Recurring multifocal leiomyosarcoma of the urinary bladder 22 years after therapy for bilateral (hereditary) retinoblastoma: A case report and review of the literature

We report on a case of urinary bladder leiomyosarcoma in a 23-year-old woman, 22 years after therapy for bilateral retinoblastoma. The tumor presented with dysuria and macroscopic haematuria. Cystoscopy revealed a tumor localized in the trigonum covered by an ulcerated urothelium. The patient underwent a transvesical tumor resection. Eight months later, a second leiomyosarcoma developed in the vertex, at a site different from the previous one. A cystoscopic trans-urethral tumor resection was performed, followed by combined chemotherapy. One year later another recurrence occurred at the site of the primary resection. Open laparotomic resection of the involved bladder wall was performed. The patient remains both recurrence and metastases free after twenty months of follow-up. Molecular analysis of the peripheral blood showed rare germline point mutation in the intron 24 of the RB1 gene. FISH analysis of the tumor tissue revealed polyploid cells with relative loss of normal RB1 gene locus, indicating deletion and second hit loss of the second RB1 allele function. Along with the ten previously reported cases, this report suggests a non-random association between the hereditary retinoblastoma and urinary bladder leiomyosarcoma. Therapy with cyclophosphamide seems to be an important risk factor. Life-long surveillance for second malignancies, including bladder leiomyosarcoma is therefore mandatory in these patients. Keywords: retinoblastoma - urinary bladder - leiomyosarcoma - secondary cancer - cyclophosphamide.     

Leiomyosarcoma of the pulmonary vein

A case of a 74-year-old man with leiomyosarcoma of the pulmonary vein is reported. The patient felt transient chest oppression while playing golf 1 week before he visited a clinic with a common cold. He underwent an ultrasonographic examination of the heart, which showed a mass lesion in the left atrium. The preoperative clinical diagnosis was myxoma of the left atrium. Cardiac surgery revealed the mass to be a leiomyosarcoma, probably extending from the left inferior pulmonary vein. The patient underwent a left lower lobectomy of the lung, and the tumor was confirmed to have originated from the wall of the left inferior pulmonary vein. Although the patient had a metastatic lesion in the right axillary lymph node 11 months later, which was excised, he remained free of disease 14 months after the initial operation. Histologically, the tumors were composed of pleomorphic cells with bizarre nuclei and spindle cells with blunt-ended nuclei with 1-4 mitotic figures in 10 high power fields. Immunohistologically, the tumor cells were positive for alpha-smooth muscle actin and desmin. We reviewed 17 cases of leiomyosarcoma of the pulmonary vein (six males and 11 females with a mean age of 50 years in each group). The present case was the oldest in age and to our knowledge was the first reported case with metastasis in a distant lymph node.     

LEIOMYOSARCOMA OF THE SPERMATIC CORD: A Case Report and a Brief Review of Literature

A case of leiomyosarcoma of the spermatic cord, occurring in a 79-year-old male patient, is reported. The tumor was resected with high inguinal ligation and orchiectomy. It was located at the scrotal part of the spermatic cord on the left side and was shown to have originated from the spermatic duct using the dissecting method. Histologlcally, it was identical to leiomyosarcoma showing a typical morphological appearance of smooth muscle cell tumor in the conventional histochemical preparations as well as a focal positive reaction to antidesmin antibody, a marked nuclear pleomorphism, and abundant mitotic figures. The patient had been untroubled for six months after the resection of the tumor. Leiomyosarcoma arising in spermatic cord is quite rare.     

Leiomyosarcoma of the heart and its pulmonary metastasis, both with prominent osteoclast-like multinucleated giant cells expressing tartrate-resistant acid phosphatase activity

