Conjunctival leiomyosarcoma in a patient with xeroderma pigmentosum: 5-year follow-up without recurrence / Leiomiossarcoma da conjuntiva em paciente com xeroderma pigmentoso: acompanhamento de 5 anos sem recidivas

ABSTRACT Conjunctival leiomyosarcoma is a very rare soft tissue malignancy. Herein, we describe a conjunctival leiomyosarcoma case in a patient with another rare disease, xeroderma pigmentosum. The 27-year-old single-eyed xeroderma pigmentosum patient complained of exophytic mass covering the ocular surface in her left eye. A vascular, hemorrhagic mass covering the entire ocular surface of the left eye was identified on the examination. Thus, total mass excision surgery was performed. The pathological diagnosis was compatible with conjunctival leiomyosarcoma. Additional chemotherapy, radiotherapy, or surgery were not accepted by the patient. No recurrence or metastasis was observed during the 5-year follow-up. Both primary conjunctival leiomyosarcoma and xeroderma pigmentosum are very rare diseases. Conjunctival masses in xeroderma pigmentosum patients should be approached carefully, and histopathological examination is warranted. For conjunctival leiomyosarcoma, early diagnosis, localized, unspread disease, and complete resection provide the best prognosis.

RESUMO O leiomiossarcoma da conjuntiva é um tumor ma­ligno de tecidos moles muito raro. Aqui é descrito um caso de leiomiossarcoma da conjuntiva em um paciente com xeroderma pigmentoso, que também é uma doença rara. Um paciente de 27 anos de idade com xeroderma pigmentoso de olho único queixou-se de uma massa exofítica cobrindo a superfície ocular do olho esquerdo. Ao exame, foi observada uma massa vascular hemorrágica cobrindo toda a superfície ocular do olho esquerdo. Foi realizada uma cirurgia de excisão total dessa massa. O diagnóstico patológico foi compatível com leiomiossarcoma da conjuntiva. O paciente recusou qualquer quimioterapia, radioterapia ou cirurgia adicional. Nenhuma recidiva ou metástase foi observada durante o acompanhamento de 5 anos. Tanto o leiomiossarcoma primário da conjuntiva quanto o xeroderma pigmentoso são doenças muito raras. Massas conjuntivais em pacientes com xeroderma pigmentoso devem ser abordadas com cuidado e deve-se realizar um exame histopatológico. Para o leiomiossarcoma conjuntival, o diagnóstico precoce, uma doença localizada e não disseminada e a ressecção completa proporcionam o melhor prognóstico.

FOXO3a deregulation in uterine smooth muscle tumors

Abstract Objective The present study aimed to investigate FOXO3a deregulation in Uterine Smooth Muscle Tumors (USMT) and its potential association with cancer development and prognosis. Methods The authors analyzed gene and protein expression profiles of FOXO3a in 56 uterine Leiomyosarcomas (LMS), 119 leiomyomas (comprising conventional and unusual leiomyomas), and 20 Myometrium (MM) samples. The authors used techniques such as Immunohistochemistry (IHC), FISH/CISH, and qRT-PCR for the present analyses. Additionally, the authors conducted an in-silico analysis to understand the interaction network involving FOXO3a and its correlated genes. Results This investigation revealed distinct expression patterns of the FOXO3a gene and protein, including both normal and phosphorylated forms. Expression levels were notably elevated in LMS, and Unusual Leiomyomas (ULM) compared to conventional Leiomyomas (LM) and Myometrium (MM) samples. This upregulation was significantly associated with metastasis and Overall Survival (OS) in LMS patients. Intriguingly, FOXO3a deregulation did not seem to be influenced by EGF/HER-2 signaling, as there were minimal levels of EGF and VEGF expression detected, and HER-2 and EGFR were negative in the analyzed samples. In the examination of miRNAs, the authors observed upregulation of miR-96-5p and miR-155-5p, which are known negative regulators of FOXO3a, in LMS samples. Conversely, the tumor suppressor miR-let7c-5p was downregulated. Conclusions In summary, the outcomes of the present study suggest that the imbalance in FOXO3a within Uterine Smooth Muscle Tumors might arise from both protein phosphorylation and miRNA activity. FOXO3a could emerge as a promising therapeutic target for individuals with Unusual Leiomyomas and Leiomyosarcomas (ULM and LMS), offering novel directions for treatment strategies.

