Type Fibromatosis Research Articles

Cutaneous Endometriosis

Endometriosis affecting the skin is a very much rare surgical entity encountered almost exclusively in women of childbearing age. It commonly presents as a discoloring painful cutaneous swelling, the pain being most commonly a cyclical one occurring during menstrual bleeding. It often needs histopathological examination to arrive at a correct diagnosis. Otherwise, the diagnosis is usually presumptive from typical and atypical presentations and other supportive imaging investigations. Wide surgical excision is the gold standard curative treatment of choice. Nonsurgical treatment includes use of such hormonal agents as danazol or leuprolide, oral contraceptive pills, etc. which can’t definitely cure the patient from this nonfatal ailing disease. All other examples of benign and malignant cutaneous swellings like desmoid tumors, fibro-histiocytoma, dermatofibroma protuberans, papilloma, melanoma, visceral malignancies and many other types of fibromatosis are to be included in the differential diagnosis. These patients with cutaneous endometriosis need to be additionally evaluated for endometriosis elsewhere in the body especially in the pelvis and the abdomen proper, though no other tissue like brain, lungs, liver etc. are not immune to it. In women of childbearing age with abdominal or pelvic wall masses or an area of soft-tissue thickening at cross-sectional imaging in or very close to previous surgical scars, endometriosis is to be strongly suspected by the consulting radiologist. Imaging investigations like CT, USG, and especially, MRI may help in the diagnosis of scar endometriosis, but it is only the biopsy that is definitely confirmatory. KYAMC Journal. 2022;12(04): 243-249

Palmar and plantar fibromatosis: a review.

Palmar fibromatosis (Dupuytren disease/contracture) is the most common type of fibromatosis, defined as a benign proliferation of fibroblasts and myofibroblasts. The disease process is most common in white, middle-aged and older men occurring at the distal palmar crease leading to nodules and contracture, which in many cases recur after surgical treatment. In a similar process, plantar fibromatosis (Ledderhose disease) is a proliferation of fibroblasts and myofibroblasts on the plantar aponeurosis of mostly middle-aged patients that may lead to painful nodules but usually does not lead to contracture. Both processes are histologically similar, composed of a bland cellular proliferation of spindle cells with a bluish appearance and with a variable amount of background collagen, depending on the age of the lesion. The etiology of both lesions is still uncertain, while treatment ranges from observation to surgery, with some pharmacologic agents being investigated with mixed success. In this paper we provide an overview of both processes with regards to clinical and radiologic findings, pathophysiology, diagnosis, treatment, and prognosis.

Advanced palmar fascia

Advances in medical treatments sometimes leads the way to advancements in diagnosis. Hand Surgeons are in a brave new world of mini endoscopes with a range of tools and implants. Thus, what was a quick 5 minute scan in the past may now take a little bit longer as the Surgeon wants more detailed information in their imaging. Dupuytren's disease is a type of fibromatosis and is the main pathological finding of the palmar fascia. This presentation will look at the palmar fascia of the hand and the detailed anatomy that can be seen on ultrasound. The palmar fascia is a lot more complicated than what is seen in the palm. Palmar fascia extends into the fingers up to the finger tips. This presentation will describe the normal and abnormal ultrasound appearances and the treatment of Dupuytren's disease.

Mesenteric fibromatosis misdiagnosed with lymph node metastasis after successful laparoscopic right hemicolectomy: a report of two cases with review of literature.

Fibromatosis is a rare type of tumor derived from the mesenchymal tissue. This is a benign tumor with infiltrating growth but may invade locally and recur following excision. As one type of fibromatosis, mesenteric fibromatosis (MF) accounts for a mere 8% of cases. Although studies have revealed that the etiology of MF is related to trauma, surgery, hormones, and heredity, the specific etiology of fibromatosis remains unclear. With such low incidence, MF has been rarely reported and tends to be misdiagnosed due to insufficient recognition. In this paper, we describe the cases of two patients with MF who were misdiagnosed with lymph node metastasis and who had previously undergone successful laparoscopic right hemicolectomy. We provide this information in order to broaden the clinical understanding of MF.

Abdominal desmoid-type fibromatosis – a case report and an overview of treatment options

Fibromatosis (desmoid, desmoid tumour) is a tumour belonging to the group of fibrous tissue proliferative diseases. Although the tumour cannot produce distant metastases, it shows local malignancy by invading adjacent tissues, as well as high recurrence rates after local resection. The incidence of fibromatosis is 2-4 cases per 1 million inhabitants per year. Three types of fibromatosis have been distinguished: extra-abdominal, abdominal wall and intra-abdominal type. Although the most common types are sporadic, coexistence with familial adenomatous polyposis (FAP), known as the Gardner’s syndrome, is observed. Available therapeutic options include surgical treatment, watchful waiting, hormone therapy, non-steroid anti-inflammatory drugs, chemotherapy and radiation therapy. This case report aims to draw attention to the difficulties in selecting an appropriate treatment option, limited surgical treatment, the psychological aspect of the disease, as well as the need to actively search for modern diagnostic and therapeutic solutions. We present a case of a 26-year-old patient who was ultimately diagnosed with a desmoid tumour invading the abdominal wall and internal abdominal organs. The patient presented to a county hospital due to suspected gastrointestinal obstruction. It was found from medical history that the patient had palpated a tumour of the abdominal wall 9 months before admission.

