Dermatofibrosarcoma protuberans

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Dermatofibrosarcoma protuberans
Other namesDFSP [1]
Dermatofibrosarcoma protuberans (1) recurrence.JPG
Histopathological image of dermatofibrosarcoma protuberans. Local recurrence long after the first excision. H&E stain
Specialty Oncology   OOjs UI icon edit-ltr-progressive.svg

Dermatofibrosarcoma protuberans (DFSP) [2] is a rare locally aggressive malignant cutaneous soft-tissue sarcoma. DFSP develops in the connective tissue cells in the middle layer of the skin (dermis). [3] Estimates of the overall occurrence of DFSP in the United States are 0.8 to 4.5 cases per million persons per year. [4] [5] In the United States, DFSP accounts for between 1 and 6 percent of all soft-tissue sarcomas [6] and 18 percent of all cutaneous soft-tissue sarcomas. In the Surveillance, Epidemiology and End Results (SEER) tumor registry from 1992 through 2004, DFSP was second only to Kaposi sarcoma.

Contents

Presentation

Dermatofibrosarcoma protuberans begins as a minor firm area of skin most commonly about to 1 to 5 cm in diameter. It can resemble a bruise, birthmark, or pimple. It is a slow-growing tumor and is usually found on the torso but can occur anywhere on the body. [7] About 90% of DFSPs are low-grade sarcomas. About 10% are mixed, containing a high-grade sarcomatous component (DFSP-FS); therefore, they are considered to be intermediate-grade sarcomas. DFSPs rarely lead to a metastasis (fewer than 5% metastasize), but DFSPs can recur locally. DFSPs most often arise in patients who are in their thirties but this may be due to diagnostic delay.

Location

Commonly located on the chest and shoulders, the following is the site distribution of DFPS as was observed in Surveillance, Epidemiology, and End Results (SEER) database between 2000 and 2010. [5] [8]

Variants

The World Health Organization in 2020 classified the fibro sarcomatous DFSP (DFSP-FS) variant (also termed dermatofibrosarcoma protuberans, fibro sarcomatous) of the dermatofibrosarcoma protuberans as a specific form of the intermediate (rarely metastasizing) fibroblastic and myofibroblastic tumors and other variants of this disorder as a specific form of the intermediate (locally aggressive) fibroblastic and myofibroblastic tumors. [9]

Bednar tumors

Bednar, or pigmented DFSP, is distinguished by the dispersal of melanin-rich dendritic cells of the skin. It represents 1–5 percent [10] of all DFSP occurring in people rich in melanin pigments. Bednar is characterized by a dermal spindle cell proliferation like DFSP but distinguished by the additional presence of melanocytic dendritic cells. It occurs at the same rate as DFSP on fairer skin and should be considered to have the same chances of metastasis. [11]

Myxoid DFSP

Myxoid DFSP has areas of myxoid degeneration in the stroma. [12] [13]

Giant cell fibroblastoma

Giant cell fibroblastoma [2] contains giant cells, and is also known as juvenile DFSP. [14] Giant cell fibroblastomas are skin and soft-tissue tumors that usually arise in childhood. They are sometimes seen in association with dermatofibrosarcoma protuberans (DFSP, hybrid lesions) or may transform or recur as DFSP. [15] [13]

Atrophic DFSP

Atrophic DFSP resemble other benign lesions such as morphea, idiopathic atrophoderma, atrophic scar, anetoderma or lipoatrophy. It behaves like classic DFSP. It commonly favours young to middle-aged adults. It has a slow infiltrative growth and a high rate of local recurrence if not completely excised. [13] [16]

Sclerosing DFSP

Sclerosing DFSP is a variant in which the cellularity is low, and the tumor consists of uniform bundles of collagen interspersed with more typical DFSP cells. [13]

Granular cell variant is a rare type in which spindle cells are mingled with richly granular cells, the granules being lysosomal, with prominent nucleoli. [13]

Fibrosarcomatous DFSP (DFSP-FS)

Fibrosarcomatous DFSP is a rare variant of DFSP involving greater aggression, high rates of local occurrences, and higher metastatic potential. [17] DFSP-FS are considered to be intermediate-grade sarcomas, [18] although they rarely metastasize (fewer than 5 percent of cases).

