Superficial desmoplastic fibroblastoma (collagenous fibroma): Clinicopathologic study of 11 cases.

BACKGROUND: Desmoplastic fibroblastoma (collagenous fibroma) is a rare soft tissue tumor that usually arises in the subcutis or skeletal muscle. Cases superficial to fascia are unusual and can cause diagnostic difficulty. We present 11 cases of superficial desmoplastic fibroblastoma involving a wide anatomic distribution. METHODS: Archives were searched using the term "desmoplastic fibroblastoma" over a 10-year period (2012-2022). Cases superficial to fascia were retrieved, and available clinicopathologic features were recorded. Only cases involving the dermis were included. RESULTS: Eleven cases were identified, all of which were received in consultation. Tumors involved the head and neck (2), lower extremity (2), back (2), foot (1), shoulder (1), axilla (1), hand (1), and breast (1). Each consisted of a hypocellular proliferation of bland stellate to spindled fibroblasts set in a collagenous to focally myxoid stroma. The immunohistochemical stains available for review demonstrated SMA positivity (4/7) and negative immunoreactivity for CD34 (0/6), EMA (0/3), desmin (0/3), and S100 (0/7). CONCLUSIONS: Desmoplastic fibroblastoma may present superficially in the dermis to subcutis, posing a potential source of diagnostic difficulty. Recognition of the characteristic histopathologic features of desmoplastic fibroblastoma with judicial use of immunohistochemical stains should allow for accurate diagnosis.

Desmoplastic fibroblastoma mimicking tenosynovial giant cell tumor encasing a tendon of the foot.

Desmoplastic fibroblastoma is an uncommon, benign fibrous soft tissue tumor that usually occurs in the arms, shoulders, neck, hands, and feet in the fifth to seventh decades of life. In general, it is commonly located in the subcutaneous tissue and skeletal muscle. The authors report an unusual case of a desmoplastic fibroblastoma mimicking tenosynovial giant cell tumor encasing a tendon of the foot in a 72-year-old woman. Ultrasonography revealed an inhomogeneously hypoechoic lobulated soft tissue lesion completely wrapped around the extensor digitorum longus tendon. Color Doppler study revealed increased vascularity in the internal and peripheral portions of the lesion. Magnetic resonance imaging revealed a well-defined, lobulated soft tissue mass encasing the extensor digitorum longus tendon with predominantly isointense signal with some areas of hypointense signal on T1-weighted images, predominantly hyperintense signal with some areas of hypointense signal on T2-weighted images, and inhomogeneous enhancement on fat-suppressed contrast-enhanced T1-weighted images. Surgical excision was performed, and the mass was diagnosed on pathological examination as a desmoplastic fibroblastoma. There has been no previously published radiologic case of a desmoplastic fibroblastoma encasing a tendon of the foot in the literature.

Rib desmoplastic fibroblastoma: Video-assisted resection.

A 46-year-old man was referred to our department due to chronic chest pain. A computed tomography showed an exophytic image arising from 5th rib that was projected on the middle lobule. The patient underwent an exploratory videothoracoscopic because we couldn't discard lung compromise. A corneal-like lesion emerging from the inner side of the 5th rib was revealed. Complete video-assited resection was done. Histopathology examination revealed a desmoplastic fibroblastoma. To our knowledge this is the first case of desmoplastic fibroblastoma arising from a rib and the second case published in all literature of chest wall involvement.

Dermal desmoplastic fibroblastoma presenting as a large sacral mass.

Desmoplastic fibroblastoma (collagenous fibroma) is a rare and recently recognised benign tumour. Most desmoplastic fibroblastomas arise in the subcutaneous tissue or skeletal muscle. Involvement of the dermis is extremely rare. We describe an unusual case of dermal desmoplastic fibroblastoma presenting as a large sacral mass in a 16-year-old male. An awareness of this entity is necessary to avoid confusion with other benign and malignant soft tissue neoplasms.

Collagenous fibroma of the right coronary cusp: a case report and literature review.

