Leiomyosarcoma is a malignant smooth-muscle tumour that has a predilection for the gastrointestinal and female genital tract. It is locally fast-spreading and highly aggressive, and the prognosis is poor. We report a rare case of leiomyosarcoma of the maxilla in a patient who sought treatment for maxillary swelling, nasal obstruction with no epistaxis, orbital involvement or cervical lymph node metastasis. The patient underwent subtotal maxillectomy followed by radiotherapy. At present, he is symptom free with no recurrence and under regular follow up.
BACKGROUND: Leiomyosarcoma of the breast is a rare neoplasm. We present a case of primary leiomyosarcoma of the breast in a middle-aged female in whom fine needle aspiration cytologic features suggested sarcoma.
CASE: A 55-year-old female presented with a rapidly growing breast lump of 1 month's duration. On examination, an ulcerating, 12 x 10 cm tumor was seen involving the lower medial and lateral quadrants of the right breast. Fine needle aspiration cytology showed variably sized, dissociated and loosely clustered polygonal, plump and spindle cells with pale blue cytoplasm and vesicular nuclei that were round, oval or irregular. Occasional giant forms and nucleolated and mitotic cells were present. A single cluster of benign ductal cells was seen. The tumor cells did not express immunocytologic reactivity to estrogen receptor protein. A cytologic diagnosis of sarcoma was given with differential diagnoses of metaplastic carcinoma and malignant phyllodes tumor. Histologic study established the diagnosis of leiomyosarcoma. Leiomyosarcoma of the breast shows fine needle aspiration cytologic features of sarcoma, but specific tumor typing may not be possible, especially when the cytologic material is inadequate for ancillary staining required to distinguish leiomyosarcoma from metaplastic carcinoma and malignant phyllodes tumor.
Leiomyosarcoma is a rare cancer and the presence of this type of cancer in the breast is even rarer. Due to its rarity, the management options for leiomyosarcoma of the breast are not well documented. Literature review was done to establish the best treatment options for this type of breast cancer.
We experienced a rare case of primary leiomyosarcoma of sigmoid mesentery. A 45-year-old woman was presented to us with left iliac fossa mass and discomfort for 4-month duration. CT scan of abdomen and pelvis revealed a huge mass 14 cm×14 cm×16 cm occupying left iliac fossa mimicked having a large left ovarian carcinoma. She was subsequently planned for elective total abdominal hysterectomy and bilateral salpingo-oophorectomy by gynaecology team. During laparotomy, a huge mass was revealed arising from sigmoid mesentery invaded to the left lower ureter. Curative resection was done and pathological findings show the tumour being leiomyosarcoma with immunohistochemistry tests on caldesmon, desmin, smooth muscle actin and CD34 reagent all positive. Clinicopathological and literature review of this rare primary leiomyosarcoma of mesocolon was discussed in our case presentation.
Laryngeal leiomyosarcoma is a rare smooth muscle malignancy of the head and neck region. Diagnosis is based on immunohistochemistry. Here we present a case of laryngeal leiomyosarcoma that was diagnosed and treated in our center, focusing on the clinical features, histological diagnosis and management of this rare disease.
Thoracic sarcomas (TS) are rare neoplasms that may present with non-specific pulmonary symptoms. Chest radiographs and computed tomography of the thorax are the investigations of choice, while pre-operative minimally invasive biopsies may not be entirely helpful with the diagnosis. The commonly identified variants of thoracic sarcomas include liposarcomas, leiomyosarcomas and synovial sarcomas. Complete excision of the tumour with clear margins remain the sole definite form of management and there is limited role with adjuvant chemotherapy and radiotherapy. In the present case series, we explore our experience with three patients who presented with TS and the management decisions that was made.
Uterine leiomyosarcoma (LMS) is rare but primary ovarian LMS is even rarer constituting less than 0.1% of all gynecologic disorders. Neither histologic features nor immunohistochemistry could be utilized to distinguish between uterine or ovarian origin. We illustrate a clinical case of metastatic LMS to the ovary in a woman with underlying uterine fibroid presenting with anemia with heavy menses.
