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E. Fisher, H. Hellstrom (1966)
Dermatofibrosarcoma with metastases simulating Hodgkin's disease and reticulum cell sarcomaCancer, 19
E. Rutgers, B. Kroon, A. Ce, E. Gortzak (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
M. Mavili, K. Gursu, A. Gokoz (1994)
Dermatofibrosarcoma with lymph node involvement.Annals of plastic surgery, 32 4
W. Wooldridge (1957)
Dermatofibrosarcoma protuberans; a tumor too lightly considered.A.M.A. archives of dermatology, 75 1
L. Kahn, N. Saxe, W. Gordon (1978)
Dermatofibrosarcoma protuberans with lymph node and pulmonary metastases.Archives of dermatology, 114 4
(1958)
Klinik and Pathologic des dermatofibrosarcoma protuberans
Shinichi Hirabayashi, A. Kajikawa, K. Kanazawa, K. Mimoto (1989)
Dermatofibrosarcoma protuberans with regional lymph node metastasis: A case reportHead & Neck, 11
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Dermatofibrosarcoma protuberans metastatic to a regional lymph node. Report of a case and reviewCancer, 36
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Dermatofibrosarcoma protuberans with lymph node involvement. A case report of simultaneous occurrence with an atypical fibroxanthoma of the skin.Archives of Dermatology, 114
Cervical lymph node showing tumor cells (Â10) (H&E stain)
R. Volpe, A. Carbone (1983)
Dermatofibrosarcoma protuberans metastatic to lymph nodes and showing a dominant histiocytic componentThe American Journal of Dermatopathology, 5
H. Gentele (1951)
Malignant, fibroblastic tumors of the skin; clinical and pathological-anatomical studies of 129 cases of malignant, fibroblastic tumors from the cutaneous and subcutaneous layers observed at Radiumhemmet during the period 1927-1947.Acta dermato-venereologica. Supplementum, 27
L. Przybora, Czes lstrok, aw Wojnerowicz (1959)
Malignancy of dermatofibrosarcoma protuberans and report of 2 cases with lymph gland metastases.Oncologia, 12
P. Lal, Rajeev Sharma, H. Mohan, M. Sekhon (1999)
Dermatofibrosarcoma protuberans metastasizing to lymph nodes: A case report and review of literatureJournal of Surgical Oncology, 72
D. Petoin, O. Verola, P. Banzet, C. Dufourmentel, J. Servant (1985)
Dermatofibrosarcome de Darier et Ferrand. Etude de 96 cas sur 15 ansChirurgie, 111
Dermatofibrosarcoma protuberans (DFSP) is an uncommon, slow growing and locally aggressive tumor of the skin with a high rate of recurrence even after supposedly wide excision. The reports of regional lymph node metastasis and distant metastasis are very rare. Because of the extreme rarity of these cases with metastasis, the experience with management of such patients is very limited. A case of recurrent DFSP of scalp, with metastasis to the regional lymph nodes, in a 17-year-old boy is reported here. This is the second case of DFSP involving scalp and 16th case of DFSP of all sites metastasizing to the regional lymph nodes reported in literature. The patient was treated with wide excision of the lesion and ipsilateral radical neck dissection (including excision of overlying involved skin). Key words: dermatofibrosarcoma protuberans, lymph nodal, metastasis Case report neck. It was fixed to sternocleidomastoid muscle and adherent to overlying skin. There was no ulceration A 17-year-old boy was admitted to Lok Nayak in the neck (Fig. 1). Multiple lymph nodes were Hospital with the chief complaints of a gradually palpable in the posterior triangle of the neck. They increasing swelling in the right occipito-temporal were mobile, firm in consistency, non-tender with region of the scalp for 11 years and multiple swellings the largest about 2 cm in size. A clinical diagnosis of in the ipsilateral neck for 1 year. His parents had first soft tissue tumor of the scalp with metastasis in the noticed a small 2 2-cm swelling on the right side of neck was made. Skiagram