Collagenous fibroma (desmoplastic fibroblastoma) is a rare benign tumor. This type of tumor mostly occurs in the subcutaneous tissues or skeletal muscle, and to the best of our knowledge, there is no unique case report of it occurring in the cardiac valves. This is the first description of collagenous fibroma in a human heart.

Bilateral collagenous fibroma of the hard palate: a case report and review of the literature.

BACKGROUND: Collagenous fibroma or desmoplastic fibroblastoma is a rare benign fibrous tissue tumor. It usually presents as a painless, slowly growing mass. Collagenous fibroma arises ordinarily inside the subcutaneous tissues or skeletal muscles. Histopathologically, the tumor consists of scattered stellate and spindle cells in a hypovascular collagenous stroma without atypia or infiltration. The oral cavity is a very uncommon site for desmoplastic fibroblastoma. Only 15 published articles in the literature reported the intraoral location. We present a case of collagenous fibroma with a bilateral distribution on the hard palate. This is the second case of bilateral collagenous fibroma after a previously reported one in literature; however, our case was larger, occupying almost the whole palate. We discuss the management of this rare tumor and how we can reach definite diagnosis. CASE PRESENTATION: A 37-year-old Caucasian female patient had a huge bilateral firm palatal mass that caused breathing problems. There was no history of trauma and the patient had no relevant medical history Total surgical excision under general anesthesia was carried out and histopathological examination suggested a benign mesenchymal tumor. Immunohistochemistry was necessary to confirm the tumor origin and to exclude aggressive fibromatosis. A diagnosis of bilateral collagenous fibroma was reached. Six months after surgery, there was no recurring lesion and the patient's health was good. CONCLUSIONS: Collagenous fibroma is a benign fibrous tissue tumor of unknown cause that is treated with simple excision. The prognosis is good with no recurrence. Reaching an accurate diagnosis is mandatory to avoid aggressive treatment since collagenous fibroma may be misdiagnosed as aggressive fibromatosis in case of massive size. Clinicians and pathologists should be aware of this unusual tumor for conservative management without side effects.

Desmoplastic Fibroblastoma of the Supraclavicular Region: A Rare Soft-Tissue Tumor With a Review from the Last Decade.

Desmoplastic fibroblastoma (DF) is primarily a sporadic, rare, benign, soft-tissue tumor with an excellent prognosis and no reported recurrence to date. DF presents as a painless, slow-growing mass with symptoms due to its mass effect. We report a case of a middle-aged man presenting with a right supraclavicular mass and numbness of the right arm, eventually diagnosed as DF. After confirming the diagnosis with imaging and biopsy studies, the tumor was successfully excised using a transcervical approach with no complication or recurrence detected during the 2-year follow-up after surgery. A review of the relevant literature is presented regarding the presentation, diagnosis, and outcomes of published case reports of DF coinciding with those of our discussed case. This case report adds to the pool of uncommon DF cases mainly for its unique symptoms and location. It also highlights the necessity of health education related to painless masses that might be attributed as trivial in the community.

An Update on Clinicopathological, Imaging and Genetic Features of Desmoplastic Fibroblastoma (Collagenous Fibroma).

Desmoplastic fibroblastoma (also known as collagenous fibroma) is an uncommon benign fibroblastic/myofibroblastic neoplasm that primarily arises in the subcutaneous tissue of upper extremity. Magnetic resonance imaging reveals a well-defined mass in intimate association with dense connective tissue and prominent low signal intensity on all pulse sequences. Peripheral and septal enhancement is usually seen after intravenous contrast. Histologically, the lesion is paucicellular and consists of spindle to stellate-shaped cells embedded in a collagenous or myxocollagenous stroma with low vascularity. Diffuse and strong nuclear immunoreactivity for FOS-like antigen 1 seems to be characteristic of desmoplastic fibroblastoma. Cytogenetic studies have demonstrated the presence of 11q12 rearrangements and an identical t(2;11)(q31;q12) translocation. This review provides an updated overview of the clinical, radiological, histological, cytogenetic and molecular genetic features of desmoplastic fibroblastoma and discusses the relationship to fibroma of tendon sheath.