Primary leiomyosarcoma with osteosarcomatous differentiation of the breast

ABSTRACT Primary leiomyosarcoma with osteosarcomatous differentiation of the breast is an uncommon entity. We present the case of a 37-year-old female who presented with a lump in the breast and pulmonary lesions on PET-CT, for which she underwent a toilet mastectomy. Histopathological examination revealed a tumor with cells arranged in sheets. These tumor cells had moderate eosinophilic cytoplasm, highly pleomorphic, irregular hyperchromatic nuclei, coarse chromatin, and prominent nucleoli. Areas with spindle-cell morphology were noted. Osteoid was seen intermingling with the tumor along with numerous osteoclast-like multinucleate giant cells. A wide panel of Immunohistochemistry was applied, and Desmin, h-Caldesmon, SMA, and Vimentin were positive. The patient died 3 months post-surgery and had a recurrence at the surgical site.

LARYNGEAL LEIOMYOSARCOMA-A RARE CASE REPORT

Mesenchymal neoplasms are sporadic in the larynx accounting for about 1% of all laryngeal malignancies. Leiomyosarcoma is a high-grade tumor of smooth muscle fibres which is very rarely seen in the larynx. The present case report aims to discuss a rare case of laryngeal leiomyosarcoma with special emphasis on immunohistochemical techniques in arriving at the diagnosis.

Spindle cell tumours of the female genital tract

Background: Our aim is to describe the clinicopathology features of spindle cell tumors of the female genital tract seen in our hospital and to compare the findings with what is seen in other parts of the world.Methods: We conducted a cross-sectional study of all spindle cell tumors diagnosed in the department of Morbid Anatomy and Forensic medicine of Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Nigeria from January 1, 2017, to December 31, 2022.Results: Leiomyoma was the most common spindle cell tumour seen. Only 570 cases met the inclusion criteria. Only one nodule was removed in 130 cases (22%) while greater than one nodule was excised in 432 cases (75.8%). The mean age of patients was 40.77 years while the age range was 19-79 years. About three-quarters of leiomyomas are removed in the age-group 31-50. There was no correlation between the number of nodules removed and the age of the patient. The next second most common spindle cell tumor seen was malignant mixed mullerian tumour. Eleven cases were seen in the study period, The average age was 57.7 years. The age range of cases was 27-72 years.Conclusions: Our study supports the general observation that leiomyomas commonly cause symptoms in the reproductive age group and that most patients with leiomyoma frequently have more than one nodule. Our study further reinforces the widely established knowledge that MMMT is found in older post-menopausal women and can occasionally be seen in younger pre-menopausal women.

Primary pulmonary leiomyosarcoma: review of the literature and case report

Primary pulmonary leiomyosarcoma (PPL) is an extremely rare malignant tumor. It has been revealed that PPL may originate from the smooth muscle of the pulmonary parenchyma, pulmonary arteries and bronchi. Patients with PPL may be asymptomatic or present with symptoms similar to those observed in other primary lung tumors. The present study reports the case of a 21-year-old woman who presented with orthopnea, medium effort dyspnea, so an extension study computed tomography (CT) is performed, finding a left thoracic tumor. Where a percutaneous biopsy is performed, concluding PPL, by image, it is considered unresecable due to pericardial infiltration, and it is sent to cycle of chemotherapy. Currently completed treatment with partial response to it, due to findings by image, ECOG and patient age, a case session is decided, and panpleuronectomy was performed.

