Primary desmoid tumor of the posterior mediastinum

We describe a unique case of a de novo desmoid tumor of the posterior mediastinum in a 21-year-old nulligravida. The tumor recurred twice despite its histologically confirmed radical extirpations. Because of the aggressive local behavior of desmoid tumors and their unusual locations, which prevent wide excision margins, we favor the early consideration of adjuvant therapy.     

Giant recurrent desmoid tumor: a case report

A case of giant recurrent intra-abdominal desmoid tumor is presented. A history of childbirth, antecedent surgery, multiple episodes of recurrence, resistance to excisional and radiation therapy, represent common features of desmoid tumors. The size of the recurrence (15.4 kg), the intra-abdominal presentation of the tumor, involvement of the chest wall and focal infiltration of the small bowel are unusual features of this case.     

Congenital desmoid tumor of the scalp: a histologically benign lesion with aggressive clinical behavior

Desmoid tumors are uncommon benign neoplasms of fibroblastic origin, frequently presenting in the abdomen or abdominal wall of adults. This tumor is well known to general surgeons and pathologists, but it has rarely been reported in the neurosurgical literature. We describe a neonate who presented with a right temporal subscalp tumor at birth. In spite of initial apparent total removal, the tumor recurred twice. We discuss the diagnostic pitfalls encountered in assessing this tumor and the difficulties in distinguishing, in the excised specimens, between desmoid and reactive fibrosis. We suggest that desmoid tumor should be considered in the differential diagnosis of scalp and calvarial masses in children. Complete excision is required to prevent recurrences, and sometimes a multidisciplinary team is needed to secure margin-free resection.     

High ratios of free to total insulin-like growth factor-I in early infancy

A case of huge desmoid tumor successfully treated by hyperthermoradiotherapy is described. A 23-year-old man with familial adenomatous polyposis was operated upon for a desmoid tumor in the mesenterium involving the right kidney and small intestine in 1988. In 1990, the tumor recurred and could not be resected because of the involvement of the vena cava. The tumor grew larger and larger, and occupied two-thirds of the right lower quadrant. Several therapies using sulindac, tamoxifen, prednisolone, indomethacin, luteinizing hormone-releasing hormone analogue, and ascorbate were all ineffective. Finally, the combination of radiation and hyperthermia was used over a 6-month period. At the end of the hyperthermoradiotherapy, the tumor in the abdominal wall was markedly reduced in size, and the protruded abdominal wall became flat. To our best knowledge, this is the first report of the successful treatment of a huge desmoid tumor by hyperthermoradiotherapy.     

Management of desmoid tumors in patients after ileal pouch-anal anastomosis for familial adenomatous polyposis

PURPOSE: The aim of this study was to review the management of desmoid tumors in patients who have undergone ileal pouch-anal anastomosis for familial adenomatous polyposis and their outcome. METHODS: A retrospective review of case notes was undertaken in a series of 196 patients with familial adenomatous polyposis who had had an ileal pouch-anal anastomosis. Eleven patients were identified in whom desmoid tumor had developed (4 male; median age, 30 (range, 16-43) years). RESULTS: Desmoid tumors developed in the abdominal wall alone in 4 of 11 patients. Three enlarging tumors were resected with no subsequent recurrence. No patient received adjuvant treatment and pouch function remained stable at a median follow-up of eight years. Mesenteric desmoid tumors developed in seven patients; the pouch has been excised in two because of ischemia; two other patients have had defunctioning stomas created because of slow progression of their desmoid tumors. One patient had a single-lumen pouch excised and a new S-pouch inserted, with no subsequent recurrence of the desmoid tumor, and in two patients the mesenteric desmoid tumor has remained unchanged. All of these patients received long-term Clinoril (sulindac, Merck & Co., Inc., West Point, PA). Although there has been no significant deterioration in pouch function in patients in whom the pouch remains in situ, the pouch function was significantly worse compared with a control group of patients after ileal pouch-anal anastomosis for familial adenomatous polyposis in whom desmoid tumors did not develop. CONCLUSION: Desmoid tumors developed in 5.6 percent of 196 patients after ileal pouch-anal anastomosis for familial adenomatous polyposis. No patient with abdominal-wall desmoid tumor had a recurrence after local excision. By contrast, mesenteric desmoid tumors eventually resulted in pouch failure in four of seven affected patients.     

