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[Epithelioid bilateral ovarian leiomyosarcoma: a study].

Primary ovarian leiomyosarcoma is extremely rare and generally affects post menopausal women. It is usually unilateral and more than 10 cm in diameter. Its histogenesis is not clear its prognosis seems to be improved by radical surgery and adjuvant therapy. We report a case of a 54 year-old, nulliparous, post menopausal woman who had abdominal distention. Physical examination revealed a large pelvi-abdominal mass. The excision of tumor with bilateral salpingo-oophorectomy and hysterectomy was carried out. A diagnosis of bilateral ovarian epithelioid leiomyosarcoma was made on pathological examination with immunohistochemistry. Adjuvant chemotherapy was given. After 3 months of follow up, abdominal sonography revealed a peritoneal recurrent tumor with hepatic metastasis. Our objectives are to discuss the histopathological features and the prognosis of this tumor.

Biomarkers, Tumor↗

Retroperitoneal leiomyosarcoma mimicking acute appendicitis: laparoscopic management.

BACKGROUND: Retroperitoneal leiomyosarcomas (RLMS) are a challenging clinical entity. The vast majority of patients are operated on when tumors are advanced. We report herein a case of RLMS, mimicking acute appendiceal disease and treated successfully via laparoscopy. METHODS: A 37-year-old woman, para 1, was admitted to our department for right lower quadrant abdominal pain, fever, and leukocytosis. She had no changes in gastrointestinal and urologic function. A physical examination revealed the presence of abdominal guarding, rebound, and a tender mass in the right lower quadrant. The abdominal ultrasound showed an inhomogeneous ovoid mass (6 cm in diameter) located below the cecum, with no definite margins, and consistent with an appendiceal abscess. The patient was referred for laparoscopy. The procedure was performed with the aid of 3 ports: a 12-mm trocar in the umbilicus (open technique), a 10-mm trocar in the left iliac fossa, and a 5-mm one in the supra-pubic space. On inspection of the abdominal cavity, a retroperitoneal 6-cm mass was immediately found below the cecum and the appendix. Neither intraperitoneal seeding nor suspected lymph nodes were present. After dissection of the parietal peritoneum, the mass appeared to be encapsulated and well demarcated from all surrounding structures. It was eventually dissected and removed via a plastic bag. A standard appendectomy was also performed. The postoperative course was uneventful, and the patient was discharged on the 3rd day. The histology analysis of the resected specimen showed a totally excised G2 leiomyosarcoma. The appendix had no signs of inflammation. Postoperatively, the patient underwent a total-body CT-scan, which had no signs of residual or distant disease. No adjuvant therapy was necessary. At an 18-month follow-up, the patient was doing well and was disease free. CONCLUSION: Surgery represents the main therapeutic option for resectable RLMS. Laparoscopy is a useful diagnostic tool that allows safe resection of incidentally discovered, small and well encapsulated RLMS.

Acute Disease↗

Leiomyosarcoma of the maxilla with spinal metastasis: a case report.

Maxillary leiomyosarcoma is an uncommon tumor, and the occurrence of its metastasis to the spine is exceedingly rare. We report the case of a patient with maxillary leiomyosarcoma who developed a spinal metastasis 18 months following a total maxillectomy. Our patient represents only the third case of metastasis to the spine that has been reported in the literature, and the only reported case of metastasis to the iliac bone.

Adult↗

[Leiomyosarcoma of the kidney].

Leiomyosarcoma of the kidney is a rare neoplasm. Diagnosis is obtained by CT-Scan and immunohistochemical study. Radical nephrectomy is the only therapeutic way whose efficacity is known. The authors report a case of two localisations (kidney and bladder) of leiomyosarcoma.

Aged↗

[Leiomyosarcoma of the inferior vena cava].

The authors report a case of leiomyosarcoma of the infrarenal inferior vena cava presenting as an abdominal mass. CT and MR imaging and immunotyping led to the diagnosis of retroperitoneal leiomyosarcoma whose origin in the inferior vena cava was only confirmed by histological examination of the resection specimen. Surgical resection was followed by radiotherapy (4,500 rads) with a favourable course after 12 months. The progress in medical imaging and histology has considerably simplified the diagnosis of these rare retroperitoneal tumours. In the absence of any truly effective adjuvant therapy, treatment essentially consists of surgery and the clinical course is characterised by a high incidence of local recurrences.

Humans↗

Epithelioid component of uterine leiomyosarcoma simulating metastatic carcinoma.

A variant of uterine leiomyosarcoma containing two morphologic bell populations is described. A smooth-muscle component with a mitotic rate of 13 per 10 high-power fields, and a second population of cells mimicking metastatic small-cell carcinoma were both present. The second population was composed of small angulated epithelioid cells with hyperchromatic nuclei and barely discernible cytoplasm growing in tight rows, often in single file between fascicles of smooth muscle. DNA flow cytometry showed a large tetraploid aneuploid tumor cell population, and further suggested that the epithelioid cells probably represent tetraploid tumor cells in S-phase. Thus, this morphologic variant of leiomyosarcoma, unlike previously described variants of smooth-muscle tumors, is explained by the variable appearance of the neoplastic cells in different phases of the cell cycle.

