Multiple Soft Tissue Masses Research Articles

A Case of Type 1 Neurofibromatosis Associated with Multiple Metastatic Gastrointestinal Stromal Tumors

1 Type 1 neurofibromatosis (von Recklinghausen's disease, NF-1) is an autosomal-dominant neurocutaneous- disorder characterized by systemic cafe'-au-lait spots, multiple cutaneous neurofibromas, axillary or inguinal freckling, and Lisch nodules (pigmented iris hamartomas). Approximately 10-25% of NF1 patients have gas- trointestinal neoplasms. Gastrointestinal stromal tumor (GIST) in patients with neurofibromatosis is most commonly found in the small bowel and the stomach, and approximately 60% of such patients have multiple tumors or multiple tumor sites. Although, the increased incidence of GIST in patients with neurofibromatosis is well documented in pathology literature in English, but has rarely been documented in Korea. Here, we report a case of multiple GISTs in a 48-year-old woman accompanied by NF1. She was admitted to Yeung- nam University Hospital with complaints of melena and dyspnea. A contrast-enhanced computed tomog- raphy (CT) scan revealed that multiple soft tissue masses were occupying the entire peritoneal cavity. An ultrasonogram- guided biopsy was performed and the tumors were found to have been composed of tumor cells that were positive for c-kit protein. The patient was put on Imatinib mesylate treatment, and further follow-up will be carried out.

Characteristic imaging features of desmoplastic small round cell tumour

Desmoplastic small round cell tumour (DSRCT) is a rare malignant neoplasm. Its radiological features have rarely been described. To assess the CT parameters characteristic of DSRCT. We also report our experience with combined FDG PET/CT in staging and follow-up for DSRCT. The pretreatment diagnostic CT's of 65 patients with DSRCT were evaluated. Pertinent imaging findings were catalogued, with histopathology or serial follow-up studies as reference standard. Combined FDG PET/CT examinations of 11 of these patients who underwent pretreatment imaging were also reviewed. Sixty-two patients presented with primary intra-abdominal disease; three had primary extra-abdominal tumours at presentation. The most common imaging finding of patients with intra-abdominal DSRCT was multiple peritoneal soft tissue masses, with a dominant mass in the retrovesical or rectouterine location in more than half of the cases. Forty percent had metastatic disease to the liver, lungs, spleen or bones at diagnosis. FDG PET/CT accurately detected 97.4% of all DSRCT lesions. DSRCT typically presents as a large abdominopelvic mass with widespread peritoneal involvement predominantly in young males. Familiarity with its radiological features can help guide diagnosis and treatment. Functional imaging with PET/CT offers advantage over anatomical imaging for accurate disease staging.

Rare pulmonary primitive neuroectodermal tumor metastasizing to the right atrium: a case report

A 28-year-old man complained of intermittent irritable dry cough for 2 months with occasional bloody sputum. Positron emission tomography-computed tomography (PET-CT) suggested multiple heterogeneous soft tissue masses in the inferior lobes of both lungs, with heterogeneous increases in 18F-FDG uptake. No metabolic disorders were found in the rest of the body. CT-guided percutaneous lung puncture and biopsy and immunohistochemical study confirmed pulmonary primitive neuroectodermal tumor (PNET). PNET is characterized by small round blue cells and positive CD99 expression. After six cycles of chemotherapy with ifosfamide, dacarbazine and cisplatin, the lesions diminished substantially. At 2 months after the last cycle of chemotherapy, the patient complained of exertional dyspnea. PET-CT and echocardiogram suggested a space-occupying lesion in the right atrium. Autopsy revealed that this space-occupying lesion had the same pathomorphology and immunophenotype with pulmonary PNET, suggesting metastasis of pulmonary PNET to the right atrium. Here, we reported this rare case of pulmonary PNET metastasizing, instead of direct infiltrating or extending, into the heart.