of the chest showed no the scalp when the patient was 6 years old. This evidence of metastasis. CT Scan of head and neck swelling was excised at a small peripheral medical was suggestive of a malignant soft tissue tumor in center when the patient was 10 years of age. Till the scalp with metastasis in the neck with normal then, it increased in size very slowly. The parents did calvarium and intracranial structures. Fine needle not have any medical record or histopathological aspiration cytology from the neck tumor was report. The swelling reappeared about 1 year after reported to be a malignant mesenchymal lesion the excision. Since then, it had been again increasing suggestive of DFSP. in size slowly and painlessly. One year prior to The patient underwent wide excision of the presentation to us, the patient also noticed a few primary on the scalp with a 4-cm margin all swellings in the right half of the neck. These were around. The neck nodes were managed by radical also painless and slow to increase in size. neck dissection on the right side of the neck with General physical and systemic examination of wide excision (taking a 4-cm margin all around) of the patient showed no abnormality or unusual the involved adherent skin. The scalp lesion was feature. All the findings were localized to head and adherent to the periosteum over a 2.5-cm diameter neck. Examination of the scalp showed an 8 6 area in the central region of the tumor, and hence 4-cm fungating, non-tender swelling over the right the periosteum also required removal. The rest of temporo-parieto-occipital region. It was mobile over the area had a healthy and intact periosteum. The the scalp and firm in consistency. There was another denuded bone was healthy. The whole of the defect swelling, 4 5 cm in size, on the right side of the on the scalp and the neck was covered with a split Correspondence to: Dr. Pawan Lal, C–63, Preet Vihar, Delhi 110092, India. Tel.: +91-11-2014727; E-mail: pawanlal@vsnl.com 1357-714X print/1369–1643 2004 Taylor & Francis Ltd DOI: 10.1080/13577140410001679257 44 P. Lal et al. Fig. 2. Five weeks postoperative photograph showing complete Fig. 1. Pre-operative photograph showing the primary scalp healing of the scalp and neck areas. tumor with cervical lymph node metastases. skin graft from the thigh. As expected, there was a small graft loss over the denuded bone, but conser- vative management using dressings with antibiotic ointment and petrolatum gauze on every third day allowed the area to develop granulation tissue and spread of the graft. Complete healing occurred in 5 weeks (Fig. 2). The patient is disease-free in the follow-up period of 18 months duration. Histopathological examination of the excised scalp specimen showed the tumor cells to be spindle- shaped and at places with a storiform pattern (Fig. 3). The tumor was poorly circumscribed and showed infiltrative margins. At a few places, the storiform Fig. 3. High power detail (40) of scalp specimen with pattern gave way to more fascicular areas. The spindle-shaped cells in storiform pattern (H&E stain). overlying skin showed ulceration and infiltration by the tumor cells. The neck specimen showed involvement is even rarer. In an extensive review of histopathological features similar to the scalp lesion. literature by Rutgers et al. involving 913 cases, 11 Although, there was no ulceration, the tumor cells were found to have regional lymph node metastasis. were reaching up to the epidermis. The lymph nodes They even tabulated the various parameters of all from the posterior triangle of neck showed tumor these cases reported by various authors. However, a metastasis (Fig. 4). later article by Mavili et al. reported their own case to be the tenth. Study of these two reports, their Discussion similarities and discrepancies, and other case reports Dermatofibrosarcoma protuberans (DFSP) is a published prior to or later than these review articles tumor with high rate of local recurrence and an brings the figure to 15, with the present author’s case extremely low but definite risk of metastasis. being the 16th case with lymph node metastasis in a Hematogenous spread is very rare and lymphatic case of DFSP. The articles reporting such cases DFSP with metastasis 45 References 1. Rutgers EJTh, Kroon Bin BR, Albus-Lutter CE, Gortzak E. Dermatofibrosarcoma protuberans treatment and prognosis. Eur J Surg Oncol 1992; 18: 241–8. 