Pathophysiology

More than 90% of DFSP tumors have the chromosomal translocation t(17;22). The translocation fuses the collagen gene (COL1A1) with the platelet-derived growth factor (PDGF) gene. The fibroblast, the cell of origin of this tumor, expresses the fusion gene in the belief that it codes for collagen. However, the resulting fusion protein is processed into a mature platelet-derived growth factor which is a potent growth factor. Fibroblasts contain the receptor for this growth factor. Thus the cell "thinks" it is producing a structural protein, but it creates a self-stimulatory growth signal. The cell divides rapidly and tumor forms.

The tissue is often positive for CD34. [19] [20]

Diagnosis

The pattern of tumor cells radiating from a center is described as "cartwheel pattern". Histopathology of cartwheel pattern in dermatofibrosarcoma protuberans, annotated.jpg
The pattern of tumor cells radiating from a center is described as "cartwheel pattern".

DFSP is a malignant tumor diagnosed with a biopsy, when a portion of the tumor is removed for examination. In order to ensure that enough tissue is removed to make an accurate diagnosis, the initial biopsy of a suspected DFSP is usually done with a core needle or a surgical incision. [21]

Clinical palpation is not entirely reliable for ascertaining the depth of a DFSP infiltration. Magnetic resonance imaging (MRI) is more sensitive addressing the depth of the invasion on some types of DFSP, particularly large or recurring tumors, [22] [23] though MRI is less accurate for identifying infiltration to head and neck tumors.

Diagnostic delay and misdiagnosis

Due to the rarity, initial presentation of flat plaque (skin hardening) and the slow-growing nature of DFSP, it may be months to years without a protuberance (bump). The dissonance between the name of the neoplasm and its clinical presentations may cause a majority of patients to experience a diagnostic delay. A 2019 research study found out of 214 patients a range between less than a year to 42 years before diagnosis (median, four years) from patients noticing a symptom to diagnosis. [24]

Currently, a majority of patients (53%) receive a misdiagnosis by health care providers. The most frequent prebiopsy clinical suspicion included cyst (101 [47.2%]), lipoma (30 [14.0%]), and scar (17 [7.9%]). [24]

It has been suggested an alternative term for DFSP should be dermatofibrosarcoma, often protuberant. [24]

Pregnancy

It is suggested that DFSPs may enlarge more rapidly during pregnancy. Immunohistochemical stains for CD34, S-100 protein, factor XIIIa, and estrogen and progesterone receptors were performed on biopsy specimens. The tumors showed the expression of the progesterone receptor. As with many other stromal neoplasms, DFSPs appear to express low levels of hormone receptors, which may be one factor that accounts for their accelerated growth during pregnancy. [25]

Treatment

Dermatofibrosarcoma protuberans of the left axilla. CT, coronal reconstruction Dermatofibrosarcoma protuberans CT cor.jpg
Dermatofibrosarcoma protuberans of the left axilla. CT, coronal reconstruction

Treatment is primarily surgical, with chemotherapy and radiation therapy used if clear resection margins are not acquired. [26]

Surgical treatment

The type of surgical treatment chosen is dependent on the location of the DFSP occurrence and possible size.

Mohs surgery

Mohs micrographic surgery (MMS) has a high cure rate and lowers the recurrence reduction of DFSP [27] if negative resection margins are achieved.

Wide local excision

Wide local excision (WLE) was the gold standard for treating DFSP but is currently under reevaluation. Presently in the United States, WLE may be suggested after the recurrence of MMS. Larger resection margins are suggested for WLE than MMS. Recurrence rate with WLE is about 8.5% with a lower recurrence rate related to wider excision. [28]

Resection margin

DFSP characteristic features are its capacity to invade surrounding tissues, to a considerable distance from the central focus of the tumor in a "tentacle-like" fashion. This fact, coupled with diagnostic delay, may lead to inadequate initial resection. Inadequate initial treatment results in larger, deeper recurrent lesions, but these can be managed by appropriate wide excision. [29]

Radiation therapy

DFSP is a radioresponsive tumor; radiation therapy (RT) is not used as the first choice for treatment. Conservative resection through MMS or WLE is attempted first. If clear margins are not achieved RT, or chemotherapy is recommended. [30]