Interesting malignant renal tumours: a tripod of cases

Renal malignancy is the 15th leading cause of annual deaths, with late detection and misdiagnosis leading to decreased patient survival. We hereby present three cases of malignant renal tumours which grabbed our attention because of their rarity and interesting presentation. Case 1-A 38 year old male presented with palpable lump in right flank, CT revealed a multilocular cystic lesion in right kidney, along with horse-shoe kidneys. In view of renal biopsy suggesting Tubulocystic carcinoma, patient underwent nephrectomy and diagnosis confirmed to be the same histomorphologically as well as immunohistochemically using AMACR and PR. Case 2-A 77-year-old male had lower urinary tract symptoms due to prostatomegaly, and a left renal mass and small hepatic cysts were detected incidentally. Kidney function was normal. Microscopy showed Chromophobe renal cell carcinoma, which was confirmed with immunohistochemical markers CK7 and CKIT. Case 3-A 50 year female presented with recurrent lump in her left flank, CT confirmed a huge left renal fossa mass. Biopsy showed features of malignant mesenchymal neoplasm consistent with leiomyosarcoma based on immunohistochemical panel of vimentin, h-Caldesmon, SMA, EMA, HMB-45 and S100, which helped in differentiating it from tumours like angiomyolipoma. Renal tumours have varied morphological overlapping and it is important to rule out close differentials using immunohistochemistry before coming to a diagnosis. Awareness of such presentations and findings can broaden our understanding of renal tumours which can help in early and accurate diagnosis for better outcome of the patient.

Primary pulmonary leiomyosarcoma: review of the literature and case report

Primary pulmonary leiomyosarcoma (PPL) is an extremely rare malignant tumor. It has been revealed that PPL may originate from the smooth muscle of the pulmonary parenchyma, pulmonary arteries and bronchi. Patients with PPL may be asymptomatic or present with symptoms similar to those observed in other primary lung tumors. The present study reports the case of a 21-year-old woman who presented with orthopnea, medium effort dyspnea, so an extension study computed tomography (CT) is performed, finding a left thoracic tumor. Where a percutaneous biopsy is performed, concluding PPL, by image, it is considered unresecable due to pericardial infiltration, and it is sent to cycle of chemotherapy. Currently completed treatment with partial response to it, due to findings by image, ECOG and patient age, a case session is decided, and panpleuronectomy was performed.

Interesting malignant renal tumours: a tripod of cases

Renal malignancy is the 15th leading cause of annual deaths, with late detection and misdiagnosis leading to decreased patient survival. We hereby present three cases of malignant renal tumours which grabbed our attention because of their rarity and interesting presentation. Case 1-A 38 year old male presented with palpable lump in right flank, CT revealed a multilocular cystic lesion in right kidney, along with horse-shoe kidneys. In view of renal biopsy suggesting Tubulocystic carcinoma, patient underwent nephrectomy and diagnosis confirmed to be the same histomorphologically as well as immunohistochemically using AMACR and PR. Case 2-A 77-year-old male had lower urinary tract symptoms due to prostatomegaly, and a left renal mass and small hepatic cysts were detected incidentally. Kidney function was normal. Microscopy showed Chromophobe renal cell carcinoma, which was confirmed with immunohistochemical markers CK7 and CKIT. Case 3-A 50 year female presented with recurrent lump in her left flank, CT confirmed a huge left renal fossa mass. Biopsy showed features of malignant mesenchymal neoplasm consistent with leiomyosarcoma based on immunohistochemical panel of vimentin, h-Caldesmon, SMA, EMA, HMB-45 and S100, which helped in differentiating it from tumours like angiomyolipoma. Renal tumours have varied morphological overlapping and it is important to rule out close differentials using immunohistochemistry before coming to a diagnosis. Awareness of such presentations and findings can broaden our understanding of renal tumours which can help in early and accurate diagnosis for better outcome of the patient.