Long‐term outcome of the treatment of high‐risk tenosynovial giant cell tumor/pigmented villonodular synovitis with radiotherapy and surgery

The treatment of diffuse tenosynovial giant cell tumor (TGCT) requires extensive surgical resection of the hypertrophic synovium and multiple soft tissue masses yet still may result in high rates of local failure. The authors of this report examined their experience in treating patients with advanced/multiply recurrent TGCT with a combination of surgery and external-beam radiotherapy. Fifty patients who were treated for TGCT with radiotherapy and surgery from 1972 to 2006 were identified. Patient demographics, radiotherapy treatment parameters, surgical treatment, and oncologic and functional outcomes were evaluated. All patients had pathologic review at presentation and required at least 1 year follow-up. Forty-nine patients had diffuse TGCT with both intra-articular and extra-articular disease (1 had malignant TGCT). Twenty-eight patients (56%) were referred after at least 1 local recurrence. Thirty patients (60%) underwent at least 2 operations before radiotherapy. The mean dose of radiation delivered was 39.8 gray. At a mean follow-up of 94 months (range, 19-330 months), 47 patients (94%) had not developed a recurrence or had stable disease/signal characteristics on serial cross-sectional imaging (for those patients who had gross residual disease at the time of radiotherapy). Two patients required subsequent total hip arthroplasty because of progressive osteoarthritis, and there were 4 cases of avascular necrosis (only 1 post-treatment). Forty-one patients had good/excellent function. For patients with extensive or multiple local relapses or when surgery alone would result in a large burden of residual disease or major functional loss, the addition of moderate-dose adjuvant radiotherapy provided excellent local control while maintaining good function with low treatment-related morbidity.

Çok Bölgeli Elastofibromanın FDG PET/BT Görüntüsü

lymphoma was sent to whole body F18-flourodeoxyglucose positron emission tomography/computerized tomography (FDG PET/CT) imaging for the initial staging. Mild to moderate hypermetabolic multiple soft tissue masses were observed between the inferior tips of the scapulae and the chest wall and between the gluteal muscles (medius and maximus) and greater trochanters of femur bilaterally in FDG PET-CT (Figure 1,2). For the differential diagnosis of these findings, CT images were consulted to a radiologist and elastofibroma was considered according to characteristic appearance of multiple soft tissue masses. Because of its diagnostic CT pattern, neither invasive procedures nor further imaging was planned for this asymptomatic patient.

Colorectal Burkitt Lymphoma

Purpose: Introduction: Patients with AIDS remain at increased risk for high-grade B cell lymphoma. The incidence of Burkitt lymphoma has dramatically increased since HIV appearance. However, in the era of HAART, only a few cases of colorectal Burkitt lymphoma have been reported these days. Burkitt lymphoma associated with AIDS is characterized by extensive lymphadenopathy and extranodal involvement. Case Presentation: A 32-yearold male with AIDS presented with rectal pain. The patient was noncompliant with HAART, and his last CD4 count was 156 cells per μl 2 months prior. He also admitted suprapubic abdominal pain associated with difficult urination. His vital signs were T 99.1F, HR 110/min, BP 149/85 mmHg, SpO2 98% on room air. Fullness with tenderness was observed in the suprapubic area. Rectal exam revealed a circumferential polypoid mass above the anal junction. Labs were significant for WBC 3800/μl, HGB 10.8 g/dL, PLT 103 K/μl and LDH 1901 U/L. The abdominal CT demonstrated multiple large soft tissue masses throughout the abdomen arising from the small bowel with the largest measuring 8.7 x 15 cm. Right hydronephrosis was also found. CTguided biopsy revealed high-grade B cell lymphoma with Burkitt's feature. Immunohistochemistry was positive for CD45, CD20, CD10, BCL-2, BCL-6, and negative for CD3, CD5, CD23, Cyclin D1. Proliferation index was more than 90%. Colonoscopy showed single large circumferential friable mass in the rectum. The patient was treated with chemotherapy consisting of etoposid, predonisone, vincristine, cyclophosphamide, and doxorubicin (EPOCH) with good clinical response. His rectal/abdominal pain and urinary symptoms improved. Discussion: A majority of AIDS-related lymphomas are high-grade B-cell tumors. Advanced stage at presentation is more frequent in the setting of HIV infection. However, with recent advances in HAART, colorectal involvement with the most aggressive type of B-cell lymphoma, Burkitt lymphoma, is not common. In our case, the patient was noncompliant with HAART and presented with extensive abdominal lymphadenopahy. Generalized lymphadenopathy is one of the main symptoms for Burkitt lymphoma associated with AIDS. Those patients can be presented with bowel obstruction, gastrointestinal bleeding or obstructive uropathy. Therefore, rectal pain or voiding symptoms in HIV patients should raise a suspicion for colorectal involvement of Burkitt lymphoma. In addition, unusual presentation of Burkitt lymphoma warrants an aggressive search for coexisting HIV infection.

