydropic degeneration within and adjacent to uterine leiomyomas. Problems in differential diagnosis.

We describe 10 uterine leiomyomas with hydropic changes that created problems in differential diagnosis. The patients ranged in age from 41 to 51 years (mean, 45 years). Their presenting symptoms did not differ from those of patients with typical uterine leiomyomas. The follow-up, available for eight patients, was uneventful. Gross and microscopic examination revealed otherwise typical or cellular leiomyomas with focal accumulations of edema fluid (hydropic degeneration), typically associated with variable amounts of collagen ("hyaline degeneration"). Significant accumulations of acid mucins (myxoid degeneration) were present in only one case. These changes resulted in one or more of the following: (a) a characteristic perinodular hydropic change that mimicked intravenous leiomyomatosis on both gross and microscopic examination; (b) hydropic changes extending beyond the confines of the leiomyomas that raised the suspicion of myxoid leiomyosarcoma; and (c) extensive or subtotal obliteration of the usual architecture of the leiomyoma, often accompanied by numerous thick-walled blood vessels, that tended to obscure its smooth-muscle nature. Awareness of these changes combined with mucin stains and immunohistochemical stains for endothelial antigens should facilitate the diagnosis. Hydropic leiomyomas are clinically similar to typical leiomyomas; it is obviously important that they not be confused with intravenous leiomyomatosis or myxoid leiomyosarcoma.     

Surgical Management of Duodenal Leiomyomas

Duodenal leiomyomas are rare neoplasms and when present are usually asymptomatic. There are, however, isolated case reports of such leiomyomas giving rise to complications including gastrointestinal hemorrhage and obstruction. The purpose of this study was to review the surgical experience with duodenal leiomyomas at a large tertiary referral center. A retrospective review was performed of all patients with histologically proven duodenal leiomyomas encountered from 1975 to 1995. Twelve patients with surgically treated duodenal leiomyomas were identified. There were eight men and four women with a mean age of 57 years (range 20–73 years) at diagnosis. Anemia was the presenting sign in 10 patients (83%). The mean hemoglobin level at presentation was 10.8 ± 2.67 g/dl. Other presenting signs included melena (n= 5) and epigastric tenderness (n= 4). No patient had obstructive symptoms. Upper endoscopy visualized the lesion in 9 of 10 patients, but endoscopic biopsy provided an accurate histologic diagnosis in only two of six patients biopsied. All resected specimens underwent frozen section and permanent histopathologic analysis. Local excision was performed in eight patients, as benignancy was anticipated at surgical exploration and frozen section findings. Two patients underwent segmental duodenal resection with primary end-to-end anastomosis, and two patients underwent a Whipple procedure. There was no perioperative mortality. Morbidity was minimal. There was no tumor recurrence during a median follow-up of 8.4 years. Gastrointestinal blood loss is the most common complication of duodenal leiomyomas requiring surgical intervention. Small symptomatic leiomyomas with benign features may be safely treated with local excision.     

Life-threatening anaphylaxis to leuprorelin acetate depot: case report and review of the literature

Gonadotropin-releasing hormone analog depots have been widely used for a variety of diseases including prostate cancer, breast cancer, endometriosis, uterine leiomyomas, and central precocious puberty. Most of the side/adverse effects of gonadotropin-releasing hormone analog depots, such as leuprorelin acetate depot, are related to hypotestosteronism in males. Anaphylaxis to gonadotropin-releasing hormone analog depot is extremely rare. We present the first case report of a Japanese man who developed anaphylaxis to leuprorelin acetate depot during the treatment of metastatic prostate cancer and recovered successfully by conservative treatment. A drug-lymphocyte stimulation test showed that not only leuprorelin acetate itself, but also its vehicle polylactic and glycolic acids, might be responsible for the anaphylaxis to leuprorelin acetate depot. Because anaphylaxis can be lethal, the present case suggests that one should bear in mind the possibility of anaphylaxis in all patients who receive gonadotropin-releasing hormone analog depot and monitor such patients carefully.     

Hysteroscopic resection of submucous myomas: long term results

STUDY AIM: To evaluate the follow-up after operative hysteroscopic resection of submucous leiomyomas. PATIENTS AND METHODS: Between January 1990 and December 1996, 200 patients underwent operative hysteroscopic resection of 289 uterine leiomyomas. Indications were: menometrorrhagia (n = 159), postmenopausal metrorrhagia (n = 22), infertility (n = 19) as sole etiology. Sixteen patients had infertility and menometrorrhagia. RESULTS: The mean follow-up was 33.4 +/- 19.2 months. Twenty-three patients were lost to follow-up. Due to the large size of the leiomyomas, 35 patients had 2 or 3 resections and a total of 241 hysteroscopic resections were performed. Twelve complications (5%) occurred without death or need for intensive care. An improvement of clinical symptoms was observed in 74% of patients. The predictive factors of failure were: size (> 5 cm), number of intracavitary leiomyomas (> 3), hysterometry (> 12 cm), intramural myoma class 2 and association of leiomyomas. Eight of the 35 infertile patients subsequently became pregnant, but with only two live births (5.8%). CONCLUSION: Hysteroscopic myomectomy appears to be safe, effective and reproducible for the treatment of menstrual disorders. Intramural class 2 and larger leiomyomas constitute the limits of the endoscopic technique.     