Tratamiento quirúrgico del leiomiosarcoma de vena cava inferior

Introducción: La baja incidencia del leiomiosarcoma de la vena cava inferior dificulta tanto la estandarización del diagnóstico como el tratamiento. Objetivo: Presentar el manejo realizado en nuestro centro de un paciente que desarrolló un leiomiosarcoma de vena cava inferior, una patología de baja incidencia y que las posibilidades de realizar un rescate quirúrgico son muy bajas. Resultados: Se presenta el caso de un paciente de 54 años con una tumoración sólida en porción infrarrenal y yuxtarrenal de vena cava inferior de 71 × 76 × 117 mm compatible con leiomiosarcoma de vena cava, con infiltración de uréter derecho que ocasiona uropatía obstructiva derecha grado I-II sin alteración de la función renal, que fue resecada y reconstruida mediante prótesis sin complicaciones. Discusión: Se discute la fisiopatología, el diagnóstico y manejo en relación con el caso presentado. Conclusión: la baja incidencia de estos tumores dificulta tanto la estandarización del diagnóstico como del tratamiento, aunque la cirugía sigue siendo el tratamiento de elección.

Introduction: The low incidence of leiomyosarcoma of the inferior vena cava hinders both the standardization of diagnosis and treatment. Objective: To present the management carried out in our center of a patient who developed an inferior vena cava leiomyosarcoma, a low incidence pathology with uncertain surgical rescue. Results: 54-year-old patient with a solid tumor in the infrarenal and juxtarenal portions of the inferior vena cava of 71 × 76 × 117 mm compatible with leiomyosarcoma of the vena cava, with infiltration of the right ureter that causes right obstructive uropathy grade I-II without kidney function changes; tumour was resected and continuity reconstructed with a prosthesis without complications. Discussion: The pathophysiology, diagnosis and management are commented. Conclusion: the low incidence of these lesions makes it difficult to standardize both diagnosis and treatment, although surgery remains the treatment of choice.

Uterine Epithelioid Leiomyosarcoma Arising Within Leiomyoma in A Postmenopausal Woman: A Case Report

@#Uterine leiomyosarcoma is a malignant smooth muscle tumour of the uterus. It is rare and accounting for less than 2% of cases in malignant gynaepathology. To date, only a few reported cases of leiomyosarcoma arising from leiomyoma documented in the literature. We shared an uncommon occurrence of leiomyosarcoma arising from leiomyoma. Presented herein is a case of a ‘rare epithelioid subtype’ of leiomyosarcoma arising from a leiomyoma in a postmenopausal woman. We highlighted the importance of recognizing the possibilities of this event to allow for a timely diagnosis of leiomyosarcoma and to provide insights on management of patients presented with clinically presumed fibroid.

Robot-assisted laparoscopic enucleation in the treatment of leiomyosarcoma of urinary bladder: A case report / 中南大学学报(医学版)

Leiomyosarcoma of urinary bladder (LMS-UB) is a highly malignant mesenchymal tumor, accounting for less than 0.5% of all bladder malignancies, with a predominant clinical presentation of hematuria. Here we report a case of low-grade LMS-UB. A 44-year-old male patient was admitted to the hospital with urodynia for 2 weeks. The patient's pelvis CT showed a mass on the right part of the bladder. For this reason, he was initially diagnosed with bladder cancer. We performed a robot-assisted laparoscopic enucleation of the bladder tumor and low-grade LMS-UB was diagnosed with the histopathological examination. He underwent 5 cycles of adjuvant chemotherapy after surgery. At 19months postoperative follow-up, the patient had no symptoms, recurrence, or distant metastasis. There is no report on the treatment of LMS-UB with minimally invasive enucleation worldwide. This case provides a new comprehensive treatment method of enucleation combined with adjuvant chemotherapy for early low-grade LMS-UB to reduce complications and improve patients' quality of life after surgery.

Primary leiomyosarcoma of the prostate complicated with acinar adenocarcinoma: a case report / 中华泌尿外科杂志

Primary leiomyosarcoma of the prostate is rare.A case of prostate leiomyosarcoma complicated with acinar adenocarcinoma in a 68-year-old man was reported. The patient's PSA was abnormally elevated, and MRI suggested prostate malignant tumor. Leiomyosarcoma was diagnosed by transperineal prostate biopsy and laparoscopic radical prostatectomy was performed. The diagnosis was confirmed by postoperative pathological examination. There was no recurrence or metastasis after 20 months of follow-up.