Chemotherapy

DFSP was previously regarded and nonresponsive to standard chemotherapy. [31] In 2006 the US FDA approved (imatinib mesylate) for the treatment of DFSP. [32] As is true for all medicinal drugs with name ending in "ib," imatinib is a small molecular pathway inhibitor; imatinib inhibits tyrosine kinase. It may be able to induce tumor regression in patients with recurrent DFSP, unresectable DFSP, or metastatic DFSP. [33] There is clinical evidence that imatinib, which inhibits PDGF-receptors, may be effective for tumors positive for the t(17;22) translocation. It is suggested that imatinib may be a treatment for challenging, locally advanced disease and in the rare metastatic cases. It was approved for use by adult patients with unresectable, recurrent and/or metastatic dermatofibrosarcoma protuberans (DFSP). [34]

Metastatic disease

Distant hematogenous metastases are extremely rare. [35] Metastases to regional lymph nodes are rarer and are most likely in patients who have had multiple local recurrences after inadequate surgical resection. [36] Repeatedly recurring tumors have an increased risk for transformation into a more malignant form (DFSP-FS). The lungs are most frequently affected, but metastases to the brain, [37] bone, [38] and other soft tissues are reported.

Studies

DFSP is not extensively studied due to its rarity and low mortality. The majority of studies are small size case studies or meta-analysis.

The most extensive research study to date was Perspectives of Patients With Dermatofibrosarcoma Protuberans on Diagnostic Delays, Surgical Outcomes, and Nonprotuberance. [24] The lead researcher, Jerad Gardner, spoke at a TED Talk in February 2020 on the topic. [39]

History

R. W. Taylor, in 1890, [40] first identified DFSP as a keloid sarcoma. Later in 1924, Ferdinand-Jean Darier and Ferrand identified it as a progressive recurrent dermatofibroma. In 1925, E. Hoffmann [41] coined the term dermatofibrosarcoma protuberans. Bednar tumor was first described by Bednar in 1957. [42] [43]

ICD coding

The following are the ICD-10 medical codes:

Additional images

See also

Related Research Articles

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Low-grade fibromyxoid sarcoma (LGFMS) is a rare type of low-grade sarcoma first described by H. L. Evans in 1987. LGFMS are soft tissue tumors of the mesenchyme-derived connective tissues; on microscopic examination, they are found to be composed of spindle-shaped cells that resemble fibroblasts. These fibroblastic, spindle-shaped cells are neoplastic cells that in most cases of LGFMS express fusion genes, i.e. genes composed of parts of two different genes that form as a result of mutations. The World Health Organization (2020) classified LGFMS as a specific type of tumor in the category of malignant fibroblastic and myofibroblastic tumors.

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Fibroblastic and myofibroblastic tumors (FMTs) develop from the mesenchymal stem cells which differentiate into fibroblasts and/or the myocytes/myoblasts that differentiate into muscle cells. FMTs are a heterogeneous group of soft tissue neoplasms. The World Health Organization (2020) defined tumors as being FMTs based on their morphology and, more importantly, newly discovered abnormalities in the expression levels of key gene products made by these tumors' neoplastic cells. Histopathologically, FMTs consist of neoplastic connective tissue cells which have differented into cells that have microscopic appearances resembling fibroblasts and/or myofibroblasts. The fibroblastic cells are characterized as spindle-shaped cells with inconspicuous nucleoli that express vimentin, an intracellular protein typically found in mesenchymal cells, and CD34, a cell surface membrane glycoprotein. Myofibroblastic cells are plumper with more abundant cytoplasm and more prominent nucleoli; they express smooth muscle marker proteins such as smooth muscle actins, desmin, and caldesmon. The World Health Organization further classified FMTs into four tumor forms based on their varying levels of aggressiveness: benign, intermediate, intermediate, and malignant.

Lipofibromatosis-like neural tumor (LPF-NT) is an extremely rare soft tissue tumor first described by Agaram et al in 2016. As of mid-2021, at least 39 cases of LPF-NT have been reported in the literature. LPF-NT tumors have several features that resemble lipofibromatosis (LPF) tumors, malignant peripheral nerve sheath tumors, spindle cell sarcomas, low-grade neural tumors, peripheral nerve sheath tumors, and other less clearly defined tumors; Prior to the Agaram at al report, LPF-NTs were likely diagnosed as variants or atypical forms of these tumors. The analyses of Agaram at al and subsequent studies uncovered critical differences between LPF-NT and the other tumor forms which suggest that it is a distinct tumor entity differing not only from lipofibromatosis but also the other tumor forms.