An autopsy case of cardiac leiomyosarcoma and its pulmonary metastasis, both with osteoclast-like multinucleated giant cells (OMGC) mimicking the so-called giant cell variant of malignant fibrous histiocytoma (MFH), is reported. The patient, a 70-year-old male, was admitted for sudden dyspnea. Extensive work-up established only a left atrial tumor mass. Three months after admission, the patient developed multiple intracranial and pulmonary metastases, followed by a worsening clinical course characterized by semicoma and dyspnea, and subsequently died 6 months after the onset of his symptoms. At subsequent autopsy, the left atrial polypoid tumor was found to have invaded destructively to the left half of the cardiac wall. Histology of the cardiac tumor revealed a bimorphic sarcoma in which a poorly differentiated leiomyosarcoma comfirmed by histologic and immunohistochemical findings was juxtaposed to a small nodule with features closely mimicking giant cell MFH. The pulmonary metastatic nodules exhibited features that were entirely indistinguishable from giant cell MFH except for the fact that a minority of polymorphic cells manifested myogenic differentiation. We believe that such a MFH-like pattern represents a pleomorphic form of leiomyosarcoma rather than a dedifferentiated one. The OMGC within the MFH-like component coexpressed CD68 and tartrate-resistant acid phosphatase activity.     

Leiomyosarcoma of the spermatic cord. A case report and a brief review of literature

A case of leiomyosarcoma of the spermatic cord, occurring in a 79-year-old male patient, is reported. The tumor was resected with high inguinal ligation and orchiectomy. It was located at the scrotal part of the spermatic cord on the left side and was shown to have originated from the spermatic duct using the dissecting method. Histologically, it was identical to leiomyosarcoma showing a typical morphological appearance of smooth muscle cell tumor in the conventional histochemical preparations as well as a focal positive reaction to antidesmin antibody, a marked nuclear pleomorphism, and abundant mitotic figures. The patient had been untroubled for six months after the resection of the tumor. Leiomyosarcoma arising in spermatic cord is quite rare.     

Autoimmune hemolytic anemia associated with postirradiation malignant stromal tumor (leiomyosarcoma) of the jejunum

We describe a patient who presented with autoimmune hemolytic anemia and small bowel obstruction secondary to a malignant stromal tumor (leiomyosarcoma) of the jejunum, 25 years postchemotherapy and radiation treatment for stage IIA Hodgkin's disease. The patient was treated with corticosteroid therapy and surgical resection of the jejunal tumor. We conclude that autoimmune hemolytic anemia may be an unusual presentation for postirradiation sarcoma.     

Pararenal leiomyosarcoma of the inferior vena cava

A complete surgical resection is the only proven therapeutic modality that prolongs the survival in patients with leiomyosarcoma of the inferior vena cava (IVC). Reconstruction of the IVC is not always necessary but is often required to facilitate venous drainage of the kidney for the tumors at the pararenal area of the IVC. Controversy exists in postoperative adjuvant therapy. Recently, we experienced four cases of pararenal leiomyosarcoma of the IVC, of which treatment consisted of a complete resection of the tumor, ringed polytetrafluoroethylene (PTFE) graft interposition, and bilateral renal vein reconstructions in all patients. Postoperative radiation therapy was instituted in 3 of 4 patients. One patient who did not receive the postoperative radiation therapy was treated with adjuvant chemotherapy. The kidneys were preserved in all patients and no deep vein thrombosis (DVT) or venous insufficiency of the lower extremity veins developed. Distant metastasis to the lung was noted in one patient at 18 months after surgery, who was not received the postoperative radiation therapy but chemotherapy. In conclusion, a complete resection of the tumor, IVC reconstruction, and bilateral renal vein reconstruction followed by adjuvant radiation therapy is recommended for the treatment of pararenal leiomyosarcoma of the IVC.     

A case of pulmonary mucormycosis presented as Pancoast syndrome and bone destruction in an immunocompetent adult mimicking lung carcinoma

Pulmonary mucormycosis is a rare opportunistic infection caused by Mucormycosis. This fungal infection is uncommon in immunocompetent individuals. Because of its various clinical and imaging manifestations, it is a diagnostic challenge to distinguish pulmonary mucormycosis from other pulmonary diseases, such as carcinoma. Herein, we report a case of pulmonary mucormycosis presenting as Pancoast syndrome and bone destruction of ribs. A 46-year-old Chinese woman was admitted due to pain in chest, right neck and arm for four months and hoarseness for one week. The pre-admission diagnosis via chest CT was pulmonary carcinoma. The subsequent bronchoalveolar lavage fluid analysis and bronchoscopic biopsy were negative for malignant cells, except chronic inflammation. Imaging-guided percutaneous biopsies were carried out after admission and the final pathological diagnosis was pulmonary mucormycosis. Although the patient was started on oral posaconazole of 400 mg bid, the disease condition continued to deteriorate. She finally died of respiratory failure.     