Predictors of outcome of forefoot surgery for ulceration and gangrene

Desmoid tumors are fibrotic neoplasms of low metastatic potential. These tumors have long been associated with major laparotomy incisions. However, to the best of our knowledge, they have not been previously associated with laparoscopic trocar placement. This report involves the case of a patient who developed a desmoid tumor at a trocar site. A 35 year-old premenopausal woman presented with an enlarging infraumbilical mass 10 years after a laparoscopic tubal ligation. The mass was immediately subjacent to the skin incision for the laparoscopic procedure. It was approximately 6 cm in diameter and fixed to the underlying abdominal wall. It was evaluated with sonographic and CT imaging, which revealed a solid mass arising from the fascia with no associated hernia. A biopsy of the mass confirmed a diagnosis of a desmoid tumor. This was subsequently excised with a wide margin and reconstructed with Marlex mesh. The patient is without recurrence 2 years following surgery. This case represents what we believe to be the first report of a desmoid tumor arising from a incision made for the placement of a laparoscopic port. Rapidly increasing laparoscopic applications make it likely that these lesions will increase in frequency. In this setting, resection with wide margins remains the mainstay of treatment for desmoid tumors.     

Abdominal desmoid tumor

Two cases of desmoid tumor of the abdominal wall are reported, together with some considerations and a review of the literature; desmoid tumor is an unusual neoplasm which affects women in prevalence. This is an histologically benign neoplasm with no metastatic capacity; however it is highly invasive in the site where it arose, so its treatment can become very difficult. The treatment is by surgical excision that must be as wide as possible, in order to obviate the great incidence of recurrence. The role of radiotherapy, hormone and chemotherapy has not been completely assessed so far.     

Desmoid tumor of the thoracic wall. A case manifesting as painful stiffness of the shoulder

BACKGROUND: Desmoid tumors are uncommon fibromatous tumors arising from musculoaponeurotic tissue characterized by spindle cell fibroblast and myofibroblast proliferation. The cause is unknown (trauma, hormonal factors, genetic anomaly...). Locally invasive, they tend to recur increasing morbidity or even mortality. CASE REPORT: Stiff shoulder resulting from blockage of the scapulothoracic articulation was the inaugural sign. Three-phase bone scintigraphy demonstrated early uptake in the soft tissue in contact with the scapula. Magnetic resonance imaging and biopsy confirmed the diagnosis of desmoid tumor. Complete resection was performed. No adjuvant radiotherapy nor hormone treatment were given since no antiestrogen receptor antibodies were identified on the surgical specimen. DISCUSSION: The clinical manifestation (stiff shoulder) and the scapular localization observed in this case are unusual for desmoid tumors. Initially tendinopathy of the shoulder, acromio-clavicular arthropathy and capsular retraction of the shoulder joint had been entertained. Early diagnosis and wide surgical resection are indicated in desmoid tumors.     

