Journal of Pathology and Translational Medicine

Review

Palmar and plantar fibromatosis: a review: Brian D. Stewart, Alessandra F. Nascimento; J Pathol Transl Med. 2021;55(4):265-270. Published online July 7, 2021; DOI: https://doi.org/10.4132/jptm.2021.06.14

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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.

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Case Study

Abdominal Fibromatosis in a Young Child: A Case Study and Review of the Literature: Hyun Hee Chu, Pyoung Han Hwang, Yeon Jun Jeong, Myoung Ja Chung; Korean J Pathol. 2013;47(5):472-476. Published online October 25, 2013; DOI: https://doi.org/10.4132/KoreanJPathol.2013.47.5.472

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Abstract PDF

Fibromatoses comprise many different entities of well-differentiated fibroblastic proliferation with variable collagen production and form a firm nodular mass. Abdominal fibromatosis is distinguishable from other forms of fibromatosis because of its location and its tendency to occur in women of childbearing age during or following pregnancy. Abdominal fibromatosis in children is an extremely rare condition. A 15-month-old boy presented with an abdominal wall mass that had recently increased in size. Mass excision was perfomed. The tumor was 4.3×4.1 cm and partly circumscribed. Histologically, the tumor was composed of parallel long fascicles of spindle-cells with a uniform appearance. The edges of the resected mass were infiltrative, and the surgical margins were positive. Mitotic figures were <1/10 high power fields. No cellular atypia or necrosis was present. The tumor cells were positive for vimentin and nuclear β-catenin staining.

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Case Report

Intracranial Fibromatosis: A Case Report.: Jeong Ju Lee, Jeoung Hun Kim, Shin Kwang Khang, Kyung Ja Cho, Jihun Kim; Korean J Pathol. 2011;45:S89-S92.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.S1.S89

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Abstract PDF

Fibromatosis can occur at various sites, but intracranial fibromatosis is exceptionally rare. Here, we report a case of intracranial fibromatosis arising in the suprasellar area of a 52-year-old woman who had undergone a surgery at that site. A computed tomography scan revealed a heavily calcified, highly enhancing, poorly demarcated mass in the left sellar area that extended into the left suprasellar, parasellar areas, and orbital apex and completely encased the left distal inferior cerebral artery. Histologic and immunohistochemical features were compatible with those of fibromatosis, although the cellularity was focally higher than usual. The etiology of extra-abdominal fibromatosis is unknown, but physical injuries such as trauma and irradiation have been reported to be associated with its occurrence. Although fibromatosis is rare in the intracranial area, it should be considered as a differential diagnosis when an intracranial mass occurs at a previously injured site.

Citations

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Infantile Intracranial Aggressive Fibromatosis: Report of Two Cases with a Review of the Literature
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Original Article

Infantile Myofibromatosis(Congenital Generalized Fibromatosis): Associated with multiple congenital malformations and basaloid follicular hamartomas in the skin.: Eun Sook Nam, Yoo Hun Kim, Han Kyeom Kim, Insun Kim, Je Geun Chi; Korean J Pathol. 1995;29(6):776-782.

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Abstract PDF: Infantile myofibromatosis with systemic involvement is a very rare disease and is characterized by numerous nodules composed of spindle cells of a myofibroblastic nature. There are often disseminated throughout the subcutis, muscle, skeleton and viscera. We report an autopsy case of infantile myofibromatosis in a stillborn female fetus of 32 weeks of gestation. The nodules, Imm to 2 cm, were found over the whole body and viscera. The involved viscera were the heart, tongue, esophagus, gastrointestinal tract, portal areas of the liver, spleen anc pancreas. There were also associated malformations, viz., frontal meningoencephalocele, flexion defer-mities, syndactyly, cleft palate, agenesis of corpus callosum, pachygyria, diaphragmatic hemia, renal hypoplasia, etc. Multiple basaloid follicular hamartomas of the skin were noted on the face and extremeties. There are no previous reports in the literature of infantile myofibromatosis in conjunction with the above skin lesion and congenital malformations.

Case Report

Benign Lymphoepithelial Cyst: A case report.: Jin Haeng Chung, Gyeong Hoon Kang, Je G Chi; Korean J Pathol. 1996;30(6):551-553.

