Review of the pathophysiology, diagnosis, and therapy of vulvar leiomyoma, a rare gynecological tumor.

The objective of this article is to discuss the pathophysiology, diagnosis, differential diagnosis, and therapy of vulvar leiomyoma. We performed a review of all English-language reports of vulvar leiomyoma published in PubMed from 1978 to 2015 using the following search terms: "vulval leiomyoma," "vulvar leiomyoma," "vulval smooth muscle tumor," and "external genitalia smooth muscle tumor." Vulvar leiomyomas, which are rare benign monoclonal tumors, most commonly occur in the fourth and fifth decades of life. The genetics of vulvar leiomyoma remain undefined. Three principal histological patterns have been identified: spindled, epithelioid, and myxoid. Imaging tests such as ultrasound, endoscopic ultrasound, and magnetic resonance imaging are used in diagnosis. Surgical excision is the only curative treatment for vulvar leiomyomas. Establishment of a full differential diagnosis list and correct final diagnosis before surgery are essential for optimal clinical management. Although recurrence of vulvar leiomyoma is extremely rare, long-term follow-up of all cases is advisable.

Ligation-assisted endoscopic enucleation for treatment of esophageal subepithelial lesions originating from the muscularis propria: a preliminary study.

An innovative ligation-assisted endoscopic enucleation (EE-L) technique was developed for the diagnosis and treatment of esophageal subepithelial lesions (smaller than 12 mm) originating from muscularis propria by combining endoscopic band ligation and endoscopic enucleation techniques. The aim of the study was to evaluate efficacy and safety of EE-L technique in the treatment of esophageal subepithelial lesions (smaller than 12 mm) originating from muscularis propria. Forty-seven esophageal subepithelial lesions (smaller than 12 mm) originating from the muscularis propria in 44 patients were treated with EE-L between September 2010 and September 2012. The lesion was first aspirated into the transparent cap attached to the tip of endoscope. The elastic band was then released around its base. The purpose of ligation was to force the lesion to assume a polypoid form with a pseudostalk. Endoscopic enucleation was then performed until the tumor was completely enucleated from muscularis propria using a hook knife and forceps. All tumors (median diameter: 8.2 ± 2.3 mm, range: 4-12 mm) were enucleated completely. Histopathology identified 45 tumors (95.7%) as leiomyoma, 2 (4.3%) tumors as gastrointestinal stromal tumor with very low risk. The mean time of the EE-L procedure was 12.5 ± 4.6 minutes (range: 6-23 minutes). Two patients experienced self-limiting, non-life-threatening hemorrhage after EE-L. No perforation and massive hemorrhage requiring further endoscopic or surgical intervention occurred. There were no recurrences during the 6-24 months follow-up period. EE-L offers the option of localized treatment of small esophageal muscularis propria tumors (smaller than 12 mm) with relatively few complications and low mortality, and provides the advantage of allowing a histopathological diagnosis. All the resected lesions in this study had a benign pathology.

Leiomioma gástrico / Gastric leiomyoma

El leiomioma gástrico es un tumor intramural, extramucoso, localizado, en la mayoría de los casos, en el tubo digestivo superior, a nivel del tercio distal del esófago y porción superior del estómago. Puede afectar ambos sexos, pero es muy raro en la edad pediátrica. Aumenta su incidencia a partir de la quinta y sexta década de la vida, y son de tamaño variable. Se presenta el caso de un paciente masculino, de 10 años de edad, blanco, con un leiomioma a nivel de la pared gástrica, que fue resuelto quirúrgicamente con resección completa de la masa tumoral que comprometía gran parte de la curvatura mayor gástrica mediante laparotomía, pero el diagnóstico inicial y toma de biopsia se realizó por vía endoscópica.

Gastric leiomyoma is an intramural, extramucosal tumor that is mostly located in the upper digestive tube, at the distal third of the esophagus and the upper portion of the stomach. It may affect both sexes but it is very unusual at pediatric ages. Its incidence is higher from the 50 and 60 years of age on, with variable sizes. This is the case of a male 10 years-old Caucasian child, who had leiomyoma inside his gastric wall and was operated on through laparotomy to completely remove the tumor mass that endangered a large part of the major gastric curvature. The initial diagnosis and the biopsy were based on endoscopic procedures.

Primary non-clear-cell adenocarcinoma of the vagina in a diethylstilbestrol exposed woman.