2. Mavili ME, Gursu KG, Gokoz A. Dermato- fibrosarcoma with lymph node involvement. Ann Plast Surg 1994; 32(4): 438–40. 3. Gentele H. Malignant, fibroblastic tumors of the skin. Acta Dermatol Venereol 1951; 31: 91–132. 4. Woolridge WE. Dermatofibrosarcoma protuberans: a tumor too lightly considered. Arch Dermatol 1957; 75: 132–5. 5. Waldermann F, Hagedoorn M. Klinik and Pathologic des dermatofibrosarcoma protuberans. Z Hautkr 1958; 60: 1886–94. Fig. 4. Cervical lymph node showing tumor cells (10) 6. Przybora LA, Wojnerowicz C. Malignancy of (H&E stain). dermatofibrosarcoma protuberans and report of 2 cases with lymph gland metastases. Oncologia 1959; 12: 236–54. according to their year of publication are those by 7. Fisher ER, Hellstrom HR. Dermatofibrosarcoma 3 4 with metastasis simulating Hodgkin’s disease and Gentele in 1951, Woolridge in 1957, Waldermann 5 6 reticulum cell sarcoma. Cancer 1966; 19: 1165–71. et al. in 1958, Przybore et al. in 1959, Fisher et al. 7 8 8. Brenner W, Schaefler K, Chhabra H, Postel A. in 1966, Brenner et al. in 1975, Kahn et al. Dermatofibrosarcoma protuberans metastatic to a 9 10 in 1978, Hausner et al. in 1978, Volpe et al.in regional lymph node. Report of a case and review. 11 12 1983, Petoin et al. in 1985, Hirabayashi et al. Cancer 1975; 36: 1897–902. 13 2 9. Kahn LB, Saxe N, Gordon W. Dermatofibrosarcoma in 1989, Mavili et al. in 1994 and Lal et al.in with lymph node and pulmonary metastasis. Arch 1999. Of these articles, only the case reported by Dermatol 1978; 114(4): 599–601. Hirabayashi et al. had involvement of the scalp as 10. Hausner RJ, Vargas-Cortes F, Alexander RW. the primary site. Thus, the case reported by the Dermatofibrosarcoma protuberans with lymph node present authors becomes the second reported case involvement: a case report of simultaneous occurrence with an atypical fibroxanthoma of skin. Arch Dermatol of DFSP of the scalp with metastasis to the regional 1978; 114(1): 88–91. cervical lymph nodes. 11. Volpe R, Carbone A. Dermatofibrosarcoma protuber- In view of the extreme rarity of regional metastasis, ans metastatic to lymph nodes and showing a domi- prophylactic lymph node dissection is not advocated, nant histocytic component. Am J Dermatopathol 1983; while it is but natural to go ahead with it if there 5(4): 327–34. 12. Petoin DS, Verola O, Banzet P, Dufourmantel Cl, is a positive involvement. In the present case, since Servant JM. Dermatofibrosarcoma de Darier et the overlying skin in the neck was also adherent over Ferrand. Etude de 96 cas sur 15 ans. Chirurgie 1985; a large area, a wide excision of the skin was also done 111: 132–8. and the resultant defect covered with split skin graft. 13. Hirabayashi S, Kajikawa A, Kanazawa K, Mimoto K. Dermatofibrosarcoma protuberans with regional lymph node metatstasis: a case report. Head Neck Acknowledgement 1989; 11(6): 562–4. 14. Lal P, Sharma R, Mohan H, Sekhon MS. Thanks to Mr. Subhankar Ghosh, Photographer, Dermatofibrosarcoma protuberans metastasizing to Department of Pathology, Maulana Azad Medical lymph nodes: a case report and review of literature. College, New Delhi, India. J Surg Oncol 1999; 72: 178–80. 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Published: Jan 1, 2004
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