Collagen and elastin synthesis by desmoid tumor in vitro

In order to characterize human desmoid tumors in vitro, the production of collagen and elastin and the expression of collagen types alpha1(I), alpha1(III) and transforming growth factor (TGF)-beta1 mRNA were investigated in six desmoid tumors; five derived from familial adenomatous polyposis patients and one from a sporadic case. The proportion of collagen production to total protein production was determined by 3H-imino acid incorporation, an indicator of collagen synthesis, using high-performance liquid chromatography (HPLC). The proportion of collagen production to total protein production was much higher in all six desmoid tumors compared with human skin fibroblasts (HSF). Quantitatively, the rate of elastin synthesis in desmoid tumor cells monitored by valine-proline peptide was also significantly higher than in HSF. Pro-alpha1(I) collagen mRNA was highly expressed in both desmoid tumors and HSF at approximately the same level, whereas pro-alpha1(III) collagen mRNA was more abundant in some of the desmoid tumors than the normal skin fibroblastic cell lines. Tumor growth factor-beta1 mRNA, which is believed to stimulate collagen synthesis, was expressed in both desmoid tumors and HSF to the same extent. These results demonstrate the increased formation of collagen and elastin in desmoid tumors in vitro and suggest that the increased synthesis of elastin rather than of collagen and TGF-beta1 may be involved in increased fibrogenesis by desmoid tumors.     

Informative value of Hemoccult test according to the number of positive slides in mass screening of colorectal cancer]

Desmoid tumors develop in the muscle or aponeurosis, but rarely in the chest wall. We report a patient with desmoid tumor in the chest wall. A 30-year-old female visited our hospital due to an abnormal shadow in the chest detected at a health examination. No definite diagnosis could be made by chest CT, MRI, or Ga scintigraphy, and finally a biopsy demonstrated desmoid tumor. The left anterior chest and posterolateral chest (lung apex) were opened, and tumors were resected together with the chest wall. No radiotherapy was performed. No recurrence has been observed for 1.5 years after operation. This tumor does not yield distant metastasis, but its recurrence rate is very high. Therefore, we intend to follow this patient for a long period.     

Fertility and schizophrenia: evidence for increased fertility in the relatives of schizophrenic patients

BACKGROUND AND PURPOSE: We present a rare case of desmoplastic fibroma of the skull. Desmoplastic fibroma is a distinctive and rare neoplasm of bone that histologically and biologically mimics desmoid soft tissue tumor. Only 6 cases have been reported in the skull and none of these was diagnosed by MRI. CLINICAL PRESENTATION: A 64 year-old woman, operated on in June 1991 for left mastectomy due to a ductal adenocarcinoma and again in October 1994 for a left frontal metastatic adenocarcinoma, was admitted in our Department of Neurosurgery because a control cerebral MRI had detected a diploic lesion, isointense on T1-weighted images and hyperintense on T2, with moderate enhancement, localized in a right parietal site. Neurological examination was negative. The lesion was surgically removed and a cranioplasty was performed. Histological diagnosis was desmoplastic fibroma. Twelve months after treatment she has no neurological symptoms or signs of cerebral lesions (MRI) or systemic metastasis (total body CT). CONCLUSION: In the literature the number of desmoplastic fibroma is too small and the follow-up period too short to permit any conclusions regarding the aggressiveness of the tumor.     

Bilateral elastofibroma: a case report and review of the literature

Elastofibroma, or elastofibroma dorsi as it was first described, is an uncommon tumor-like process that characteristically appears as an ill-defined mass in the infrascapular region of elderly patients. This lesion can occur bilaterally and may appear asynchronously. The occurrence of elastofibroma in other anatomic sites has been reported, although with much less frequency. Elastofibromas display typical diagnostic histologic, cytologic, and electron microscopic features. The use of magnetic resonance imaging can lead to a presumptive diagnosis in elderly individuals with suprascapular lesions. It is important to differentiate this lesion from other soft-tissue lesions, such as sarcomas and desmoid tumors. This study presents a case of bilateral elastofibromas in a 72-year-old man and a review of the literature.     