A 54-year-old woman with a history of in-utero diethylstilbestrol (DES) exposure, who had a prior hysterectomy for symptomatic leiomyomata and dysmenorrhea, presented for vaginal bleeding. Vaginal biopsies showed a non-clear-cell adenocarcinoma, and the patient was subsequently treated with radiation therapy. We present a case of primary vaginal non-clear-cell adenocarcinoma in a patient with in-utero DES exposure. Continued monitoring of older DES-exposed women for vaginal lesions is warranted because of reported cases of non-clear-cell adenocarcinoma and persistent risk of clear cell adenocarcinoma.

[Leiomyomatosis of the colon: case report and literature review]. / Leiomyomatose des Rektums: Fallbericht und Literaturübersicht.

We present the findings of a 67 year old male patient with an intestinal leiomyomatosis localized in the rectum. To our knowledge, this is the fifth case of intestinal leiomyomatosis reported so far. The most characteristic findings of this rare disease include a cuff-like tumorous proliferation of smooth muscle within the bowel wall which may extend into extramural tissue and result in a stenosis of a longer bowel segment. Because of severe obstructive symptoms over 5 years, the patient had to undergo surgery with resection of the rectum. The histological examination revealed a morphology and immunophenotype comparable to usual leiomyomas with the exception of hyalinosis-like changes in the blood vessels, apparently a special feature of leiomyomatosis. A novel finding in our case was the occurrence of skeinoid fibers which have so far only been reported in gastrointestinal stromal tumors.

Epithelioid vascular leiomyoma of the uterus mimicking glomangiomyoma.

CASE REPORT: An unusual case of epithelioid vascular leiomyoma mimicking glomangiomyoma arising in the uterine corpus of a 55-year-old Japanese woman is presented. The surgically resected uterine mass, measuring 4.0x3.5 cm2, demonstrated a dark red well-circumscribed tumor. Histologically, the rounded epithelioid cells around the dilated vessels showed gradual transition to spindled smooth muscle cells. Immunohistochemistry was positive for smooth muscle actin, but negative for collagen IV. CONCLUSION: The experience in this case emphasizes that glomangiomyoma-like feature is interesting and might indeed be a new histological variant of uterine leiomyomas.

Myxoid epithelioid leiomyoma of the vulva: a case report.

BACKGROUND: Smooth muscle tumors of the vulva are rare and sometimes create diagnostic difficulty because of unusual morphological features. CASE: A 29-year-old African-American female presented with a vulval mass during her 5th week of pregnancy. The lesion was surgically removed under the clinical impression of a Bartholin cyst. Histopathological findings of the lesion and immunohistochemical stain results are characteristic of myxoid epithelioid leiomyoma. CONCLUSION: Up to now, there are only a few cases of myxoid epithelioid leiomyoma of the vulva reported in English literature. We review previously reported cases and discuss the morphologic differential diagnosis. The mass presented in this report was the largest benign epithelioid leiomyoma in the literature.

Renal angiomyolipoma with contrast-enhancing elements mimicking renal malignancy: radiographic and pathologic evaluation.

The diagnosis of angiomyolipoma can typically be made on the basis of characteristic computed tomography findings. Varying tissue compositions within an angiomyolipoma can create difficulty in pathologically differentiating benign from malignant lesions. Epithelioid angiomyolipoma is a variant of angiomyolipoma characterized by the presence of epithelioid cells. We report a case of two discrete contrast-enhancing lesions within an enlarging angiomyolipoma that radiographically mimicked malignant elements. This finding presented a diagnostic challenge in terms of selecting medical versus surgical intervention. Surgical excision of the lesions was performed and the pathologic examination revealed a benign angiomyolipoma with epithelioid features.

Clinicopathologic study of primary malignant gastrointestinal stromal tumor of the stomach, with special reference to prognostic factors: analysis of results in 140 surgically resected patients.