An extremely rare case of desmoplastic fibroblastoma exhibiting rapid growth in the chest wall: a case report.

BACKGROUND: Desmoplastic fibroblastoma is an uncommon, benign, fibrous tumor exhibiting infiltrative growth. Most of these tumors are small, slow-growing, and develop as subcutaneous lesions in the extremities. Cases of desmoplastic fibroblastoma in the chest wall are quite rare, and the preoperative diagnosis of such cases remains challenging as these tumors can mimic the characteristics of desmoid-type fibromatosis, which often occurs in the chest wall. We aimed to describe a rare case of desmoplastic fibroblastoma exhibiting rapid growth in the chest wall of a patient that was successfully treated with marginal excision only by diagnostic imaging before surgery. CASE PRESENTATION: A 79-year-old man was admitted to our hospital after experiencing right shoulder pain lasting for a few months. A 4 × 4 × 2 cm mass was incidentally detected at the right second rib two years prior. Chest computed tomography revealed a well-defined homogeneous mass with a muscle-like density along the right lateral chest wall, the size of which had increased to 12 × 10 × 4.5 cm in two years. Dynamic contrast-enhanced computed tomography revealed abundant vascularity at the periphery of the tumor. Magnetic resonance imaging revealed iso-intensity to muscle on T1-weighted images, slightly high intensity on T2-weighted images, and rim-like contrast enhancement at the periphery of the tumor, with uniform thickness on gadolinium-enhanced T1-weighted images with fat suppression. Rim-like contrast enhancement is an imaging feature that can distinguish cases of desmoplastic fibroblastoma from desmoid-type fibromatosis. We diagnosed the tumor as desmoplastic fibroblastoma by diagnostic imaging without tissue biopsy. Marginal excision with videoscopic assistance was performed through a small incision. The pathological diagnosis was desmoplastic fibroblastoma. The patient's postoperative course was uneventful, and his shoulder pain was relieved after the surgery. CONCLUSIONS: Desmoplastic fibroblastoma in the chest wall is extremely rare, but should be considered in the differential diagnosis when desmoid-type fibromatosis is clinically suspected. Gadolinium-enhanced magnetic resonance imaging is helpful in confirming the differential diagnosis.

A desmoplastic fibroblastoma that developed in the anterior mediastinum: a case report.

BACKGROUND: Desmoplastic fibroblastoma (also known as collagenous fibroma) is a benign, slowly growing soft-tissue tumor. Most desmoplastic fibroblastomas develop in the limbs, neck, or trunk. A mediastinal origin is quite rare. CASE PRESENTATION: A 32-year-old Asian female was referred to us for the diagnosis and treatment of an anterior mediastinal tumor. The tumor was 80 mm in the largest diameter and was located on the pericardium. No invasion was evident. She underwent resection of the tumor via video-assisted thoracoscopic resection. The tumor was totally encapsulated, and its pedicle was on the pericardium. The resected specimen was very rigid, making it difficult to remove from the intercostal space. Histologically, the tumor was composed of a paucicellular dense collagenous tissue. Mitosis was rarely observed, and cellular atypia was not evident, suggesting that the tumor was benign. We diagnosed the tumor as a desmoplastic fibroblastoma by morphology and immunohistochemistry. CONCLUSIONS: Desmoplastic fibroblastoma of the mediastinum is an extremely rare disease. Preoperative diagnosis is difficult. Early surgical resection is suitable for diagnosis and treatment planning.

Multimodal radiological imaging of collagenous fibroma arising from the subacromial region in a patient with osteosarcoma: A case report.