Leiomyosarcoma of the scrotum is a rare tumor. Johnson H Jr in 1987 reported first case. Only 39 cases been reported in the literature. A 74 years old Indian gentleman presented with progressive painless right testicular swelling for 3 months. On examination, a firm right testicular mass. We proceeded with right scrotal exploration with excision of the right scrotal mass. Histopathology showed scrotum leiomyosarcoma. We would like to discuss the rarity of presentation, dilemma of diagnosis and managing in a district hospital. We advocate wide local excision with clear margin of 2 cm which give good overall survival benefits.
Extensive full thickness anterior abdominal wall defects pose a difficult challenge to the reconstructive surgeon. The objectives of reconstruction are the support of the intra-abdominal structures in order to preserve the functional integrity and achieve an aesthetically-acceptable appearance. Autologous tissues are versatile and provide the best reconstructive option in this type of defects. The tensor fascia latae myocutaneous flap provides identical abdominal wall musculofascial cover for full thickness defects. In extensive defects, the extended tensor fascia latae flap is a versatile option with a second microvascular anastomosis at the distal end of the flap. A total anterior abdominal wall soft tissue tumour resection defect was reconstructed with the use of the double pedicle extended free tensor fascia latae myocutaneous flap in a 60-year-old man. The patient however succumbed to the disease process six months post-reconstruction. During the follow-up period, there was no evidence of hernia at the anterior abdominal wall.
Objectives: The effects of water and 50% ethanolic-water extracts of Orthosiphon stamineus Benth (OS) on cell proliferation and apoptotic activity against uterine leiomyosarcoma (SK-UT-1) cells were investigated. Methods: Anti-proliferation effect was evaluated through cell cycle analysis whereas apoptotic activity was determined via screening and quantifying using fluorescence microscopy and flow cytometric analysis, respectively. The effect of extracts on molecular mechanism was studied using real-time reverse transcription polymerase chain reaction and Western blotting. Results: Cell cycle flow cytometric analysis showed the induction of cell cycle arrests were behaves in a p53-independent manner. The examination using fluorescence microscopy and Annexin V flow cytometry revealed the presence of morphological features of apoptotic bodies. Downregulation of anti-apoptotic gene (Bcl-2) supports the apoptotic activity of OS extracts although poorly induce PARP-1 cleavage in Western blot analysis. The extracts also inhibit the SK-UT-1 growth by suppressing VEGF-A, TGF-β1 and PCNA genes, which involved in angiogenesis and cell proliferation. Conclusion: This study demonstrates that O. stamineus extracts are able to inhibit proliferation and induced apoptosis of uterine fibroid cells and is worth further investigation.
Inflammatory Pseudotumor (IP) of bladder is one of a variety of lesions which may arise from spindle cell lesions in the bladder. It is benign in nature compared to the other end of the spectrum of spindle cell lesions, such as sarcoma, sarcomatoid carcinoma and leiomyosarcoma. The diagnosis of IP and its differentiation from a malignant pathology is a diagnostic dilemma for Urologist and Pathologist as both entities share certain similar morphological and histo-pathological appearance as well as immuno-histo chemistry staining. It is however crucial to avoid misdiagnosis as the treatment option varies significantly between a radical or partial cystectomy with future surveillance cystoscopes. Hereby, we highlight a case of a benign variant of spindle cell tumour of bladder which needed second pathology review for confirmation and thus avoiding a radical cystectomy. We also review the literature on its presentation and emphasis characteristics differentiating a benign from a malignant pathology
Leiomyosarcoma of the Inferior Vena Cava (IVC) is a rare soft tissue tumour which accounts for only 0.5% of all soft tissue sarcomas. The patients usually presented with non specific clinical signs and often diagnosed at advanced stage. We reported a case of a 58-year-old lady who presented with right sided abdominal pain for 6 months. CT scan showed large Level 2 IVC tumour which encased the right renal vein and had no clear plane of demarcation with the caudate lobe of the liver. She underwent radical resection of the IVC tumour with the right kidney removed en bloc. Vascular reconstruction was not performed as established collateral veins present. She was nursed in Intensive Care Unit for 2 days and discharged well at day 5 post operatively. Histopathological examination results revealed leiomyosarcoma of IVC.