Radiation therapy in the management of desmoid tumors

PURPOSE: To evaluate the outcome of patients with extra-mesenteric desmoid tumors treated with radiation therapy, with or without surgery. METHODS AND MATERIALS: The outcome for 75 patients receiving radiation for desmoid tumor with or without complete gross resection between 1965 and 1994 was retrospectively reviewed utilizing univariate and multivariate statistical methods. RESULTS: With a median follow-up of 7.5 years, the overall freedom from relapse was 78% and 75% at 5 and 10 years, respectively. Of the total, 23 patients received radiation for gross disease because it was not resectable. Of these 23 patients, 7 sustained local recurrence, yielding a 31% actuarial relapse rate at 5 years. Radiation dose was the only significant determinant of disease control in this group. A dose of 50 Gy was associated with a 60% relapse rate, whereas higher doses yielded a 23% relapse rate (p < 0.05). The other 52 patients received radiation in conjunction with gross total resection of tumor. The 5- and 10-year relapse rates were 18% and 23%, respectively. No factor correlated significantly with disease outcome. There was no evidence that radiation doses exceeding 50 Gy improved outcome. Positive resection margins were not significantly deleterious in this group of irradiated patients. For all 75 patients, there was no evidence that radiation margins exceeding 5 cm beyond the tumor or surgical field improved local-regional control. Ultimately, 72 of the 75 patients were rendered disease-free, but 3 required extensive surgery (amputation, hemipelvectomy) to achieve this status. Significant radiation complications were seen in 13 patients. Radiation dose correlated with the incidence of complications. Doses of 56 Gy or less produced a 5% 15-year complication rate, compared to a 30% incidence with higher doses (p < 0.05). CONCLUSIONS: Radiation is an effective modality for desmoid tumors, either alone or as an adjuvant to resection. For patients with negative resection margins, postoperative radiation is not recommended. Patients with positive margins should almost always receive 50 Gy of postoperative radiation. Unresectable tumors should be irradiated to a dose of approximately 56 Gy, with a 75% expectation of local control.     

The burst phase in ribonuclease A folding and solvent dependence of the unfolded state

A 45-year-old man was referred to our hospital for recurrent desmoid tumor of the chest wall. He underwent chest wall resection with reconstruction of Marlex mesh. But we could not resect it enough widely, because the tumor invaded beside left subclavian artery and subclavian vein, brachioflexus. So he had additional radiation therapy (50 gry). The patient is now doing well without recurrence 1 year after the operation.     

Function-sparing surgery for desmoid tumors and other low-grade fibrosarcomas involving the brachial plexus

OBJECTIVE: Low-grade fibrosarcomas and desmoid tumors present a surgical challenge in that they have a strong tendency for local invasion, surgical margins are poorly delineated, and complete resections are difficult. Nowhere is this more evident than in those lesions involving the brachial plexus. We review our experience with these difficult lesions. METHODS: From a prospective database of 2900 patients admitted for treatment of sarcoma between 1982 and 1996, we identified 15 patients with involvement of the brachial plexus by a low-grade fibrosarcoma or desmoid tumor. All patients underwent resection, with 13 of 15 receiving adjuvant radiotherapy. The 15 patients had a mean age at initial operation of 47 years. The male-to-female ratio was 8:7. The mean follow-up period was 65 months (median, 53 mo). RESULTS: Gross total resection was achieved in 12 patients (80%), although 11 of these patients had positive surgical margins. Overall, 64% of the tumors have recurred locally. There were no distant metastases, and no patients died as a result of their disease. One patient died as a result of unrelated cancer. An assessment of the functional outcomes revealed seven patients with normal function or mild neurological deficits and eight who were suffering from significant weakness, debilitation, or chronic pain. One patient required forequarter amputation. CONCLUSION: Surgical resection plus postoperative radiotherapy is the treatment of choice for low-grade fibrosarcomas and desmoid tumors involving the brachial plexus. However, aggressive surgical management with the goal of achieving a gross total resection with negative histological margins can produce unnecessary morbidity. Preserving function should be a primary goal of the operations, although this will be associated with residual disease and will risk local recurrence but rarely death resulting from the disease.     