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Abstract PDF: An intraparotid benign lymphoepithelial cyst is a rare disease characterized by unilateral painless swelling of parotid region. The histogenesis is controversial. Surgical excision is recommended for diagnosis and curative treatment. We present a case of benign lymphoepithelial cyst arising in a patient with neurofibromatosis. A 46-year-old woman presented with a slowly growing multilocular cystic mass in the left cheek. The cystic mass measured 4 cm in maximal outer diameter and the cystic wall was thick and yellowish pale to gray, soft with well circumscribed margin. Microscopically, the multilocular cyst was lined by stratified squamous epithelium for the most part and underlying lymphoid tissue aggregates with follicles and sharply demarcated from adjacent salivary parenchyma which is of normal appearance and without lymphoid aggregates. Since this lesion is absolutely benign, it is important to separate this benign cyst from cystic salivary gland tumors.

Original Article

Juvenile Hyaline Fibromatosis in an Adult.: Young A Kim, Seoung Wan Chae, Chong Jai Kim, Je G Chi; Korean J Pathol. 2000;34(3):239-242.

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Abstract PDF: Juvenile hyaline fibromatosis is a rare disorder probably inherited as an autosomal recessive trait. It is characterized by multiple slowly growing subcutaneous nodules, hypertrophy of gingiva, flexion contracture, and radiolucent bone destruction. The histological features of the tumor-like lesions are characterized by the deposition of amorphous hyaline material in which spindle shaped cells are embedded. We report a case of juvenile hyaline fibromatosis in a 26 year-old-woman. She had multiple subcutaneous nodules in scalp, ear, forearms, right knee, and back. Surgical excision of the tumors in the scalp and ear was done. The largest one measured 13 9 6 cm, and had homogeneous, grayish yellow cut surface with calcification. Light microscopic examination showed abundant eosinophilic hyaline material with extensive calcification and metaplastic bone formation. Spindle cells were rarely observed at the periphery of the tumor. Hyaline matrix was PAS positive, diastase resistant, and alcian blue negative. Scattered spindle cells were positive for vimentin but negative for S-100 protein and smooth muscle actin. There were many reports regarding early lesions of juvenile hyaline fibromatosis; however in this patient, tumor existed for more than 20 years and the histology was somewhat different from the early lesions reported in the literature.

Case Reports

Multiple Plexiform Schwannomas Associated with Neurofibromatosis Type 2: A case report.: Ho Sung Park, Myoung Ja Chung, Myoung Jae Kang, Dong Geun Lee, Byung Cook Ahn; Korean J Pathol. 2000;34(5):389-392.

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Abstract PDF: Plexiform schwannoma is a rare benign tumor arising from the peripheral nerve sheath and characterized by a multinodular and plexiform growth pattern. This tumor usually arises sporadically. In rare cases, plexiform schwannomas have been associated with neurofibromatosis type 2. Plexiform schwannoma should be differentiated from plexiform neurofibroma, because the latter is pathognomonic tumor of neurofibromatosis type 1 and has a potential of malignant transformation. We report a case of multiple plexiform schwannomas associated with bilateral acoustic neuromas and meningioma.

Fibromatosis of the Parotid Gland: A Case Report.: Dae Su Kim, Chulhwan Kim, Insun Kim; Korean J Pathol. 2002;36(2):112-114.

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Abstract PDF: A 51-year-old woman was presented with a palpable mass in the infraauricular area that had existed for 4-months. The mass from the superficial lobe of the parotid gland was 2.7 cm in the greatest dimension and was ill-defined with rubbery, homogeneous, and fibrotic appearance. Histologically, the lesion was infiltrative into the lobules of the paratid gland, and was composed of a proliferation of spindle or stellate cells with cellular and sclerotic areas. On immunohistochemistry, the cells were only positive for vimentin and focally for smooth muscle actin. Differential diagnosis from nerve sheath tumors and solitary fibrous tumors involving the parotid gland was emphasized.