Myxofibrosarcoma (MFS), although a rare type of tumor, is one of the most common soft tissue sarcomas, i.e. cancerous tumors, that develop in the soft tissues of elderly individuals. Initially considered to be a type of histiocytoma termed fibrous histiocytoma or myxoid variant of malignant fibrous histiocytoma, Angervall et al. termed this tumor myxofibrosarcoma in 1977. In 2020, the World Health Organization reclassified MFS as a separate and distinct tumor in the category of malignant fibroblastic and myofibroblastic tumors.

Sclerosing epithelioid fibrosarcoma (SEF) is a very rare malignant tumor of soft tissues that on microscopic examination consists of small round or ovoid neoplastic epithelioid fibroblast-like cells, i.e. cells that have features resembling both epithelioid cells and fibroblasts. In 2020, the World Health Organization classified SEF as a distinct tumor type in the category of malignant fibroblastic and myofibroblastic tumors. However, current studies have reported that low-grade fibromyxoid sarcoma (LGFMS) has many clinically and pathologically important features characteristic of SEF; these studies suggest that LGSFMS may be an early form of, and over time progress to become, a SEF. Since the World Health Organization has classified LGFMS as one of the malignant fibroblastic and myofibroblastic tumors that is distinctly different than SEF, SEF and LGFMS are here regarded as different tumor forms.

Dermatofibrosarcoma protuberans, fibrosarcomatous (DFSP-FS), also termed fibrosarcomatous dermatofibrosarcoma protuberans, is a rare type of tumor located in the dermis. DFSP-FS tumors have been viewed as: 1) a more aggressive form of the dermatofibrosarcoma protuberans (DFSP) tumors because they have areas that resemble and tend to behave like malignant fibrosarcomas or 2) as a distinctly different tumor than DFSP. DFSP-FS tumors are related to DFSP. For example, surgically removed DFSP tumors often recur with newly developed fibrobosarcoma-like areas. Nonetheless, the World Health Organization (WHO), 2020, classified DFSP and DFSP-FS as different tumors with DFSP being in the category of benign and DFSP-FS in the category of rarely metastasizing fibroblastic and myofibroblastic tumors. This article follows the WHO classification: the 5-15% of DFSP tumors that have any areas of fibrosarcomatous microscopic histopathology are here considered DFSP-FS rather than DFSP tumors.

Gardner fibroma (GF) is a benign fibroblastic tumor. GF tumors typically develop in the dermis and adjacent subcutaneous tissue lying just below the dermis. These tumors typically occur on the back, abdomen, and other superficial sites but in rare cases have been diagnoses in internal sites such as the retroperitoneum and around the large blood vessels in the upper thoracic cavity. The World Health Organization, 2020, classified Gardner fibroma as a benign tumor in the category of fibroblastic and myofibroblastic tumors.