Aggressive juvenile mandibular fibromatosis

Aggressive juvenile fibromatosis of the jawbones is a rare tumor presenting as infiltrative mass with unpredictable evolution. We report herein a 17-year-old student with a 6-month history of radiologically proven resorption of a part of the mandible, lingual displacement of tooth 34 and malocclusion. Alveolar ridge resorption and three dark-brown foci in the bone were seen after the tooth was extracted. Histological study showed the tumor tissue to have a bundle-like structure; immunohistochemically it was positive for vimentin, smooth muscle actin, beta-catenin, Ki-67 (5%), and negative for desmin and cytokeratin 34bE12. The golden standard in the diagnostics of desmoid fibromatoses is the nuclear or membrane expression of beta-catenin, which is found in 90% of the cases. Differential diagnosis include mandibular fibroma, well-differentiated fibrosarcoma, fibrosing histiocytoma, and infiltration from adjacent soft-tissue tumor. Aggressive juvenile fibromatosis should be managed by radical excision. Local recurrences are not rare, but metastases do not develop. In rare cases this type of fibromatosis has been known to regress spontaneously. Aggressive fibromatosis is a diagnostic challenge, since it remains in the grey zone between benign and malignant lesions of the oral cavity.

Desmoid tumor of the facet joint: a case report.

Desmoid tumors represent a particular type of fibromatosis. The common sites for extra-abdominal desmoid tumors, known as aggressive fibromatosis, are the shoulder, chest wall, and thigh. Desmiod tumors are rare at facet joints of the spine. We describe a patient with a desmoid tumor of a lumbar facet joint.

Recent Surgical and Medical Advances in the Treatment of Dupuytren’s Disease - A Systematic Review of the Literature

Dupuytren’s disease (DD) is a type of fibromatosis which progressively results in the shortening and thickening of the fibrous tissue of the palmar fascia. This condition which predominantly affects white-northern Europeans has been identified since 1614. DD can affect certain activities of daily living such as face washing, combing hair and putting hand in a glove. The origin of Dupuytren’s contracture is still unknown, but there are a number of treatments that doctors have come across throughout the years. Historically surgery has been the mainstay treatment for DD but not the only one. The objective is to make a structured review of the most recent advances in treatment of DD including the surgical and medical interventions. We have looked at the most relevant published articles regarding the various treatment options for DD. This review has taken 55 articles into consideration which have met the inclusion criteria. The most recent treatments used are multi-needle aponeurotomy, extensive percutaneous aponeurotomy and lipografting, injecting collagenase Clostridium histolyticum, INF-gamma and shockwave therapy as well as radiotherapy. Each of these treatments has certain advantages and drawbacks and cannot be used for every patient. In order to prevent this condition, spending more time and money in the topic is required to reach better and more consistent treatments and ultimately to eradicate this disease.

Aggressive fibromatosis of the oral cavity

Fibromatosis are proliferations of highly differentiated fibrous tissue. The deep or musculoaponeurotic types of fibromatosis are known as desmoid tumors, aggressive fibromatosis, or desmoid-type fibrosarcoma. It is commonly seen as abdominal tumors; the occurrence of these tumors in maxillofacial region is a rare entity. We report a case of aggressive fibromatosis in a 50-year-old female, who presented with a swelling of the left side of the face. The tumor was extending in to the maxillary antrum which was confirmed as aggressive fibromatosis on histopathological examination. The detailed discussion of etiology, incidence, management and prognosis of the same in corelation with the literature is presented in the article.

Fine needle aspiration cytology in fibromatosis

Fibromatosis form a spectrum of clinicopathologic entities characterized by the infiltrative proliferation of fibroblasts that lack malignant cytologic features. The fibromatosis can be localized or infiltrative and multicentric and can involve internal tissues and organs as the mesentery, retroperitoneum, breast, and almost every organ and region of the body, including the bones, the meninges and the central nervous system. We report a case of 37-year-old male who presented with a right supraclavicular mass with superficial infiltrative type of fibromatosis and fine needle aspiration cytology (FNAC) was performed. We report this case because of limited literature of FNAC in fibromatosis and quick role of FNAC in the diagnosis of fibromatosis.