Aged↗

[Leiomyosarcoma of the vena cava inferior. The correlation: ultrasound and fine-needle puncture biopsy].

The leiomyosarcoma of the inferior vena cava is a non-frequent tumor, mesenchymal, that has its origin in the smooth muscular tissue of the vascular wall. Its growth is slow and expansive, more frequently found on females. The symptomatology is related to the cava segment where it is located. The diagnosis is performed by ultrasonics, computed tomography and cavography, actually magnetic resonance acquires more and more importance. The treatment is specifically surgery along with radiotherapy and chemotherapy. A case of leiomyosarcoma of the inferior vena cava is presented in which diagnosis was performed using ultrasonics, tomography and cavography confirmed before surgery by biopsy punction with thin needle. In the diagnosis of this pathology it is described the use of intro-surgery ultrasonics.

Biopsy, Needle↗

[Leiomyosarcoma of the larynx: presentation of a case].

The authors report a new case of laryngeal leiomyosarcoma. The patient was a 43-year-old-man. The tumor extended to the left vocal cord, the paraglottic space and the deep layers of the left false vocal cord. Histologically, the lesion showed the typical pattern of a grade-3 leiomyosarcoma. Immunochemical studies revealed a strong positive staining with the actin smooth muscle antisera. A partial laryngectomy was performed. There was no evidence of recurrent or metastatic disease 10 months after surgery.

Adult↗

Rectal leiomyosarcoma: acute presentation after local injury.

Rectal leiomyosarcoma is rare, often large and found in the distal third of the rectum. Most symptoms differ from those of an adenocarcinoma. Staging is difficult and should include tumour size, necrosis, cellularity, number of mitoses and anaplasia. Abdominoperineal excision (APE) remains the only effective treatment. A 66 year old asymptomatic female presented with painless rectal bleeding after a fall. Digital examination and proctoscopy revealed a mass on the posterior rectal wall. Pelvic ultrasonography, arteriography and CT-Scan showed a huge retro-rectal lesion. Following APE, histopathology confirmed a leiomyosarcoma. No adjuvant therapy was given; there is no recurrence 4 years later.

Aged↗

Leiomyosarcoma in Poland's syndrome. A case report.

We describe a 56-year-old female with absence of the right pectoralis muscles, aplasia of the right breast, and skeletal deformities of the right hand, typical of Poland's syndrome. Following complaints of lower abdominal pain, a CT examination revealed an 8-cm mass in the right anterior pelvic wall. Surgical resection of the mass revealed a high-grade, poorly differentiated leiomyosarcoma. Poland's syndrome is known to be associated with a high incidence of leukemia but this is the first description of its association with leiomyosarcoma. Although we cannot exclude the possibility of a chance association, it is reasonable to assume that, similar to other syndromes with multiple congenital anomalies, the association with an increased incidence of malignancy is an integral part of the underlying genetic abnormality.

Female↗

Colorectal leiomyosarcomas: a pathobiologic study with long-term follow-up.

Colorectal leiomyosarcoma (CLM) is an uncommon tumour. Reports of its occurrence have been published mostly as single cases or small series. This study documents 12 cases of CLM that were seen over a 28-year period in Saskatchewan. The annual incidence of CLM was 0.45 per million people and constituted 0.12% of all colorectal malignant tumours seen during the study period. CLMs had a predilection for the rectum and sigmoid and commonly were associated with rectal bleeding or abdominal pain. More than half the tumours were detected by sigmoidoscopy. A correct preoperative or intraoperative histologic diagnosis of leiomyosarcoma was made in only two out of six cases. A potentially curative surgical procedure was done in 10 of the 12 patients. The mean follow-up was 6.9 years. Eight patients had tumour recurrence or metastasis, or both. From the findings of this study the authors recommend wide excision of colorectal smooth-muscle tumours whenever there is a suggestion of malignancy.

Adult↗

[Leiomyosarcoma of the breast 16 years following successful treatment of a rhabdomyosarcoma of the orbit in childhood].

A case of a mammary leiomyosarcoma in a 23-year-old woman is presented. The tumor appeared 16 years after successful treatment of an embryonal rhabdomyosarcoma of the orbit. Rhabdomyosarcomas are the most frequent soft tissue tumors of childhood, the orbit and the paratesticular region being the most common primary site for this tumor. In contrast, leiomyosarcomas other than those evolving from the viscera or the urogenital organs are rare neoplasms at any age. With the improvement of cancer treatment and survival rates, the risk of late effects after successful treatment for malignant tumors during childhood is increasing. Growth, development and fertility may be impaired and cosmetically disturbing facial and dental complications are common. Development of novel primary tumors is a known further consequence of successful treatment of brain tumors, retinoblastoma and acute leukemias. This is the case when high dose local radiation therapy and/or chemotherapy, especially alkylating agents, were used. Development of novel primary tumors is also known after treatment of childhood rhabdomyosarcomas. This report is intended to show that a second primary tumor may occur many years after a first successfully treated malignant neoplasm, and that young people are at risk for development of tumors at sites that are uncommon to this age group.