BACKGROUND: Malignant gastrointestinal stromal tumors (GISTs), previously termed leiomyosarcomas or epithelioid leiomyosarcomas, are known to show wide variability in their malignancy. We evaluated the clinicopathological features of a large number of primary malignant gastric GISTs to clarify which features were independent prognostic factors. METHODS: Clinicopathologic features (age, sex, tumor location, mode of growth and size, surgical method, ulceration, cell type, nuclear atypia, cellularity, mitotic index, growth pattern, necrosis, hemorrhage, direct tumor invasion, peripheral lymphoid cuffing, expression of alpha-smooth muscle actin [alpha-SMA], desmin, caldesmon, vimentin, CD34, c-kit protein and s-100 protein, and MIB-1 index) were evaluated by multivariate analysis in 140 patients with resected primary malignant gastric GISTs identify independent prognostic factors. RESULTS: Univariate analysis showed that each of the following factors had a significant deleterious influence on prognosis: male sex, tumor size 10 cm or more, presence of ulceration, an epithelioid cell component, severe nuclear atypia, high cellularity, a mitotic index of more than 10, an exogastric or invasive growth pattern, necrosis, hemorrhage, direct tumor invasion of surrounding tissue, negative caldesmon immunoreactivity, positive S-100 protein immunoreactivity, and a MIB-1 antigen labeling index of more than 10%. multivariate analysis showed that male sex, tumor size 10 cm or more, presence of an epithelioid cell component, and a mitotic index of more than 10 were statistically significant indicators of a poor prognosis ( P = 0.013, 0.001, 0.014, and <0.001, respectively). Multivariate analysis using the MIB-1 index instead of a mitotic count showed that male sex, tumor size 10 cm or more, presence of necrosis, and a MIB-1 antigen labeling index of more than 10% were independent predictors of a poor prognosis ( P = 0.009, 0.001, 0.043, and <0.001, respectively). CONCLUSION: Male sex, tumor size 10 cm or more, and cell proliferation as estimated by the mitotic index or MIB-1 index are independent indicators of a poor prognosis in primary malignant gastric GIST.

[Direct experience in the treatment of unusual, primary, and secondary malignant tumors of the stomach]. / Esperienza diretta nel trattamento di tumori maligni rari, primitivi e secondari dello stomaco.

The reduced incidence of gastric cancer, due to a better patients surveillance and more accurate understanding of prophylactic measure, has allowed a detection of early stages as well as cancers with different origin cells. Either the difficulty of differential diagnosis or the various surgical and integrated approaches, make these neoplasm hard to enroll in standard treatment protocols. Our experience consists of 5 clinical cases with four different histology: lymphoma, leiomioma, carcinoid and gastrinoma. A rare case of secondary involvement of the stomach by an adrenal adenocarcinoma is also described. Lymphomas benefit of the surgical therapy only in early stages (IE, IIE), eventually associated to chemio- and radiotherapy, in relationship with local diffusion of the disease; gastric resection is more supported than gastrectomy. Polychemotherapy, with or without radiotherapy, is used for advanced stages (IIIE and IVE), leaving to surgery the role of controlling hemorrhagic or occlusion compliances. GIST have a different therapeutic approach: surgery represents the only choice since chemio- and radiotherapy have no benefits for the biological characteristics of such neoplasm. Surgery is the ideal choice for the carcinoids with some indications for chemio radiotherapy in the palliative surgery or in the relapsing. We emphasize the rarity of the secondary gastric neoplasm coming from the suprarenal glands. We analyze our diagnostic and therapeutic protocols, comparing them with the current literature.

Partial resection for leiomyoblastoma of stomach.

We herein report the case of a 51-year-old male with a submucosal tumor of the stomach which was histopathologically demonstrated to have features compatible with leiomyoblastoma. As the submucosal tumor of the posterior wall of the antrum, which was initially found in the upper gastrointestinal series done during a health examination, had grown 3.8 cm in diameter, the partial resection of the full thickness of the gastric wall containing the tumor with a sufficient tumor margin was performed.

Successful resection of a liver metastasis from gastric leiomyoblastoma: report of a case.

A 20-year-old woman was referred to our hospital for detailed investigation of a gastric submucosal tumor. A leiomyoma was preoperatively diagnosed and laparoscopic-assisted enucleation was performed. The resected tumor was 4 x 3 x 1.5 cm in size and postoperative histological examination identified it as a gastric leiomyoblastoma. Therefore, a secondary resection in the form of a distal gastrectomy was carried out. No tumor cells were found in the gastric specimen or in the lymph nodes; however, 5 months after the operation, an abdominal computed tomography scan revealed a recurrence in the liver, and she was readmitted for further examinations. The lesion was diagnosed as a single liver metastasis from the gastric leiomyoblastoma and successfully resected. The histopathological findings of the liver tumor resembled those of the primary gastric tumor. Her postoperative course was uneventful and she has been well, without any evidence of recurrence, to date. Only 12 other cases of leiomyoblastoma of the stomach with liver metastasis have been reported in Japan, all of which were associated with a very poor prognosis. Therefore, patients with this unusual disease entity should be carefully followed up after resection of the primary tumor.