Cholangiocarcinoma accompanied by desmoid-type fibromatosis

Cholangiocarcinoma complicated by intra-abdominal desmoid-type fibromatosis (DTF) is uncommon. There are no reports on patients with this type of fibromatosis, in which the pre-operative treatment (including diagnosis), surgical approach, post-operative pathologic reports, and prognosis are discussed. The clinicopathological features of a 49-year-old man were retrospectively analyzed. Cholangiocarcinoma located in the inferior segment of the bile duct was considered pre-operatively on the basis of clinical findings. At the time of pancreaticoduodenectomy, the mesojejunum was stiff without nodules or a mass at a distance of approximately 80 cm from the ligament of Treitz. Complete excision of the entire lesion of the intestinal mesenteric contracture and its subsidiary was performed. Post-operative pathologic findings confirmed an adenocarcinoma located at the extremity of the common bile duct and infiltrating the full thickness of the common bile duct as well as the deep muscular layer of the duodenum. The contracted jejunal mesentery was shown to have DTF. The patient was alive with no evidence of recurrence after a follow-up of 6 months. The patient had a rare hereditary disease with intra-abdominal DTF, which manifests the characteristics of an aggressive growth pattern and a high rate of local recurrence; conservative therapy is recommended. Complete excision of the fibromatous lesion during pancreaticoduodenectomy may maximally decrease the risk of local recurrence.

Extra-abdominal desmoid around the shoulder joint: one of the differential diagnoses of frozen shoulder

Extra-abdominal desmoid tumor is a type of fibromatosis derived from fascial or musculoaponeurotic structures.3,8 When the tumor occurs around joints, movement is restricted, resulting in pain with motion. We report 3 cases of extra-abdominal desmoid tumor around the shoulder misdiagnosed as frozen shoulder.

A CASE REPORT OF DESMOID TUMOR OF THE CHEST WALL

We report herein a rare case of desmoid tumor of the chest wall in a 39-year-old woman. She was admitted to the hospital beacause of an abnormal shadow on a chest X-ray film. Chest CT and MRI revealed the soft tissue of the chest wall at the left apical portion. She underwent a surgical resection of the tumor through thoracotomy with video-assisted thoracoscopy. The tumor arose from 1st intercostal muscle, and was resected combined with the 1st rib. The tumor was 4×3×2cm in size. Histological examination revealed findings of desmoid type of fibromatosis. A local recurrence of the tumor without any symptoms was found 17 months after the surgical resection. Radiotherapy was administered at a dose of 50 Gy to the recurrent desmoid tumor that resulted in no more growth of the tumor in size so far. The patient is followed on an ambulant basis.

Cutaneous adult myofibroma: a vascular neoplasm.

Infantile myofibromatosis is a distinctive type of fibromatosis that usually develops during the immediate perinatal period. There are variants with solitary and multiple tumors. Lesions confined to the skin, soft tissue, and bone carry a good prognosis, showing spontaneous regression. The prognosis, however, is much less favorable when visceral lesions are present and the outcome may be fatal. Only recently it became obvious that there is an adult counterpart of infantile myofibromatosis, characterized by solitary lesions that have a predilection for involve the dermis and show no tendency to regression, although they have an entirely benign biological behavior. These lesions have been named cutaneous myofibroma or solitary myofibroma of adults. We have studied the clinical, histopathological and immunohistochemical characteristics of 53 examples of cutaneous adult myofibroma. In addition, 2 cases were examined ultrastructurally. The patients were mostly adults with ages ranging from 6-83 years. The lesions presented as solitary, usually painless nodules of variable duration on the skin, usually located on the extremities. Histopathologically, four patterns were identified: nodular or cellular type, multinodular or biphasic type, leiomyoma-like or fascicular type, and vascular type. A correlation between the histopathologic pattern and the lesional age was observed: vascular type of cutaneous adult myofibroma in early lesions, nodular and multinodular lesions in fully developed lesions, and leiomyoma-like or fascicular type in late lesions. Immunohistochemically, the spindle cells were desmin negative, but expressed immunoreactivity for vimentin, pan-smooth muscle actin, and alpha-smooth muscle actin. Ultrastructurally, neoplastic cells showed characteristics of undifferentiated mesenchymal cells with features of fibroblasts, myofibroblasts and pericytes. Primitive vascular formations were seen in the form of irregular clefts between adjoining cells. We conclude that cutaneous adult myofibroma is a little-known benign vascular neoplasm probably derived from myopericytes.

CT findings of Desmoid tumor arising at Abdominai Wall

Desmoid tumor is a type of fibromatosis usually arise in deep musculo-aponeurotic structures, primarily of the trunk and extremities. It is characterized by proliferation of fibroblastic tissue and does not metastasize but may be locally aggressive. Eventhough the surgical margin reveals clean, recurrence often occurs. To analyze the extent of the tumor and homodynamic characteristics exactly, we performed IV bolus CT. Desmoid tumors show peripheral rim enhancement on early phase scan and more strong, central enhancement on late phase IV bolus CT, which reflects abundant fibroblastic components of the tumor. We report two cases of pathologically confirmed desmoid tumor performed IV bolus CT.

Infantile myofibromatosis: Diagnosis suggested by fine‐needle aspiration biopsy

A newborn male infant presented with multiple subcutaneous and parenchymal lesions. These lesions, thought clinically to represent disseminated neuroblastoma, were assessed via fine-needle aspiration biopsy. The spindled cells and associated collagen were interpreted as a type of fibromatosis, most probably infantile myofibromatosis. This diagnosis was subsequently confirmed histologically, and the patient experienced spontaneous involution or regression of all lesions by 18 mo of age. To our knowledge, this is the first documented case of this entity initially suggested on the basis of a fine-needle aspiration biopsy specimen.