Fine Needle Aspiration Cytologic Findings of Fibromatosis Colli: A Report of Three Cases.: In Suh Park, Lucia Kim, Suk Jin Choi, Jee Young Han, Joon Mee Kim, Young Chae Chu, Sun Geun Choi; Korean J Cytopathol. 2005;16(1):61-65.

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Abstract PDF: Fibromatosis colli is a benign fibrous tissue proliferation of sternocleidomastoid muscle, which is usually observed during the first month of life, often associated with congenital torticollis. It should be differentiated from other neck masses in infants because the usual initial treatment of fibromatosis colli is conservative management and invasive therapy should be avoided. Fine needle aspiration cytology provides an excellent minimally invasive diagnostic way for evaluation of infantile neck masses. We describe three cases of fibromatosis colli diagnosed by fine needle aspiration cytology. All of them were younger than one month and presented as a neck mass. Clinical impressions were malignant tumors in two cases and fibromatosis colli in one case. Fine needle aspiration cytology revealed benign and mature fibroblasts and atrophic striated muscle fibers. The cytologic diagnosis was fibromatosis colli or benign fibous lesion.

Intraneural Perineurioma in the Tongue: A Case Report.: Jun Kang, Shin Kwang Khang, Jene Choi, Jeong Won Kim, Eul Ju Seo, Bu kyu Lee, Eunsil Yu; Korean J Pathol. 2007;41(1):51-54.

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Abstract PDF: We report a case of an intraneural perineurioma that developed in an unusual location, the tongue. A 16-year-old male presented with a 1 cm sized protruding submucosal mass in his tongue without any sensory or motor signs or symptoms. The mass was excised. The mucosa was intact, with an ill-defined firm mass measuring 1.0 x 0.8 x 0.6 cm in the submucosa and muscle. The cut surface of the mass was pinkish gray and fibrotic. Microscopically, the mass contained tortuous and thickened peripheral nerve bundles in the submucosa, showing onion bulb like structures. The onion bulb like structures consisted of centrally located S-100 protein positive Schwann cells surrounded by Glut-1 positive perineurial cells. The FISH study did not reveal any genetic aberrations in chromosome 22.

Gastrointestinal Stromal Tumors associated with Neurofibromatosis Type I: A Report of Two Cases.: Joo Heon Kim, Ock Seong In, Seong Kyu Lee, Haing Woon Baik, Seong Ho Kim, Dong Wook Kang, Kyung Hee Kim, Mee Ja Park, Yong Il Kim; Korean J Pathol. 2006;40(2):137-141.

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Abstract PDF: Gastrointestinal stromal tumor (GIST) is the most common non-epithelial neoplasm arising in the gastrointestinal tract, but this tumor is rarely seen in association with type 1 neurofibromatosis (NF-1). We report here on two cases of multiple GISTs of the small intestine that occurred in NF-1 patients. We also analyzed the mutations of c-kit exons 9, 11, 13 and 17 and the plateletderived growth factor receptor-alpha (PDGFRA) exons 12 and 18 in two GIST patients. Histologically, the NF-1-associated GISTs were similar to those of non-the NF-1 GISTs, but they characteristically revealed hyperplastic interstitial cells of Cajal around the GISTs. Immunohistochemically, these tumors showed strong co-expressions of CD117 and CD34. The molecular genetic analysis of the GISTs showed that all of the c-kit and PDGFRA exons that were analyzed in the GISTs of the two patients were the wild-type, suggesting a limited role for the c-kit and PDGFRA mutations in the tumorigenesis of NF-1-associated GISTs.

Fibromatosis of the Breast: A Case Report.: Hyun Joong Kim, Kyung Hwa Lee, Jo Heon Kim, Min Keun Shim, Ji Shin Lee, Chan Choi; Korean J Pathol. 2005;39(2):137-139.

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Abstract PDF: Fibromatosis of the breast is a rare tumor. We describe here a case of mammary fibromatosis in a 37-year-old woman. The mass from the right breast was 3 cm at the greatest dimension. The lesion was poorly circumscribed, firm and white-gray on the cut surface. Histologically, the lesion infiltrated into the lobules of the breast, and the tumor was composed of relatively uniform fibroblasts and collagen. Neither mitotic activity nor cellular atypia was seen. On the immunohistochemistry, the cells were positive for vimentin and they were focally positive for smooth muscle actin. Staining results for estrogen receptor and progesterone receptor were negative.

