Infantile Digital Fibromatosis- Surgical Indications · Infantile Digital Fibromatosis- Surgical...
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Infantile Digital Fibromatosis- Surgical Indications Abstract Infantile digital fibromatosis (IDF) was first described by Reye in 1965 as involving the fingers and toes exclusively. This is a rare, benign tumor that affects the distal digits on the dorsal or border surfaces. The pink, smooth surfaced tumor can present in a nodular pattern or as individual tumors on neighboring digits as kissing lesions. Demonstrating a predictive histology, the tumor appears as with eosinophilic inclusion bodies with spindle-like fibroblasts. The other key finding are dense sheets of small actin filaments. IFD has a very high potential for recurrence, several studies quoting up to 60%. This makes surgical management challenging. Further, the tumor can spontaneously regress as the child ages, altering the potential for necessitating a surgical excision. This project reviews the English publications on management of this rare entity and hopes to elucidate more clear guidelines to surgical indications. History of Present Illness 11 month old male presents to clinic with history of dorsal mass on left long finger. Birth history was normal. After his birth the mother questioned that he was having difficulty moving the long finger spontaneously with flexion and extension. His mother states the mass developed since 2 months of age. The mass has continued to increase in size. No family history of soft tissue masses. Physical Exam Capillary brisk. Firm nonblanchable multi-lobed mass at the dorsal aspect of left long finger, crossing the distal interphalangeal (DIP) joint. Sensation patient moves all fingers to stimulation. No transillumination. PROM of MP and PIP joint normal. DIP greatly to 0-15 degrees. Mass size estimated at 3 x 3 x 2.5 cm. Impression Left LF dorsal soft tissue mass- fibrous, no cystic component Surgical intervention deemed necessary An ultrasound was obtained of the left long finger to evaluate the nature of the mass and the underlying structure. The mass did not have a distinct separation from the underlying extensor tendon at the distal aspect of the mass. Indications: Loss of range of motion at DIP joint Suspected involvement into the extensor mechanism Patient hygiene compromised Surgery performed Excision of left long finger infantile digital fibroma (Figure A) Split thickness skin graft to left long finger from left groin (Figure B) Literature Review of management References: 1. Reye RDK. Recurring digital fibrous tumors of childhood. Arch Pathol 1965; 80:228-231 2. Kang SK et al. A case of congenital infantile digital fibromatosis. Pediatr Dermatol 2002; 19:462-463 3. Kanwar AJ et al. Congenital infantile digital fibromatosis. Pediatr Dermatol 2002; 19:370-371 4. Sungur N et al. Infantile digital fibromatosis: an unusual localization. J Pediatr Surg 2001; 36:1587-1589 5. Chirayil PT et al. Infantile digital fibromatosis: a case report. Burns 2001; 27:89-90 6. Ishii N et al. A case of infantile digital fibromatosis showing spontaneous regression. Br J Dermatol 1989;121:129-133 7. Kawaguchi M et al. A case of infantile digital fibromatosis with spontaneous regression. J Dermatol 1998;25:523-526 8. Spingardi O et al. Infantile digital fibromatosis: our experience and long-term results. Chir Main 2011;30:62-65 9. Holmes WJ et al. Intralesional steroid for management of symptomatic Infantile Digital Fibromatosis J Plast Reconstr Aesthet Surg 2011;64:632-637 10. Failla V et al. Congenital infantile digital fibromatosis: a case report and review of literature. Rare tumors 2009;1:e47 11. Netscher DT et al. Non-malignant fibrosing tumors in the pediatric hand: a clinicopathologic case review. Hand 2009;4:2-11 12. Taylor HO et al. Infantile digital fibromatosis. Ann Plast Surg 2008; 61:472-476 13. Niamba P et al. Further documentation of spontaneous regression of infantile digital fibromatosis Pediatr Dermatol 2007;24:280-284 14. