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.     

Measurement of histidinohydroxylysinonorleucine and hydroxyproline in skin collagen by reversed-phase high-performance liquid chromatography after 9-fluorenylmethyl chloroformate labeling

The management of intra-abdominal desmoid tumors in patients with familial adenomatous polyposis (FAP) is very difficult. Non-steroidal anti-inflammatory drugs (NSAIDs), anti-estrogenic agents, and steroids are most commonly used, because surgical removal of these tumors may result in severe morbidity, with local recurrence being common. We report a patient with FAP and intra-abdominal desmoid tumors that regressed markedly after prednisolone therapy. The patient, a 38-year-old woman, had undergone total colectomy and ileorectal anastomosis with a diagnosis of FAP with colon cancer. Approximately 17 months after the surgery, she noticed an elastic firm lump in the abdominal wall. She also experienced lower abdominal distension. Computed tomography (CT) of the lower abdomen showed an invasive heterogenous low-density mass occupying the intra-abdominal space. She was treated with sulindac, NSAID, at 300 mg/day, the diagnosis being intra-abdominal desmoid tumors. She exhibited an intestinal obstruction about 9 months after the initiation of sulindac therapy. We changed the treatment and began prednisolone (initial dose, 40 mg/day). This treatment was continued for two years; subsequently, the lesions regressed markedly. She is currently well, more than 3 years after the withdrawal of prednisolone.     

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.     

Desmoid tumor of the vulva associated with pregnancy

Extra-abdominal desmoid tumor is a locally aggressive neoplasm that occurs most commonly in the pelvic or shoulder region in the third or fourth decade. We have identified one previously reported case of primary desmoid tumor of the vulva. Herein, we describe another case and, to our knowledge, the first reported case of vulvar desmoid tumor associated with pregnancy.     

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.     

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.     

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.     

Primary leiomyosarcoma of the greater omentum: case report and review of the literature

Omental leiomyosarcomas are rare intra-abdominal tumors. This report describes a case of primary leiomyosarcoma of the greater omentum discovered on abdominal CT scan. The mass was removed via laparotomy and an omentectomy performed. At 2 years postoperatively there is no sign of tumor recurrence or metastasis.     

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.     

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.     

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.     

Fine-needle aspiration of intra-abdominal desmoplastic small cell tumor

The cytologic features of four cases of histologically confirmed intra-abdominal desmoplastic small cell tumor (DSCT) that occurred in males between 18-27 yr of age are presented. Smears showed small cells with scant cytoplasm which were primarily arranged in loosely cohesive clusters. Nuclei were oval to round with evenly distributed, finely granular chromatin and inconspicuous nucleoli. As is typical of DSCT, the cells strongly expressed keratin and desmin in all cases. In the one case studied by electron microscopy, it was demonstrated that the cells were joined by small junctions and contained paranuclear aggregates of intermediate filaments. The absence of the characteristic desmoplastic stroma in DSCT aspirates and the nonspecific cytologic features of this small round-cell tumor (SRCT) made cytologic interpretation difficult. Cytopathologists should be aware of this entity so that DSCT is included in the differential diagnosis of SRCTs that occur in intra-abdominal sites. A panel of markers that includes keratin and desmin should be used to assist in the identification of DSCT.     

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.     

Early detection of recurrent hepatocellular carcinoma

BACKGROUND/AIMS: Early detection and treatment of recurrent hepatocellular carcinoma (HCC) are keys to patient survival after hepatic resection. In attempts at early detection, we make use of the alpha-fetoprotein (AFP) test every month and abdominal ultrasound (US) and computed tomography (CT) are carried out every three months after hepatectomy. The objective of the present study was to evaluate the most appropriate interval for follow-up re-examinations after resection for HCC. PATIENTS AND METHODS: Eighty-five patients with recurrent HCC were divided into two groups according to the state of the tumor when recurrence was detected: Group I (n = 70); tumor size < or = 2.0 cm, and group II (n = 15); tumor size > or = 2.1 cm. Clinicopathological comparisons were made between the two groups. RESULTS: AFP positivity in group I was significantly lower than group II at the time of recurrence. Rates of extrahepatic intra-abdominal recurrences, i.e. recurrence at the surgical stump and in the abdominal cavity and lymph nodes around the liver, were more frequent in group II than in group I (47% vs 4%; p < 0.001). The average tumor size was larger in 10 patients with extrahepatic intra-abdominal recurrence than in 75 patients with intrahepatic recurrence (3.4 +/- 2.0 vs 1.6 +/- 0.6 cm; p < 0.0001). There was a statistically significant difference regarding the histological grade of initial HCC between the two patterns of recurrence. CONCLUSIONS: Measurements of AFP were seen to have limited value for detecting recurrence, at an early stage. Close postoperative follow-up, including bedside US in the outpatient clinic, should be carried out when the initial HCC is histologically less differentiated HCC.     

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.     

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.     

A bladder nephrogenic adenoma. A case report

OBJECTIVE: To report an additional case of nephrogenic adenoma of the bladder. METHODS/RESULTS: A case of nephrogenic adenoma of the urinary bladder in a 28-year-old female is described. Patient clinical history and diagnostic imaging findings are presented. CONCLUSIONS: Nephrogenic adenoma of the bladder is a rare benign tumor with specific histological features that has been associated with previous surgery, trauma, infections and lithiasis. Although it is currently not considered to be a premalignant lesion, its rate of recurrence is high (37%-49%). The treatment of choice is by transurethral resection and yearly cytological, ultrasound and cystoscopic follow-up evaluation to detect recurrence.     

Eccrine porocarcinoma

Eccrine porocarcinoma is a malignant tumor of eccrine sweat glands. It is a very rare, slow growing tumor and clinically resembles other skin cancers. We report a case and review its clinical and pathological features. These tumors have a propensity for local recurrence, and wide excision with negative margins is recommended.     

An autopsy case of extraskeletal Ewing's sarcoma followed up for 24 years

A long-term (24 years) follow-up case of extraskeletal Ewing's sarcoma is reported. The light microscopic examination showed features hardly indistinguishable from Ewing's sarcoma of the bone, that is, the tumor cells were diffusely arranged and uniform in size and shape, and possessed glycogen in the cytoplasm. Homer-Wright rosettes were found only in the autopsy material. An immunohistochemical study using a neural marker (neuron-specific enolase) demonstrated positive staining in most tumor cells. An ultrastructural study revealed intracytoplasmic glycogen particles and incomplete neural characters as follows: the cytoplasmic processes resembled neural processes without neurosecretory granules, microtubules or neurofilaments. These findings suggest that this case finally acquired an incomplete neural character with repeated recurrence. This tumor was diagnosed extraskeletal Ewing's sarcoma, but in future it may be categorized as primitive neuroectodermal tumor (PNET).