True smooth muscle neoplasms of the gastrointestinal tract: morphological spectrum and classification in a series of 85 cases from a single institute

BACKGROUND AND AIM: True smooth-muscle neoplasms of the GI tract have been only rarely studied in the KIT era. Their incidence among other GI mesenchymal tumours and their clinicopathological spectrum have not been sufficiently analysed. MATERIALS AND METHODS: We reviewed all GI mesenchymal lesions at the Pathology Institute of the Nuremberg Clinic Centre from 1994 through 2005. RESULTS: Among 262 lesions, there were 142 GISTs (54%) and 85 true smooth muscle neoplasms (32%). Smooth muscle neoplasms comprised 72 polypoid leiomyomas (78%, 5 oesophageal and 67 colorectal), 10 intramural leiomyomas (11%, 5 oesophageal, 4 gastric and one ileal), two intramural leiomyosarcomas in the sigmoid colon and ileum (2%) and one polypoid leiomyosarcoma involving the stomach, descending colon and the retroperitoneum concurrently. None of the leiomyomas with available follow-up have recurred or metastasised. CONCLUSION: Smooth muscle neoplasms are the second most common mesenchymal neoplasms in the GI tract after GISTs. They may arise either from the muscularis mucosae or proper muscle layer forming polypoid and intramural lesions, respectively. Polypoid leiomyomas are more common in the rectosigmoid, while intramural ones mainly arise in the vicinity of the oesophagogastric junction. Polypoid leiomyomas are sufficiently treated by endoscopic resection, and local surgical excision is the treatment of choice for intramural leiomyomas. Intramural leiomyosarcomas are rare high-grade sarcomas that commonly have infiltrated into the surrounding tissue or metastasised by the time of diagnosis.     

BENIGN MESENCHYMAL TUMOURS OF THE COLON AND RECTUM: A RETROSPECTIVE STUDY

Sixteen patients were diagnosed as having a benign mesenchymal tumour of the colon or rectum at the Repatriation General Hospital, Concord, between 1950 and 1979. There were six lipomas and ten leiomyomas. Lipomas occurred more commonly in the colon, while leiomyomas predominated in the rectum. The symptoms produced by the lipomas included abdominal pain, rectal bleeding and diarrhoea. Leiomyomas were found incidentally at sigmoldoscopy in six patients, while three presented with rectal bleeding and one with constipation. Lipomas were larger than leiomyomas and were associated with more complications. Both tumours, however, produced few physical signs.     

Multiple solitary leiomyomas in the esophagus: Report of a case

We herein report our findings for an asymptomatic 65-year-old man presenting with multiple solitary leiomyomas in the esophagus, who was undergoing follow-up for primary aldosteronism with high levels of serum progesterone. Esophageal endoscopy and computed tomography showed multiple submucosal tumors with calcification in the thoracic esophagus. A subtotal esophagectomy was performed because the possibility of malignancy could not be ruled out based on a needle biopsy taken of the specimen. The final resected specimen showed eight solitary, encapsulated nodules between the middle and lower thoracic esophagus. Histopathological examinations showed these nodules to have the typical histological findings of leiomyomas. In addition, staining with antibodies against the progesterone receptor revealed diffuse expression in the nuclei of the leiomyoma cells. Only four cases with more than eight solitary leiomyomas have been reported, including the current case. However, this is the first reported case where primary aldosteronism with elevated serum progesterone levels has been implicated in the pathogenesis of multiple solitary leiomyomas in the esophagus.     

MSCT和MRI诊断巨大子宫肌瘤

目的分析巨大子宫肌瘤的临床表现与MSCT、MRI特征。方法收集28例经手术、病理证实的最大直径>7cm的巨大子宫肌瘤患者,分析其临床及MSCT、MRI表现并与手术病理对照。结果 28例共37个瘤体,其中浆膜下肌瘤15例,肌壁间肌瘤9例,黏膜下肌瘤4例。MSCT平扫肌瘤密度均混杂不均,增强扫描呈不均匀强化,其中4例主要表现为囊性变性。病变MR平扫T1WI为等或略低信号,T2WI为混杂信号,增强扫描呈不均匀强化。结论巨大子宫肌瘤的MSCT、MRI表现有一定特征性,对诊断巨大子宫肌瘤有较高临床价值。     

Leiomyoma of Testis –A Case Report

Leiomyomas, benign tumors, can arise from any organ containing smooth muscle. In the genitourinary tract, the renal capsule is most commonly involved. Intrascrotal leiomyomas are infrequently seen. Testis-associated leiomyomas are extremely rare. We report here testis associated leiomyoma.