Non-skeletal multicentric chondrosarcoma in the hindlimb of a dog

An eight-year-old, spayed, female golden retriever was presented with progressive right hindlimb lameness and polyuria/polydipsia. Multiple soft tissue masses were palpable within the hindlimb muscles. A tentative diagnosis of sarcoma was made on fine needle aspiration. A computed tomography scan of the hindlimb and thorax confirmed the presence and location of the masses, none of which were associated with the bones of the hindlimb. In addition, two pulmonary lesions were identified in the right cranial lung lobe. A diagnosis of chondrosarcoma was confirmed on histopathology with a final diagnosis of extraskeletal chondrosarcoma. A high, hindlimb amputation was performed, and chemotherapy was initiated. Polyuria and polydipsia resolved 2 weeks postoperatively. Numerous lung lesions, suspected to be metastases were found on routine followup radiographs, 73 days post surgery after which the dog was lost to follow-up.

Multiple soft tissue masses in a young female patient presenting six years after radiation therapy for Hodgkin disease

Desmoid fibromatosis is a rare soft tissue tumor, that while often occurring sporadically, is also thought to be related to tissue trauma, hormonal factors, familial adenomatous polyposis syndromes or a late reaction to radiation treatment. Here, it is presented in a young adult who received radiation therapy for Hodgkin lymphoma. Cross-sectional and sagittal images using plain radiography, CT and MR are shown, demonstrating the heterogeneous appearance of these lesions and locally invasive pattern of growth.

Malignant Lymphoma in an Atomic-bomb Survivor

Atomic bomb survivors outside of Japan are few and often hard to follow-up. Spinal malignant lymphoma among these survivors is rare in established studies from Japan or the United States. Here, we report an 81-year-old woman, who experienced the atomic bomb explosion in Nagasaki when she was 19 years old, who presented with papillary thyroid carcinoma when she was 70 years old. Both follicular lymphoma over the right elbow region and vertebral malignant lymphoma were found when she turned 81 years old. Bone scan did not show any increased uptake of isotope. However, thoracolumbar spine magnetic resonance imaging showed multiple infiltrative soft tissue masses involving vertebral bodies at the T10-11 level. Computed tomography-guided biopsy further showed lymphocyte infiltration. Fortunately, the neurological deficit was improved after chemotherapy. The diagnosis of malignant lymphoma in atomic bomb survivors should be more careful and aggressive, even when their bone scan results show negative findings. In addition, the authors suggest that atomic bomb survivors should be followed-up carefully throughout their entire life.

Metastatic Breast Carcinoma to Renal Angiomyolipomas in Tuberous Sclerosis

Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disease manifesting as hamartomatous involvement of several organs, including brain, kidneys, skin, heart, lungs, and retina. We report the first case of a patient with TSC, with large bilateral renal angiomyolipomas (AML), who developed multiple soft tissue masses within her primarily fatty AMLs, resulting from metastatic breast cancer.

Spinal extra-dural metastasis from Merkel cell carcinoma: a rare cause of paraplegia

We report a rare case of Merkel cell carcinoma with extra-dural spinal metastasis causing paraplegia. There are only four reported cases in literature. A 57-year-old lady presented with a breast lump, multiple truncal skin swellings, low back pain and rapidly progressive paraplegia. MRI showed multiple epidural soft tissue masses causing neural compression. A biopsy from the truncal skin lesion was diagnosed as Merkel cell carcinoma (MCC). Posterior decompression and tumor debulking at all three sites of neural compression was performed. Histopathology of the epidural tumor was consistent with MCC and the diagnosis was confirmed by immuno-histochemistry staining for cytokeratin-20. She was started on chemotherapy and radiotherapy. One month after diagnosis she died due to extensive metastasis. The short term palliative response seen in our patient demonstrates the poor prognosis for patients with spinal metastasis.