Collagenous fibroma arising from the subacromial region is extremely rare. It is important to distinguish collagenous fibroma from other fibrous tumors including desmoid tumors, to differentiate between the prognoses and management strategies, including surgical treatment. The present case report describes the case of a 42-year-old man with a collagenous fibroma of the subacromial region. He received a follow-up examination following treatment for osteosarcoma. Positron emission tomography (PET) scans used to assess for metastatic lesions indicated uptake in his left shoulder. The maximum standardized uptake value was 2.4. Magnetic resonance imaging demonstrated iso-intensity to muscle on T1-weighted images and iso-intensity with slightly high intensity on T2-weighted images. Post-contrast fat-suppressed magnetic resonance images indicated slightly heterogeneous enhancement of the lesion. There were no notable results from X-rays, bone scintigraphy and thallium-201 scintigraphy. Histological examination revealed collagenous fibroma. To the best of our knowledge, the present case is only the second incidence of collagenous fibroma arising from the subacromial region, and the first description of thallium-201 scintigraphy and PET scans in collagenous fibroma. The multimodal radiological data of this case may be useful for assisting in the differentiation of fibrous tumor types, including collagenous fibroma.

Desmoplastic Fibroblastoma of the Scalp Accompanied by Severe Pain; Unusual Location and Symptom.

Desmoplastic fibroblastoma is a rare fibrous tumor that usually presents as a painless, slow-growing mass in the subcutaneous tissues and skeletal muscles. It has a wide anatomic distribution, with the most common involvement being the arm and shoulder. Here, we report a case of a tiny painful desmoplastic fibroblastoma arising on the scalp. According to a microscopic examination, this tumor was composed of spindle-shaped fibroblasts in the dense collagenous stroma. On immunohistochemical staining, tumor cells were positive for vimentin and negative for smooth muscle actin, CD34, and S100. Our case is unique in that desmoplastic fibroblastoma developed on the scalp and there was presence of pain despite its small size.

US and MRI appearance of a collagenous fibroma (desmoplastic fibroblastoma) of the shoulder.

Collagenous fibroma (desmoplastic fibroblastoma) is a recently described rare benign tumor. Since this tumor was characterized by Evans in 1995, fewer than 100 cases have been reported in English literature with the largest series published by Miettinen and Fetsch. Preoperative radiological diagnosis is important to avoid over-treatment and unnecessary extensive procedures, but is difficult because diagnostic imaging findings for collagenous fibroma have not been established. Here we report the US and MRI appearance of a lesion localized deep under the deltoid muscle in a 34-year-old woman with a palpable painless mass. Histological findings were consistent with a collagenous fibroma. As far as we know, this is the first description of US appearance of a collagenous fibroma in English literature.

Fibroma of tendon sheath with 11q rearrangements.

Fibroma of tendon sheath is an uncommon, benign fibroblastic tumor that usually occurs in the upper extremities of young and middle-aged adults. A clonal chromosomal aberration, t(2;11)(q31-32;q12), has been described in one case. We herein present a unique cytogenetic finding of fibroma of tendon sheath arising in the first web space of the right hand of a 38-year-old woman. Physical examination showed a 3.5-cm, firm, mobile, non-tender mass. Magnetic resonance imaging showed a well-defined soft tissue mass with iso- to slightly-low signal intensity relative to skeletal muscle on both T1- and T2-weighted sequences. Contrast-enhanced T1-weighted sequences demonstrated moderate patchy enhancement of the mass. A fibroma or giant cell tumor of tendon sheath was suggested, and the lesion was marginally excised. Histological examination confirmed the diagnosis of fibroma of tendon sheath. Cytogenetic analysis revealed a novel t(9;11)(p24;q13-14) translocation among other karyotypic abnormalities. The postoperative course was uneventful, and the patient is doing well without local recurrence two months after surgery. To the best of our knowledge, this is only the second report of fibroma of tendon sheath with clonal chromosomal abnormalities.

Desmoplastic (collagenous) fibroma of the femur: A case report and review of the literature.