Pulmonary vein myxoid leiomyosarcoma

A case of myxoid leiomyosarcoma located in the right pulmonary veins is presented. The patient complained of progressive dyspnea, orthopnea, sputum cruentum and right chest pain. Angiography revealed an obliteration of right pulmonary veins by a tumor mass that expanded into the left atrium. Histologically, the lesion contained densely packed fusiform cell areas that alternated with other much less cellular and richer in interstitial myxoid matrix. The tumor cells showed specific immunoreactivity to desmin antibodies and contained abundant thin filaments with focal densities and micropinocytic vesicles.     

Clear Cell "Sugar" Tumor of the Lung: A Well-Enhanced Mass with an Early Washout Pattern on Dynamic Contrast-Enhanced Computed Tomography

Clear cell tumor of the lung is a rare and very unusual benign pulmonary tumor. As clear cell tumor of the lung contains abundant cytoplasmic glycogen, this tumor is called "sugar tumor". We report a case of sugar tumor in a 64-yr-old man presenting as a round pulmonary nodule. On dynamic computed tomography (CT) scans, the solitary pulmonary nodule showed early wash-in enhancement with an early washout pattern like a lung malignancy. The patient underwent wedge resection for the tumor. Pathologic examination, including immunohistochemical studies, revealed that the nodule was a benign clear cell tumor, so-called "sugar tumor". Because only a small number of cases have been reported previously, clinical aspects, radiological characteristics on dynamic contrast-enhanced CT, and differential diagnosis of the tumor are not well established. Herein we present a clear cell tumor of the lung and discuss the clinical, radiological, and pathological features of the tumor.     

Primary leiomyosarcoma of bone. A case report and review of the literature

A 62-year-old female with primary leiomyosarcoma of the left femur is reported with a review of 21 cases reported in the literature. The resected specimen showed that the tumor extended from the femoral head to the diaphysis for 13 cm in length. The tumor showed mainly intramedullary proliferation, but extraosseous growth was also noted at the great trochanter. Microscopic examination revealed well differentiated leiomyosarcoma characterized by interlacing bundles of fusiform cells with eosinophilic cytoplasm and rod-shaped hyperchromatic nuclei. PAP stain of actin on the tumor cells was positive. On electron microscopy, microfilament of 6-8 nm in diameter, dense bodies, pinocytotic vesicles, marginal attachment plate, and basal lamina were noted. The patient died with pulmonary metastasis, 1 year and 7 months after the operation. An autopsy showed metastases in the right pelvic cavity and bilateral lungs, and confirmed the primary site to be the left femur.     

PRIMARY LEIOMYOSARCOMA OF BONE

A 62-year-old female with primary leiomyosarcoma of the left femur is reported with a review of 21 cases reported in the literature. The resected specimen showed that the tumor extended from the femoral head to the diaphysis for 13cm in length. The tumor showed mainly intramedullary proliferation, but extraosseous growth was also noted at the great trochanter. Microscopic examination revealed well differentiated leiomyosarcoma characterized by interlacing bundles of fusiform cells with eosinophilic cytoplasm and rod-shaped hyperchromatic nuclei. PAP stain of actin on the tumor cells was positive. On electron microscopy, microfilament of 6–8 nm in diameter, dense bodies, plnocytotic vesicles, marginal attachment plate, and basal lamina were noted. The patient died with pulmonary metastasis, 1 year and 7 months after the operation. An autopsy showed metastases in the right pelvic cavity and bilateral lungs, and confirmed the primary site to be the left femur.     

Epithelioid leiomyosarcoma in a non-immunocompromised infant: additional differential diagnosis of pediatric "round cell tumors".

We report an 18-month-old Japanese girl with purely epithelioid leiomyosarcoma presenting as a huge intraabdominal mass. The patient had been well from birth and had shown no signs of immunodeficiency. She was negative for human immunodeficiency virus. Blood examination revealed elevated serum neuron specific enolase (NSE). Histologically, the tumor was comprised of solid growths of round or polygonal cells with vesicular nuclei and often vacuolated cytoplasm rich in glycogen. The tumor cells were positive for vimentin, NSE, and MIC2, and were negative for desmin and neurofilament. The age, clinical presentation, and histologic findings mostly favored Ewing's sarcoma/primitive neuroectodermal tumor. Silver stain, however, demonstrated well-developed reticulin fibers often outlining individual tumor cells. An expanded panel of immunostains showed that the tumor cells were intensely positive for smooth muscle actin, and ultrastructural study revealed abundant fine cytoplasmic filaments with focal subsarcolemmal densities, various amounts of glycogen, and irregularly arranged, thick basal lamina. The diagnosis of epithelioid leiomyosarcoma was made. Following reduction in tumor size by chemotherapy, the serum NSE level was normalized. From the surgical finding, the primary site was presumed to be the urachus or the urinary bladder dome. Although extremely rare, epithelioid leiomyosarcoma should be added in the list of differential diagnoses of pediatric "round cell tumors."     

Achromobacter xylosoxidans infection presenting as a pulmonary nodule mimicking cancer

Achromobacter xylosoxidans is typically isolated from pulmonary sources, presenting as pneumonia in immunosuppressed individuals. We describe a novel clinical presentation of A. xylosoxidans infection presenting as multiple spiculated, pulmonary nodules mimicking cancer for which the patient underwent a wedge resection of the lung for diagnosis and staging of presumptive cancer.     

Inflammatory pseudotumor and sarcoma of urinary bladder: differential diagnosis and outcome in thirty-eight spindle cell neoplasms.

We assessed diagnostic criteria among 38 spindle cell tumors of the urinary bladder and obtained follow-up in 36 patients. Patients comprised 28 males and 10 females aged 2.5 months to 87 years. Hematuria was the commonest presenting symptom (27 patients). After review and immunohistochemical workup, 17 patients had inflammatory pseudotumor (myofibroblastic tumor), 4 postoperative spindle cell nodule, 1 leiomyoma, 13 sarcoma (7 low-grade; 6 high-grade), and 3 carcinoma. Mean age was 38 years for pseudotumor (range 15 to 74), 65 for postoperative spindle cell nodule, 51 for sarcoma, and 76 for carcinoma. Size of pseudotumor averaged 4.4 +/- 0.7 cm (range 1.5 to 13.0), similar to sarcoma, 4.0 +/- 0.6 cm (range 0.5 to 7.0). Similar proportions of benign tumors and sarcomas had muscularis propria invasion. The criteria that best differentiated sarcoma from inflammatory pseudotumor were presence of necrosis at the tumor-detrusor muscle interface in muscle-invasive cases, and nuclear atypia. Sarcoma also had less prominent microvasculature, less variable cellularity, consistently > or =1 mitotic figure per 10 high-power fields, and predominant acute inflammation without plasma cells. p53 protein nuclear immunostaining was moderate, unlike the rare to absent staining in pseudotumors. Because all 12 sarcomas were desmin-negative, we did not call them leiomyosarcoma; they overlapped with benign tumor in epithelial, mesenchymal, and actin immunostaining. Among 12 sarcoma patients, 2 died of tumor (at 3 months). Two of four experienced tumor recurrence after partial cystectomy (2 and 26 months). No pseudotumors recurred after transurethral resection or partial cystectomy, although one patient, 5 months after transurethral resection, had histologically identical pseudotumor that the surgeon considered residual. Another patient with pseudotumor, not a candidate for tumor ablation after transurethral resection, had continued tumor growth and he died of urosepsis. In conclusion, inflammatory pseudotumor, although overlapping with sarcoma in presentation, age range, and size, does not metastasize and remains histologically distinct from low-grade sarcoma.     

Metastatic uterine leiomyosarcoma to the submandibular gland

Metastasis of uterine leiomyosarcoma to the salivary glands is exceedingly rare. In this report, we present the clinicopathologic features of one such case. The patient was diagnosed with uterine leiomyosarcoma at age 58 years and was treated by hysterectomy only. The tumor was confined to the uterine body. The patient remained free of disease for 4 years and then developed a left submandibular mass. Imaging studies also showed lung involvement but no tumor in the abdomen or pelvis. The submandibular mass was resected; it had histologic and immunohistochemical features similar to those seen in the previous uterine leiomyosarcoma. After 7 months of follow-up, the patient is alive with disease, with progression of disease in the lungs despite receiving chemotherapy.     

Syntopes und synchrones Leiomyosarkom und papilläres Karzinom der Mamma

A 61 years old female was treated by total lumpectomy due to a palpable and radiologically demonstrable tumor measuring 22 cm in diameter in the left breast. Two different but directly neighboured malignant tumours were found: a poorly differentiated leiomyosarcoma and a well differentiated invasive papillary carcinoma of the breast. The latter had metastasized in 4 axillary lymph nodes. Three months after surgery the patient died. Post-mortem studies showed many metastases of the leiomyosarcoma in both lungs, the heart, the liver, the left kidney and both adrenals. Cause of death were relapsing pulmonary thrombembolisms. Case reports of leiomyosarcomas following radiotherapy of the breast cancer are published but coincidental occurrence of both metastasizing tumors are so far not known from the literature.     

肺原发性恶性外周神经鞘瘤的临床病理分析

目的 探讨肺脏原发性恶性外周神经鞘瘤(MPNST)的临床病理学特征及诊断、鉴别诊断要点。方法 对2例肺MPNST进行临床病理学分析及免疫组织化学与超微结构研究。结果 2例光镜下均显示MPNST的形态特点，免疫组织化学显示S-100蛋白、MBP、Vim、NSE肿瘤细胞呈阳性表达，电镜观察1例可见特征性Luse小体。结论 肺原发性MPNST极为罕见，临床诊断较困难。根据其光镜病理形态特征，S-100蛋白、MBP免疫组织化学检测和(或)电镜检查可确诊。结合文献，该肿瘤早期无明显临床症状，一般发现时已属晚期，预后差。病理学上应与肺脏平滑肌肉瘤、纤维肉瘤、单相型滑膜肉瘤、肉瘤样癌鉴别。     

Benign metastasizing leiomyoma of the uterus: histologic and immunohistochemical characterization of primary and metastatic lesions.

Benign uterine leiomyoma metastasizing to the lung is a recognized entity that has been reported infrequently in the medical literature. There is persisting controversy regarding the pathogenesis and biology of these lesions. We report a well-studied and well-characterized case of benign leiomyoma metastasizing to the lung. The patient was a 72-year-old woman with an enlarged uterus that contained several leiomyomas with usual histology. Areas of fibrosis, hyalinization, edema, and focal infarction together with small foci with mildly increased cellularity and minimal nuclear pleomorphism were seen. Careful and repeated mitotic counts ranged from 0 to 2 mitoses per 10 high-power fields. In summary, based on histopathologic criteria, the neoplasm was determined to be a focally cellular benign leiomyoma. Four years later, the patient underwent surgical resection of a single nodule in the lung, which had been detected on routine radiographs. Histopathologic evaluation showed a low-grade leiomyosarcoma with moderate nuclear pleomorphism, necrosis, and brisk mitotic activity. Immunohistochemical studies performed on both neoplasms showed them to be of mesenchymal derivation with smooth muscle differentiation. Both neoplasms expressed estrogen receptors with moderate to strong intensity. The patient received no further treatment and, to date, shows no evidence of recurrent disease. The diagnosis of benign metastasizing leiomyoma can only be made with certainty after careful and extensive sampling of the primary tumor to exclude small foci of sarcoma and of the pulmonary tumor to rule out a primary neoplasm. Although it is biologically peculiar, benign metastasizing leiomyoma should continue to be recognized as a distinct entity because current morphologic criteria do not allow primary myometrial tumors to be reclassified as leiomyomas of uncertain malignant potential even if they have metastasized to the lung.     

Uterine leiomyosarcoma with intracerebral metastasis: a case report.

A case of a 36-year-old woman with a past history of uterine leiomyosarcoma and an intracerebral metastasis is reported. The patient presented with a 24-hour history of severe headache with coma, and the CT findings were consistent with a metastatic lesion. Pathological examination of the operative specimen showed features of a leiomyosarcoma. Uterine leiomyosarcoma is an uncommon tumor and metastasis to the brain is rare.