Original Articles

Diffuse Neurofibromas: Clinicopathologic Analysis of 11 cases.: So Young Park, Hye Kyung Lee, Se Min Baek; Korean J Pathol. 1995;29(2):181-188.

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Abstract PDF: We reviewed surgical specimens from 11 patients with diffuse neurofibroma to define the specific clinicopathologic characteristics. Ten cases were cutaneous neurofibromas and one case was an uncommon gastrointestinal neurofibroma involving the rectum. The most frequent sites of involvement were the head and neck, especially the eyelids and the periorbital areas. They usually presented as a plaque-like elevation of the skin. They primarily occured in children and young adults and positive family histories of von Recklinghausen's neurofibromatosis were obtained in 45.4%. Pathologically, the involved skin & rectum were diffusely thickened by an infiltrative growing mass, showing proliferation of short fusiform cells in the uniform matrix of fine fibrillary collagen. The characteristic prominence of Wagner-Meissner bodies (45.4%) suggests they could be associated with pathogenesis of diffuse neurofibroma. On the basis of these findings, we could confirm diffuse neurofibroma to be a distinct form of neurofibroma.

Optic Nerve Glioma with Neurofibromatosis.: Na Hye Myong, Seung Sook Lee, Yun Lim Shu, Je G Chi; Korean J Pathol. 1993;27(5):524-530.

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Abstract PDF: Optic nerve gliomas manifest either as a solitary form or a component of von Recklinghausen's neurofibromatosis. The reported incidence of coexistence with neurofibromatosis varies from 12% to 70%. Usually there are no significant cytological differences between the gliomas that accomapny the disease and those that are deemed to be solitary manifestations. The only possible difference between them is the apparently more common association, with the former, of extensive arachnoid hyperplasia and of a more florid local gliomatous infiltration into the leptomeninges, altogether resulting in perineural thickening. Our cases were 8 and 6 years old girls, respectively, presented with slowly progressive proptosis for 4 years and visual disturbance for 2.5 months. There were multiple cafe au lait spots on their trunks, and case 2 showed Lisch nodules in the iris. MRI of brain revealed unilateral optic nerve thickening with involvement of chiasm or multiple intracranial lesions. Resection of optic nerve tumor was performed. Microscopically, variable degree of tumorous change was seen. Most typically enlarged area was composed of intraneural and perineural portions surrounded by a layer of intact dura. Intraneural tumor revealed proliferation of elongated, spindle-shaped pilocytic astrocytes in intertrabecular spaces and distention of the pial septa with fibrovascular thickening. Another segment had areas with reactive gliosis. Perineural tissue was considerably thickened and, associated with proliferation of meningothelial cells and fibroblasts intermingled with astrocytes and Rosenthal fibers. There were increase of the optic nerve diameter and distention of the overlying dura. Foci of arachnoid cell hyperplasia were noted in both cases, although differed in degree. Immunohistochemically, the tumor cells expressed glial fibrillary acidic protein in intraneural and perineural portions particularly in case 2.

Case Report

A Case of Ocular Neurofibromatosis.: Je G Chi, In Ae Park; Korean J Pathol. 1987;21(1):62-65.

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Abstract PDF: Neurofibromatosis is one of the neurocristopathies that involve many system or tissues forming various types of lesion. Almost every tissue or organ can be involved by this disease. However, the eyeball itself is very rarely affected by this process. The findings seen in our case indicate the diversity of lesions in neurofibromatosis, and also suggest hamartomatous nature. Heterotopic ganglion cells and glial cells in uveal tract are not easily understood. We reported here a case of intra-occular neurofibromatosis with its characteristic involvement of the uveal tract, in a 21 year old female. Her ocular symptoms began at her age of 11 as poor vision and were slowly progressive together with multiple facial neurofibromas. The involved left eyeball showed many ganglioneuroglial cell nests in iris, ciliary body and retina. Minute plexiform neurofibromas were also seen in small nerve twigs around the eyeball.

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