Ressecção cirúrgica de leiomiossarcoma de veia cava inferior retro-hepática sem reconstrução vascular: relato de caso / Surgical resection of retrohepatic inferior vena cava leiomyosarcoma without vascular reconstruction: case report

Resumo O leiomiossarcoma de veia cava inferior (LVCI) é um raro tumor maligno mesenquimal. Seu tratamento cirúrgico é um desafio, pois necessita combinar margens cirúrgicas livres com reconstrução vascular, usando prótese ou enxerto autólogo, sutura primária ou ligadura simples sem reconstrução da veia. A ligadura é possível graças ao lento crescimento do tumor, permitindo o desenvolvimento de circulação venosa colateral. Apresentamos um caso de LVCI tratado por ressecção radical sem reconstrução vascular. Paciente feminina, 48 anos, com dor abdominal em hipocôndrio direito, astenia e sintomas dispépticos pós-prandiais. Tomografia de abdome revelou massa de formação expansiva localizada no segmento infra-hepático da veia cava inferior com redução da luz do vaso. Na cirurgia, o clampeamento da veia não indicou repercussões hemodinâmicas, sugerindo formação de circulação colateral suficiente. Decidiu-se pela ressecção radical em toda a porção da veia cava retro-hepática e ligadura da veia cava sem reconstrução vascular. A paciente evoluiu sem intercorrências.

Abstract Inferior vena cava leiomyosarcoma (IVCL) is a rare malignant mesenchymal tumor. Surgical treatment is a challenge because it must combine free surgical margins with vascular reconstruction, using prosthetic or autologous grafts, primary suture, or simple ligation without vein reconstruction. The ligation option is possible thanks to the slow growth of the tumor, allowing collateral venous circulation to develop. We present a case of an IVCL treated with radical resection without vascular reconstruction. The patient was a 48-year-old female with abdominal pain in the right upper quadrant, asthenia, and postprandial dyspeptic symptoms. Abdominal tomography revealed a mass with an expansive formation located in the infrahepatic segment of the inferior vena cava and reduced vessel lumen. During surgery, vein clamping did not provoke hemodynamic repercussions, suggesting sufficient collateral circulation formation. It was decided to perform a radical resection of the entire portion of the retrohepatic vena cava and ligate the vena cava without vascular reconstruction. The patient recovered without complications.

Leiomiosarcoma de vena cava inferior, reconstrucción mediante interposición de prótesis y anticoagulación / Leiomyosarcoma of the inferior vena cava, reconstruction with interposition of prosthesis and anticoagulation / Leiomiossarcoma da veia cava inferior, reconstrução por interposição de prótese e anticoagulação

El leiomiosarcoma primario de vena cava es una neoplasia poco frecuente originada a nivel de las células musculares lisas de la túnica media. Representa el 2% de todos los leiomiosarcomas y el 60% de los tumores de vena cava. Presentamos el caso clínico de una paciente de 64 años que consulta por dolor lumbar derecho. La tomografía computada evidencia una gran masa retroperitoneal que engloba la vena cava inferior y contacta con el riñón derecho; el estudio histopatológico de la biopsia certifica un leiomiosarcoma. Se realiza tratamiento quirúrgico mediante resección tumoral con vena cava inferior e interposición de prótesis sintética y nefrectomía derecha. El estudio anatomopatológico de la pieza certifica un leiomiosarcoma de alto grado sin compromiso de la cápsula renal con bordes de resección libres. La resección radical con márgenes negativos actualmente ofrece la mejor tasa de supervivencia. Aún se encuentra en discusión la indicación de la anticoagulación en pacientes con colocación de prótesis.