[Imaging features of gastric stromal tumors: radiologic-pathologic correlation. Report of 4 cases]. / Imagerie des tumeurs stromales gastriques: corrélations radio-anatomopathologiques. A propos de quatre cas.

GOAL: This study concerns the new anatomo-pathologic semantics of the ancient gastric leiomyoblastoma that become gastric stromal tumors (GST) and identified as stemming of "pace-maker" cells of Cajal related to the immunohistochemical characterization of the phenotype. MATERIAL: and methods. We limited the study to the mesenchymatous tumors to "pacemaker" cells. For this purpose, we report four documented observations of gastric stromal tumors correlated to the histology and to the immunohistochemical study. RESULTS: Although some signs are often described (exogastric development, heterogeneity with cystic and necrotic component, predominating peripheral enhancement...), radiological aspects of these tumors are not specific because, analogues to the other mesenchymal tumors (leiomyoma or schwannoma). The topographic diagnosis is difficult, realized by echoendoscopy, CT scan and MRI. The irregularity of contours evokes the malignancy as hemoperitoneum. GST do not show lymphophilic behavior that differentiates them from the adenocarcinomas and gastric lymphomas. The surgical treatment is the best treatment, allowing the immuno-histological diagnosis of certainty from the complete operative specimen. CONCLUSION: Some tumors are difficult in classifying despite classic histology necessitating immunohistochemical tests for the identification of muscular, nervous, autonomous nervous system flexion of mesenchymatous tumors with epithelioid or spindle cells of the gastrointestinal tract. Then a radio-clinical follow-up is therefore indicated: the evolution is the alone real marker of malignancy.

Laparoscopic treatment of gastric stromal tumors.

BACKGROUND: The laparoscopic resection of gastric stromal tumors (GST) is being performed with increased frequency. METHODS: Between November 1993 and October 1998, nine consecutive patients with benign and low-grade gastric stromal tumors underwent laparoscopic resection using intraoperative endoscopy. For lesions located on the anterior wall (three cases), a direct approach was utilized. Lesions located on the posterior wall were resected via a transgastric approach (four cases) or through a small opening on the omentum or on the gastrocolic ligament (two cases). Excision of the lesions was performed manually by means of electrocautery and scissors in eight cases; the gastric incisions were closed by manual running suture. An endoscopic stapler device was used in one case only. RESULTS: All patients were successfully treated laparoscopically; there were no conversions to open surgery. Operative time ranged from 75 to 120 min. There was one bleeding from the suture line of the gastric wall postoperatively that was treated conservatively. The average postoperative hospital stay was 4 days (range, 2-6). CONCLUSIONS: In light of the results reported in the literature and on the basis of the present work, it seems that laparoscopic resection of GST should be considered as the treatment of choice. Wedge resection of anterior wall lesions is generally performed. The treatment of posterior wall lesions is still controversial. In our opinion the direct approach should be reserved for lesions located on the posterior wall of the body, which can be easily reached through the greater omentum, while the transgastric approach should be preferred for lesions located on the fundus and antrum. Manual excision allows a tailored operation; hand-sewn sutures are always feasible, and they are cheaper than stapled ones.

Minimally invasive surgery for posterior gastric stromal tumors.

BACKGROUND: Because involvement is extremely rare, surgery for gastric stromal tumors consists of local excision with clear resection margins. The aim of this study was to report the results of a consecutive series of nine patients with posterior gastric stromal tumors that were excised using a minimally invasive method. METHODS: Patients received a general anesthetic before placement of three laparoscopic ports- a 10-mm (umbilical) port for the telescope and two working ports, a 12-mm port (left upper quadrant) and a 10-mm port (right upper quadrant). Grasping forceps were placed through an anteriorly placed gastrotomy to deliver the tumor through the gastrotomy into the abdominal cavity, thus allowing an endoscopic linear cutter to excise the tumor with a cuff of normal gastric tissue. RESULTS: Nine consecutive patients with a median age of 73 years (range, 47-83) were treated. In seven patients, laparoscopic removal of the tumor was achieved. Two patients required conversion to an open operation because the tumor could not be delivered into the abdominal cavity. The median length of postoperative stay for the seven patients in whom the procedure was completed laparoscopically was 3 days (range, 2-6). CONCLUSIONS: Posterior gastric stromal tumors can be removed safely using this minimally invasive method. Delivery of the tumor through the gastrotomy is essential for success.