A 30-year-old, nulliparous woman presented with a history of subfertility. On examination she was found to have uterine fibroid of 28 weeks size of gravid uterus and subsequently laporatomy myomectomy was performed. Multilobulated masses, with diameters ranging from 22 mm to 160 mm were found. Cut sections of the lobulated masses showed whitish whorled cut surface. One of the multilobulated masses had a cystic cavity, measuring 60x50x35 mm(3). Light microscopic findings of the mass with the cystic cavity showed a well-circumscribed cellular tumour composed of cells exhibiting moderate nuclear atypia which were enlarged, nuclei with prominent chromatin clumping and were distributed in areas. Some tumour cells showed large nuclear pseudoinclusions, multinucleated or multilobated tumour giant cells, smudging and few enlarged nucleoli. Mitotic activity was 4 MFs per 10 HPFs. Occasional cells with intracytoplasmic inclusions resembling rhabdoid - like features were seen. There were no atypical mitoses or tumour necroses were noted. Diagnosis of atypical leiomyoma or symplastic leiomyoma was made. Atypical or symplastic leiomyomas are rare in the region of Malaysia and the present case discusses its incidence in younger age, its morphological features along with diagnosis and clinical outcome.
Cancer metastasis to the thyroid gland from non-thyroid sites is a rare presentation in clinical practice. The most frequent primary cancers that metastasise to the thyroid are renal cell carcinoma, followed by colorectal, lung and breast. We report a case of a 64-year-old Malay lady who presented with anterior neck swelling 4 years after an initial diagnosis of uterine leiomyosarcoma. She had undergone a hysterectomy procedure four years ago. Fine needle aspiration cytology of the thyroid mass suggested undifferentiated thyroid carcinoma. After multi-disciplinary discussion, the patient underwent thyroidectomy and the final histopathological diagnosis was metastatic leiomyosarcoma of the thyroid. The diagnosis was aided by an immunohistochemistry panel of positive myogenic markers, negative epithelial markers as well as the previous medical history of uterine leiomyosarcoma. Metastatic leiomyosarcoma of the thyroid may mimic primary undifferentiated (anaplastic) thyroid carcinoma (UTC) with a sarcomatoid pattern, medullary thyroid carcinoma (MTC) with spindle cells morphology and spindle cell tumour with thymus-like differentiation (SETTLE). Hence, a multidisciplinary approach must be practised by pathologists, surgeons and radiologists to consider metastatic lesions of the thyroid gland, especially when a previous history of cancer exists or is suspected.
Introduction: The proto-oncogene c-kit is the cellular homologue of the oncogene v-kit of HZ4 feline sarcoma virus. It is located on chromosome 4 (4q11-12) in the human genome. Interaction between the c-kit receptor and its ligand, stem cell factor, is essential in the development of tissues. C-kit expression has been identified in a number of different neoplasms like seminoma/dysgerminoma, and gastrointestinal stromal tumors (GIST). Recently it has been reported that c-kit is also present in leiomyosarcomas. Tyrosine kinase inhibitors (TKIs) are a promising new therapy in the treatment of cancer. These agents target cellular proteins like kit and its related homologues decreasing cellular proliferation and survival. TKIs may be helpful in treating leiomyosarcomas expressing c-kit. Materials and Methods: In this study a total of 6 cases diagnosed as leiomyosarcomas at Department of Pathology, Universiti Sains Malaysia, Kubang Kerian, Malaysia, were investigated for reactivity for c-kit using immunohistochemical stain. Stain was considered positive if more than 10 percent of the cells showed membrane or cytoplasmic positivity. Results: Two leiomyosarcomas stained faintly with c-kit and in less than 10 percent of the cells. The other 4 cases showed no staining. The control showed good membrane and cytoplasmic positivity. Conclusion: Uterine leiomyosarcomas did not express c-kit. The reason for this could be that the tumors are inherently c-kit negative. More study using larger number of cases is required to validate these findings and further molecular characterization of these mesenchymal tumors is needed to identify the true nature of these sarcomas.