Primary leiomyosarcoma of the vena cava is a very rare neoplasm that originates from the smooth muscle cells of the tunica media. It represents 2% of all the leiomyosarcomas and 60% of the vena cava tumors. We report a case of 64-year-old patient consulting for a lower right back pain. The computed tomography shows a large retroperitoneal mass which encompasses the inferior vena cava and involves of right kidney. The surgical treatment of tumor resection is performed with right nephrectomy and interposition of Dacron prosthesis. The anatomopathological study shows a high-grade leiomyosarcoma, not compromising the renal capsule, with resection free. Radical resection with negative margins currently offers the best survival rate. Anticoagulation treatment for patients with prosthesis placement is still under discussion.

O leiomiossarcoma primário da veia cava é uma neoplasia rara originada das células musculares lisas da túnica média. Representa 2% de todos os leiomiossarcomas e 60% dos tumores de veia cava. Apresentamos o caso clínico de um doente de 64 anos que consultou por lombalgia direita. A tomografia computadorizada revelou grande massa retroperitoneal que envolvia a veia cava inferior e contatava o rim direito; o estudo citológico da biópsia revelou um leiomiossarcoma. O tratamento cirúrgico foi realizado por excisão tumoral e de veia cava inferior com interposição de prótese sintética e nefrectomia direita. O diagnóstico anatomopatológico foi leiomiossarcoma de alto grau sem envolvimento da cápsula renal com bordas de ressecção livres de patologia. A ressecção radical com margens negativas atualmente oferece a melhor taxa de sobrevida. A indicaçãode anticoagulação em pacientes com colocação de prótese ainda está em discussão.

Primary leiomyosarcoma of ovary: A rare malignancy as an incidental finding

Primary leiomyosarcoma (PLMS) of the ovary is extremely rare tumors comprising 1% of ovarian tumors. About 3% of all ovarian malignancies are primary ovarian sarcomas. Only 72 cases have been reported till date. A 57-year-old postmenopausal female presented with abdominal pain for the last 6 months. Ultrasonography and MRI revealed a heterogeneously enhancing solid lobulated mass in the left adnexa abutting the fundus of the uterus and bowel loops. The endometrial cavity was normal. Ovarian markers CA 125, CEA, CA 19.9, and all hematological parameters were within normal limits. LDH was near normal (284 IU/ml). The specimen was sent for frozen section and a diagnosis of malignant spindle cell lesion of ovary was rendered. Histopathology of the ovarian mass revealed intersecting fascicles of tumor cells consisting of ovoid to spindle-shaped cells having a moderate amount of cytoplasm. Bizarre and atypical cells were seen singly dispersed and in small aggregates along with the brisk mitotic activity. Focal areas of necrosis and hemorrhage were also noted. Immunohistochemistry showed strong positivity for smooth muscle actin and Caldesmon while focal positivity for Desmin and Epithelial Membrane Antigen (EMA) was noted. The lesion was negative for Inhibin, Calretinin, and CD 117 and S100. The final diagnosis of primary ovarian Leiomyosarcoma was given based on histopathology and Immunohistochemistry. PLMS of the ovary are rare incidental findings in postmenopausal women. These are highly malignant tumors and carry a poor prognosis. Hence, early diagnosis and surgical treatment with cytoreduction improve patient survival.

Leiomioma submucoso de gran tamaño: una presentación inusual. Reporte de Caso / Large submucosal leiomyoma: an unusual presentation

Se ha postulado que más del 70 % de las mujeres antes de la menopausia son diagnosticadas de leiomiomas; de estas un 25 % presentan sintomatología grave. La ecografía es la modalidad de imagen de primera elección para su estudio. Se tiene registros de progresión de leiomiomas a leiomiosarcomas, que conllevan a un mal pronóstico y son responsables de una cuarta parte de las muertes por neoplasias uterinas, con una sobrevida a 5 años que van desde 46-53 %, lo cual incentiva el diagnóstico precoz y eficaz de masas uterinas. El caso reportado es una presentación inusual de un leiomioma de gran tamaño, que por los hallazgos de imagen se reportó como una neoplasia maligna, su aspecto macroscópico totalmente atípico reforzaba la sospecha imagenológica. Solo se pudo determinar el diagnóstico definitivo mediante estudio histopatológico posterior a histerectomía. Las evaluaciones ginecológicas de rutina deben incluir exámenes especializados de imagen pélvica, la ausencia de presentación típica o síntomas característicos de una patología no debe ser motivo para obviar exámenes complementarios que permitan un diagnóstico oportuno y tratamiento eficiente de leiomiomas, incluso en casos de presentaciones inusuales y desafiantes para el diagnóstico, como el que se ha reportado en esta obra.