Subcutaneous panniculitis-like T-cell lymphoma: US and CT findings in three patients

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare disorder. We examined two females and one male with multiple soft-tissue masses in the abdominal wall. One of these three patients also had soft-tissue masses in the right thigh and right buttock. The histologic diagnosis was revealed as SPTCL in all three cases. The ultrasound (US) findings in two of these cases were diffuse, ill-defined hyperechoic areas with a linear vascular signal. The findings of the abdominal and pelvic computed tomography (CT) scanning with contrast enhancement were multiple enhancing nodules with an infiltrative pattern of peripheral subcutaneous fat layer in all three cases. We report US and CT findings of SPTCL in these three patients.

Acute Myeloid Leukemia Presenting as Spinal Cord Compression

We report the findings of a 10-year-old boy who presented to a pediatric emergency department with symptoms of spinal cord compression. Radiological imaging demonstrated multiple soft tissue masses in the head and spine. The surgical pathology showed that the masses were comprised of myeloid leukemia cells. These findings were consistent with those observed on bone marrow aspiration. The characteristics and management of extramedullary leukemia are discussed.

Rosai-Dorfman disease presenting as multiple soft tissue masses

Rosai-Dorfman disease is a rare clinical disorder which may present in many forms. While classically a disease of lymph nodes, soft tissue involvement is fairly common. Soft tissue involvement can occur without any lymphatic or systemic involvement, and may be difficult to diagnose. We describe a patient presenting with multiple soft tissue masses which on biopsy proved to be isolated cutaneous Rosai-Dorfman disease. MR imaging showed two well-defined nonspecific superficial masses that enhanced intensely. Review of the literature suggests that when this disease presents in soft tissue, multiple foci of involvement may be common. Although rare, Rosai-Dorfman disease should be considered in the differential diagnosis of patients presenting with multiple soft tissue masses.

Intra-abdominal desmoplastic small round cell tumors: CT findings and clinicopathological correlations in 13 cases

Purpose: We report computed tomography (CT) findings in 13 patients with a primary abdominal desmoplastic small round cell tumor. Materials and methods: 13 cases (12 men, 1 woman, mean age = 24.8 years) were found in our hospital database between 1991 and 2003. Clinical, CT and histopathological features were studied retrospectively. Results: Peritoneal involvement was the most common feature. In 10 cases, several lobulated peritoneal soft tissue masses (with a mean of four masses per patient) were seen. Two patients had diffused irregular peritoneal carcinomatosis without any distinct peritoneal masses. One patient had a solitary mass in the pelvic space. The main sites of peritoneal involvement were the pelvic space ( n = 7), omentum ( n = 5), retroperitoneal space ( n = 4), small bowel mesentery ( n = 3), paracolic gutter ( n = 2 on the right and n = 1 on the left), transverse colon mesentery ( n = 1), peri-splenic space ( n = 1), peri-hepatic space ( n = 1). The soft tissue masses were often bulky (mean 6 cm, range 1–28 cm), lobulated and heterogeneous with hypodense areas (in 73% of cases). In six cases, moderate ascites was seen. In one case of pelvic involvement, unilateral hydronephrosis was seen. Adenopathies were present in seven cases at the time of the diagnosis (at intraperitoneal, retroperitoneal and pelvic sites in six patients and in the groin in one patient). Five patients had liver metastases (four lesions per case excepted one patient with 30 metastases). Associated thoracic metastases were seen in three patients. The diagnosis was confirmed with four CT-guided percutaneous biopsies. Conclusion: Although CT features are nonspecific, the diagnosis of desmoplastic small round cell tumor may be suspected in young men with multiple bulky heterogeneous peritoneal soft tissue masses. Imaging is useful for staging and also to guide biopsies.