Absence of a post-antibiotic effect (PAE) of beta-lactams against Helicobacter pylori NCTC 11637

PURPOSE: The study contained herein was undertaken to demonstrate that mesenteric desmoid tumors can cause significant symptoms other than by a mass effect and that surgery can be effective in treating these symptoms. METHODS: The medical records of patients with intra-abdominal desmoid disease in the David G. Jagelman Inherited Colorectal Cancer Registry were reviewed. Four cases are described in which intramesenteric desmoid tumors adjacent to the bowel caused mucosal ischemia in patients with familial adenomatous polyposis. RESULTS: In one patient with an ileorectal anastomosis, this was manifest by multiple small-bowel strictures and treated by multiple strictureplasties. The other three patients had ileal pouches and presented with bleeding and pain from mucosal ulceration. All pouches needed to be removed. CONCLUSION: Intra-abdominal desmoid tumors may cause problems other than by mass effect. Patients with familial adenomatous polyposis and symptoms suggestive of desmoid disease but with no detectable mass should not be denied surgery.     

Carcinosarcoma of the adrenal cortex presenting with mineralocorticoid excess

An adrenal carcinosarcoma is reported in a 79-year-old woman presenting with clinical signs of hyperaldosteronism. The tumor weighed 199 g and consisted of areas typical of adrenal carcinoma and areas of sarcoma. The sarcomatous component of the tumor showed osteogenic and chondroid differentiation. Vimentin stained both the carcinomatous and sarcomatous regions. Four months after resection, the patient developed metastases. This is the third reported case of adrenal carcinosarcoma and the only case in which hyperaldosteronism or bony differentiation was observed.     

Desmoid tumor sensitive to tamoxifen

BACKGROUND: Desmoid tumors are the second leading cause of mortality in patients with familial adenomatous polyposis who have undergone proctocolemy. Both medical and surgical treatment of these non-metastasizing but locally aggressive tumors are difficult. CASE REPORT: A 20-years-old woman consulted 6 years after coloproctectomy for familial adenomatous polyposis. A mesenteric desmoid tumor was found. Resection was not possible. The tumor progressed under sulindac treatment and subocclusion developed. The patient was given tamoxifen, 20 mg/d and the size of the tumor began to regress within a few days together with pain and signs of compression. The treatment was well tolerated for 4.5 years and tumor volume has remained unchanged after a 20-fold reduction compared with the size prior to treatment. The patient has remained symptom-free. DISCUSSION: In this critical clinical situation, tamoxifen provided rapid and prolonged effect and was well tolerated.     

Primary intracerebellar osteosarcoma arising within an epidermoid cyst

The authors report a case of a primary extraskeletal osteosarcoma arising within an epidermoid cyst in the parenchyma of the cerebellum in a 64-year-old woman. On initial presentation, the tumor involved the midline cerebellum without attachment to the surrounding dura mater or calvarium. Complete medical and radiologic evaluation failed to reveal a primary skeletal or other extraskeletal osteosarcoma. To our knowledge, this is the first reported case of a primary extraskeletal osteosarcoma within the cerebellum. Osteosarcoma as a primary brain tumor is exceedingly rare, and only three cases (all occurring within the cerebral hemispheres) have been reported previously. The histogenesis of primary sarcomas of the brain is not evident. The associated finding of an epidermoid cyst suggests the tumor originated from a teratoma.     

Benign retroperitoneal schwannoma mimicking a pancreatic cystic tumor: case report and literature review

A rare case of benign retroperitoneal schwannoma mimicking a pancreatic cystic tumor is reported herein. The tumor mass, based on a computed tomography scan and an abdominal echo examination, was initially suspected to be a mucinous cystadenoma or cystadenocarcinoma of the pancreas. However, the surgical and pathohistological findings made a final diagnosis of benign pancreatic schwannoma. The patient is doing well at a 2-year follow-up. The tumor, 18x17x15 cm in size, represented the largest among reported pancreatic schwannomas. Furthermore, a comprehensive review of reported cases of pancreatic schwannoma was carried out to summarize corresponding findings, including benign or malignant forms, adherence to other tissues, cystic change of the tumor, as well as relation to von Recklinghausen's neurofibromatosis.