References

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  24. 1 2 3 4 David, Marjorie Parker; Funderburg, Ashley; Selig, James P.; Brown, Rebecca; Caliskan, Pip M.; Cove, Lee; Dicker, Gayle; Hoffman, Lori; Horne, Tammi; Gardner, Jerad M. (2019-08-30). "Perspectives of Patients With Dermatofibrosarcoma Protuberans on Diagnostic Delays, Surgical Outcomes, and Nonprotuberance". JAMA Network Open. 2 (8): e1910413. doi:10.1001/jamanetworkopen.2019.10413. ISSN   2574-3805. PMC   6724159 . PMID   31469398.
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  27. ↑ Malan, Malumani; Xuejingzi, Wu; Quan, Song Ji (2019-08-13). "The efficacy of Mohs micrographic surgery over the traditional wide local excision surgery in the cure of dermatofibrosarcoma protuberans". The Pan African Medical Journal. 33: 297. doi:10.11604/pamj.2019.33.297.17692. ISSN   1937-8688. PMC   6815477 . PMID   31692830.
  28. ↑ Kim, Byung Jun; Kim, Hyeonwoo; Jin, Ung Sik; Minn, Kyung Won; Chang, Hak (2015). "Wide Local Excision for Dermatofibrosarcoma Protuberans: A Single-Center Series of 90 Patients". BioMed Research International. 2015: 642549. doi: 10.1155/2015/642549 . ISSN   2314-6133. PMC   4673335 . PMID   26688814.
  29. ↑ Khatri, Vijay P.; Galante, Joseph M.; Bold, Richard J.; Schneider, Philip D.; Ramsamooj, Rajendra; Goodnight, James E. (November 2003). "Dermatofibrosarcoma protuberans: reappraisal of wide local excision and impact of inadequate initial treatment". Annals of Surgical Oncology. 10 (9): 1118–1122. doi:10.1245/aso.2003.03.581. ISSN   1068-9265. PMID   14597453. S2CID   41310745.
  30. ↑ Suit, H.; Spiro, I.; Mankin, H. J.; Efird, J.; Rosenberg, A. E. (August 1996). "Radiation in management of patients with dermatofibrosarcoma protuberans". Journal of Clinical Oncology. 14 (8): 2365–2369. doi:10.1200/JCO.1996.14.8.2365. ISSN   0732-183X. PMID   8708729.
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  33. ↑ Rastogi, Sameer; Dhamija, Ekta; Barwad, Adarsh; Aggarwal, Aditi; Sharma, Atul; Panday, Rambha (December 2018). "Advanced Dermatofibrosarcoma Protuberans Treatment With Imatinib: Experience From a Dedicated Sarcoma Medical Oncology Clinic in India". Journal of Global Oncology. 4 (4): 1–7. doi:10.1200/JGO.18.00007. PMC   6223511 . PMID   30085879.
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  35. ↑ Mavili, M. E.; Gursu, K. G.; Gokoz, A. (April 1994). "Dermatofibrosarcoma with lymph node involvement". Annals of Plastic Surgery. 32 (4): 438–440. doi:10.1097/00000637-199404000-00022. ISSN   0148-7043. PMID   8210168.
  36. ↑ Rutgers, E. J.; Kroon, B. B.; Albus-Lutter, C. E.; Gortzak, E. (June 1992). "Dermatofibrosarcoma protuberans: treatment and prognosis". European Journal of Surgical Oncology: The Journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology. 18 (3): 241–248. ISSN   0748-7983. PMID   1607035.
  37. ↑ Mahajan, B. B.; Sumir, Kumar; Singla, Monika (July 2015). "Metastatic dermatofibrosarcoma protuberans: A rare case report from North India". Journal of Cancer Research and Therapeutics. 11 (3): 670. doi: 10.4103/0973-1482.146099 . ISSN   1998-4138. PMID   26458720.
  38. ↑ Garg, Mandeep Kumar; Yadav, Mukesh Kumar; Gupta, Suruchi; Kumar, Narender; Khandelwal, Niranjan (2009-09-29). "Dermatofibrosarcoma protuberans with contiguous infiltration of the underlying bone". Cancer Imaging. 9 (1): 63–66. doi:10.1102/1470-7330.2009.0011 (inactive 31 January 2024). ISSN   1740-5025. PMC   2792083 . PMID   19933019.{{cite journal}}: CS1 maint: DOI inactive as of January 2024 (link)
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  40. ↑ Shen, Kuang-Hsuan; Leu, Yi-Shing (2017-09-01). "Dermatofibrosarcoma protuberans of the cheek". Journal of Cancer Research and Practice. 4 (3): 119–121. doi: 10.1016/j.jcrpr.2017.03.001 . ISSN   2311-3006.
  41. ↑ Hoffmann, Erich (1925). "I. Über das knollentreibende Fibrosarkom der Haut (Dermatofibrosarkoma protuberans)". Dermatology. 43 (1–2): 1–28. doi:10.1159/000250699. ISSN   1018-8665.
  42. ↑ "Bednar tumour: an infrequent diagnosis | British Journal of Medical Practitioners". www.bjmp.org. Retrieved 2020-06-09.
  43. ↑ Bednar, B. (March 1957). "Storiform neurofibromas of the skin, pigmented and nonpigmented". Cancer. 10 (2): 368–376. doi: 10.1002/1097-0142(195703/04)10:2<368::aid-cncr2820100218>3.0.co;2-3 . ISSN   0008-543X. PMID   13426994. S2CID   42490204.
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