METASTATIC LEIOMYOMATOSIS CAUSING SEVERE LOWER GIBLEED

A 41 year-old woman presented with tachycardia and black, tarry stools. Her past medical history included a diagnosis of “lung fibroids,” myomectomies for large uterine fibroids, and ultimately TAH/BSO. Three weeks after TAH/BSO she had a second operation for hemorrhage into a pelvic mass. The patient was then placed on conjugated estrogen. In March 2003 she required transfusion for hemoglobin (Hgb) of 4. 1g/dl. In June, palpitations awakened her. Blood tests revealed Hgb of 5.31g/dl. Patient was transfused 2 units PRBCs and sent home. Two days later, she again awoke tachycardic and was hospitalized. The patient was pale, but in no acute distress. Temperature was 36.1°C, RR 18, BP 115/60, P 100 without orthostasis. She had no rashes, petechiae or scleral icterus. There was a normal S1 S2, without murmur. Bowel sounds were normoreactive. Abdomen was nondistended, soft, nontender, without palpable masses. Rectal exam revealed guaiac positive black stool. White count was 5.3 k/mm3, Hgb and Hct were 8.1 g/dl and 26.0%, respectively, with MCV of 93.5 fl and RDW of 17.5%. Platelets were 243,000 g/mm3. PT/PTT/INR were normal. An upper and lower endoscopy; small bowel series; three bleeding scans; and a capsule endoscopy study were inconclusive. A chest/abdomen/pelvis CT revealed multiple soft tissue masses within the pelvis, one abutting the sigmoid junction and one abutting the upper rectum; multiple pulmonary nodules increased in size from prior CT in 1998; and a leiomyoma of the inferior vena cava (IVC) without invasion of the right atrium. Tissue from the TAH/BSO was retrospectively tested for estrogen (ER) and progesterone (PR) receptors with positive results. Gastrointestinal bleeding progressed to hematochezia. The patient required 22 units of PRBC's. Exploratory laparotomy revealed soft tissue masses entangling and invading both the ileum and the sigmoid colon. Pathology showed multiple benign hemorrhagic degenerating leiomyomata with vascular invasion. Leiomyomatosis is a rare, histologically benign smooth muscle tumor with a tendency to intravascular invasion. It is infrequently associated with life-threatening symptoms. What is unique to our case is the impressive intravascular invasion of the lower GI tract, which has never been reported in the literature, and which resulted in near-exsanguination. The ER/PR positivity of the tissue supports our hypothesis that estrogen therapy resulted in metastatic leiomyomatosis and the rapid invasion of multiple sites including the peritoneum, IVC, lungs, and GI tract.

Epstein-Barr virus-associated smooth muscle tumours in a patient with an immuno-osseous dysplasia

A term female baby born via a normal delivery to consanguinous parents, presented with a lower respiratory tract infection in the neonatal period. At this time the patient was noted to have a low white cell count associated with short limbs. Immunological investigation revealed lymphopenia and markedly reduced lymphocyte proliferation in keeping with a T-cell immunodeficiency. This episode of lower respiratory tract infection responded well to antibiotic therapy and the patient remained well until 15 months of age. At 15 months of age the patient developed jaundice and was noted to have haemoglobin of 3.0 g/dl, which was treated with transfusion. Laboratory indices revealed an autoimmune haemolytic anaemia. This caused repeated anaemic episodes, requiring blood transfusions on multiple occasions. In an attempt to control the autoimmune haemolytic anaemia, therapy in the form of cyclosporin A and steroids were commenced. The autoimmune haemolytic anaemia was controlled on this regimen until the age of 3 years when it was noted that the patient was developing increasing abdominal distension and breathlessness. Plain film radiography of the limbs was performed to investigate the nature of her limb shortening, which demonstrated generalized osteopenia, delayed bone age and symmetrical limb shortening. The metaphyses were noted to be flared, with under modelled, wide diaphyses and wide ribs. In the hands ivory and cone-shaped epiphyses were seen. These features were consistent with an immuno-osseous dysplasia, most likely to be metaphyseal chondrodysplasia McKusick type (Figs. 1 and 2). An abdominal ultrasound was performed which showed hepatomegaly, with multiple well-defined reduced reflectivity focal lesions throughout both liver lobes, which were hypovascular on colour Doppler. No biliary dilatation was seen and the remaining abdominal viscera had a normal ultrasound appearance. Owing to her breathlessness a chest radiograph was performed which revealed a right lower zone soft-tissue density. Computed tomography (CT) of the thorax was then performed, demonstrating multiple soft-tissue density masses within the lungs. Percutaneous biopsy of both a chest lesion and a liver lesion was performed, revealing the lesions to be leiomyomata. The tumours were demonstrated to be positive for EBV using insitu hybridization (Figs. 3–5). This case presents a grave dilemma for future management. The optimal treatment for the congenital immunodeficiency and auto-immune haemolytic anaemia is ablation of the native bone marrow, followed by bone marrow transplant. The danger of this treatment strategy in this patient is the risk of unchecked proliferation of further smooth muscle tumours, which could become overwhelming. In patients with solitary EBV-associated leiomyomata, surgical excision is a viable option. In this patient with mutiple EBV-driven leiomyomata this is not a treatment option. Currently the patient is not receiving treatment for the tumours, and they are not rapidly progressing.