Adult↗

[The differential diagnosis between uterine leiomyosarcoma and the special subtypes of leiomyoma].

OBJECTIVE: To explore the more sensitive and specific immunohistochemical markers in differential diagnosis of uterine leiomyosarcoma (ULMS) and the specific subtypes of leiomyoma. METHODS: The routine SP immunohistochemical technique was used to examine the expression of desmin, alpha smooth muscle actin (SMA), calponin h1, h-caldesmon, estrogen receptor (ER), progesterone receptor (PR), proliferating cell nuclear antigen (PCNA), mast cells, and CD44v3 in 82 patients with smooth muscle tumors of the uterus, including 17 patients with leiomyosarcoma (LMS), 25 with cellular leiomyoma (CL), 15 with bizarre leiomyoma (BL) and 25 with ordinary leiomyoma (OL). RESULTS: The expression rates of desmin, SMA, h-caldesmon and CD44v3 in uterine LMS were all lower than those in CL and BL (all P<0.05), but these markers had limited significance in differentiation of ULMS from CL or BL. Compared to those in CL or BL, the expression rates of ER and PR and the number of mast cells were significantly lower in LMS (all P<0.01), while the expression of PCNA in LMS was higher (P<0.01). CONCLUSION: The increase of PCNA and decrease of ER and PR expression help differentiate ULMS from CL and BL. The number of mast cells was helpful in differential diagnosis of ULMS with high sensitivity and specificity.

Adult↗

Mesectodermal leiomyosarcoma of the ciliary body.

We report a case of low-grade mesectodermal leiomyosarcoma of the ciliary body, an extremely rare tumor of neural crest origin, occurring in a 12-year-old boy. MR imaging showed a well-marginated, ovoid soft tissue mass at the right temporal ciliary body, accompanied by total retinal detachment. The mass was slightly hyperintense relative to contralateral vitreous on T1-weighted images and markedly hypointense on T2-weighted images and enhanced very well. Initial biopsy of the mass suggested a peripheral nerve sheath tumor. The mass grew rapidly after biopsy, and enucleation was performed. The final diagnosis based on histology and immunohistochemistry was a low-grade mesectodermal leiomyosarcoma. To our knowledge, this is the first reported case of a malignant form of mesectodermal leiomyoma of the ciliary body.

Child↗

[Inflammatory leiomyosarcoma: a case report].

We report an additional case of inflammatory leiomyosarcoma, arising in the shoulder of a 31-year-old male. The rare inflammatory variant of leiomyosarcoma tends to affect young adults. Histologically it is characterized by a prominent lymphohistiocytic infiltrate often masking fascicles of spindle cells, as well as the presence of clusters of xanthoma cells and psammoma bodies. Smooth muscle differentiation is supported by desmin and smooth muscle actin expression. The differential diagnosis includes so-called inflammatory 'malignant fibrous histiocytoma'and inflammatory myofibroblastic tumor. The outcome of this variant seems to be favorable.

Adult↗

Leiomyosarcoma of the broad ligament arising in a pre-existing pure neurilemmoma-like leiomyoma.

A 35-year-old woman presented with an adnexal mass which came out as a leiomyosarcoma of the broad ligament arising in a pre-existing pure neurilemmoma-like leiomyoma. This is the first reported case of leiomyosarcoma arising in a pure neurilemmoma-like leiomyoma, the third reported case at an unusually young age showing evidence of a pre-existing leiomyoma and the 12th reported case of leimyosarcoma of the broad ligament. Is the likelihood of malignant transformation, especially in large leiomyomas of the broad ligament, a rare event?

Adult↗

[Case report: Pedunculated leiomyosarcoma of the inferior vena cava].

Leiomyosarcoma of the inferior vena cava is a rare mesenchymal tumor which originates from the smooth muscle cells of the vascular wall. Its radiographic presentation varies from that of intraluminal lesions resulting in obstruction of the inferior vena cava to those of giant retroperitoneal masses extending to the surrounding perivascular tissues although still with minimal intraluminal protrusion. In this report, we present one such case in which a 31-year-old woman had complaints of severe abdominal pain, abdominal distension and vomiting. Computed tomography demonstrated a giant well-defined right-sided retroperitoneal mass extending from the subhepatic region down to the pelvis. She was operated on and the tumor was discovered to be attached to the wall of the inferior vena cava with a peduncle, a leiomyosarcoma being proven histologically. CT features of this rare tumor are presented in this report.

Adult↗