It has been postulated that more than 70% of premenopausal women are diagnosed with leiomyomas; Of these, 25% have severe symptoms. Ultrasound is the imaging modality of choice for its study. There are reports of progression from leiomyomas to leiomyosarcomas, which leads to a poor prognosis, and is responsible for a quarter of deaths from uterine neoplasms, with a 5-year survival ranging from 46-53%; this should encourage early and effective diagnosis of uterine masses. The reported case is an unusual presentation of a large leiomyoma, which due to its imaging characteristics, was reported as a malignant neoplasm. Its completely atypical macroscopic appearance reinforced the imaging diagnosis suspicion. The definitive diagnosis could only be determined by histopathological study after hysterectomy. Routine gynecological evaluations should include specialized pelvic imaging exams, the absence of a typical presentation or characteristic symptoms of a pathology should not be a reason to obviate ancillary testing that would allow a timely diagnosis and effective treatment of leiomyomas, even in unusual and challenging presentations, such as with the patient in this case report.

Inferior vena cava leiomyosarcoma with liver metastasis at presentation in a young male: A challenging diagnostic quandary

Leiomyosarcomas of vascular origin are very rare tumors, predominantly affecting the inferior vena cava (IVC). Although vascular leiomyosarcomas are slow-growing, their non-specific and late presentation results in delayed diagnosis which portends a very poor prognosis. Here we report a case of a 24-year-old man who presented with abdominal pain since 15 days and was found to have unresectable metastatic leiomyosarcoma of the inferior vena cava at initial diagnosis.

Primary Leiomyosarcoma of Liver � Case Report of a Rare Cancer

Primary Hepatic Leiomyosarcoma are very rare Tumours with only 30 cases reported Worldwide1. Patient complains of nonspecific symptoms and often presents with Enlarged Liver. Diagnosis is usually delayed and patients have poor prognosis2. 35-year-old woman presented to Outpatient Department (OPD) complaining of dull aching pain and mass in upper part of abdomen for 6 months. On palpation liver was enlarged and lower border extended up to umbilicus. Ultrasonography of abdomen showed Liver enlarged 23 cm with multiple hypoechoic nodules noted in both lobes of liver. Triphasic Contrast-Enhanced Computed Tomography (CECT) abdomen multiple variable sized Peripherally Enhanced Hypodense Nodules in both lobes of Liver suggestive of Multifocal Hepatocellular Carcinoma (HCC)/ Metastasis. Tumour markers S alpha fetoprotein, S. CA 19-9, S. CEA was within normal limits. CECT Chest was normal. Positron Emission Tomography and Computed Tomography (PET-CT) was done, which showed Liver enlarged 24.5cm with FDG avid multiple nodules in both lobes of Liver and multiple enlarged FDG avid periportal, Portocaval and Peripancreatic Lymph Nodes. Rest of body organs were normal. Biopsy from the Liver showed Mesenchymal Tumour composed of spindle cells arranged in fascicular growth pattern, nucleus cigar shaped with atypia and mitotic figures seen. On Immunohistochemistry (IHC) Tumour was positive markers were Smooth Muscle Actin (SMA), VM, Desmin and H-caldestron and negative for CK, S-100, SOX-10, CD-117 and Dog-1. From above work up a diagnosis of primary Leiomyosarcoma of Liver was made. Palliative Chemotherapy was offered to the patient.