Radiologic manifestations of granulocytic sarcoma in adult leukemia.

This study was conducted to summarize the clinical and radiologic presentations including pertinent imaging features of granulocytic sarcoma, a rare extramedullary tumor of acute myeloid leukemia, in 11 adult patients. In patients in our study, the clinical and radiologic manifestations of granulocytic sarcoma were variable and occurred most commonly as disease progression in acute myeloid leukemia (73% of the patients). Granulocytic sarcoma lesions were multiple soft-tissue masses with variable enhancement, recurring in nearly 50% of patients at different sites and points of time during the course of the disease. Lesions in the central nervous system, subcutaneous tissues, and genitourinary system accounted for nearly 52% of all lesions. In general, granulocytic sarcoma masses were isodense to muscle on CT scans, and isointense and hyperintense (mild to moderate) on T1- and T2-weighted MR images, respectively. The presence of peripheral rim enhancement with hypodense or hypointense centers on T1-weighted images may, however, mimic an abscess.

두경부에 광범위하게 발생한 기무라씨병 1예

Kimura’s disease, a disease of unknown origin, is a chronic inflammatory disorder presenting with a triad of painless soft tissue masses, regional lymphadenopathy and peripheral eosinophilia but sometimes with pain and tenderness. There are solitary or multiple soft tissue masses or subcutaneous nodules on the head and neck in most cases. Kimura’s disease usually occurrs in younger asian men and has regional localization, but renal manifestations may be the only visceral localization of the disease. We experienced a 38-year-old female patient with much frequently relapsing Kimura’s disease once per month and renal disease, so we report this case with the current literatures. (J Clinical Otolaryngol 2001;12:129-133)

Myositis ossificans progressiva.

In 1692 Patin (1) described the first case of progressive muscle ossification and in 1868 von Dusch (2) gave the disease its present name of myositis ossificans progressiva. A detailed survey of the literature was published by Rosenstirn (3) in 1918, and in 1932 Mair (4) reviewed the subject thoroughly and published two cases of his own. In Mair's report the suggestion was made that the name be changed to “fibrositis ossificans progressiva” because actually the disease involves the connective tissue rather than the muscles. Although this term is more descriptive of the pathological process, the original nomenclature has common usage and will be employed in this paper. Numerous case reports of myositis ossificans progressiva have appeared in the medical literature in the past half century, and yet it remains a medical enigma. Antecedent infection, especially rheumatic fever, has frequently been associated with this disease but there is no proof that the relationship is anything but coincidental. Trauma, although definitely responsible for localized isolated areas of ossified muscle, has not been definitely established as an etiological factor in the progressive type of myositis ossificans. The occasional occurrence of the disease in the same family and the relatively frequent occurrence of the same associated developmental osseous defects in members of the same genetic linkage suggest a familial predisposition. The observation of progressive myositis ossificans in homozygotic twins as reported by Vastine (5) adds substantiating evidence that heredity is influential in the etiology. The clinical features are frequently characteristic. The disease makes its appearance in infancy or early childhood in the form of solitary or multiple soft-tissue masses located, as a rule, on the dorsum of the neck or back. The initial soft-tissue lesions may be firm or soft in consistency and occasionally have a tendency to ulcerate the overlying skin (6). These lesions are frequently painful and may be accompanied by local inflammatory changes. After variable periods, from a few days to several weeks, the inflammatory masses may either regress and disappear or become ossified. Frequently during the course of the illness these soft-tissue tumors shrink suddenly in size, and remission of the entire disease may appear to occur. This change has at times been erroneously attributed to various types of treatment, whereas actually it is characteristic of the disease. Gradually the necrotic reaction and resulting ossification spread to involve the connective tissue both in the intermuscular septa and in fascial planes and tendons until complete immobility of the trunk and upper extremities has occurred. Low-grade fever may occasionally be present during the acute phase of the disease, but this is by no means a constant finding. Congenital osseous anomalies are present almost invariably, the great toes being most frequently involved.