Desmoplastic fibroma is a rare, benign soft-tissue tumor composed of spindled and stellate-shaped cells that are embedded in a dense collagenous stroma. Clinically, desmoplastic fibroma presents as a firm, mobile, slow-growing mass that is located in the subcutaneous tissue or near the deep aspect of the skeletal muscles. The present study describes the case of a 66-year-old female who presented with an inactive, firm, slightly tender mass in the lower medial segment of the right femur. An open biopsy was performed and the result of the pathological examination indicated a desmoplastic fibroma. The patient underwent a radical resection of the tumor and the accompanying bone, which was then reimplanted using devitalized tumor bone, self-ilium graft and homologous allograft bone transplantation, with an internal fixation by locking the compression plate. This was followed by a reconstruction of the anterior and posterior cruciate ligaments and the lateral and medial collateral ligaments. There was no evidence of local recurrence at five years post-surgery.

Collagenous fibroma (desmoplastic fibroblastoma) with trisomy 8 as the sole cytogenetic abnormality.

Collagenous fibroma (desmoplastic fibroblastoma) is a benign fibrous soft tissue tumor that usually occurs in the subcutaneous tissue or skeletal muscle of adults. Recent cytogenetic studies have revealed clonal rearrangements of the chromosomal band 11q12. We present a unique case of collagenous fibroma arising in the right shoulder of a 63-year-old female. Magnetic resonance imaging showed a solid soft tissue mass deeply relative to the deltoid muscle, with low-to-intermediate signal intensity on T1-weighted sequences and low-to-slightly high signal intensity on T2-weighted sequences. Contrast-enhanced fat-suppressed T1-weighted sequences demonstrated heterogenous internal enhancement with rim enhancement. Following an open biopsy, marginal excision of the tumor was performed. Histological examination confirmed the diagnosis of collagenous fibroma. Cytogenetic analysis displayed a simple karyotypic change with trisomy 8. The postoperative course was uneventful, and the patient is doing well without local recurrence two months after the surgery. To the best of our knowledge, this is the first case of collagenous fibroma with trisomy 8 as the sole cytogenetic abnormality.

Collagenous fibroma (desmoplastic fibroblastoma) of the oral cavity.

Collagenous fibroma (desmoplastic fibroblastoma) is a distinctive yet uncommon fibrous soft tissue tumor. These tumors are rather nondescript in their morphological appearance and have been diagnosed as fibromas or some other benign mesenchymal lesions for years. The most common sites are the upper extremities, followed by the lower extremities. Rare lesions arise in the head and neck region. We report a rare case in the oral cavity and present its unique histopathological features (central fat entrapment) besides others, and diffusely strong vimentin immunopositivity.

Collagenous fibroma (desmoplastic fibroblastoma) of the neck presenting with neurological symptoms.

Collagenous fibromas are rare fibrous soft tissue tumours that usually arise in subcutaneous tissue or skeletal muscle at a variety of anatomical sites. These lesions commonly present as painless, slow-growing mobile masses. We describe a unique case of a 41-year-old woman presenting with a posterior neck swelling and longstanding history of severe ongoing pain in the right scapular region, shoulder and neck, weakness of the palmar grip and limited right lateral neck flexion and rotation. A history of trauma to the right neck in adolescence was noted. Histological analysis revealed a paucicellular lesion with spindle and stellate-shaped fibroblasts involving the cervical nerve roots, typical of collagenous fibroma. In a literature search on Medline and Pubmed, we found no reported cases of collagenous fibromas presenting with neurological symptoms. This report highlights the potential of these lesions to present with neurological symptoms due to infiltration of surrounding tissues, and that preceding trauma may contribute to the aetiology.

Collagenous fibroma of the deltoid muscle: clinical, surgical and histopathological aspects.

We report the case of a 66-year-old woman with a tumorous swelling of the right shoulder of approximately 6 months' duration with morphological features consistent with desmoplastic fibroblastoma or collagenous fibroma, a benign fibrous soft tissue tumor with distinct clinico-pathological features. Clinical history, radiological and pathological findings are presented and other cases of collagenous fibroma with unusual location are discussed.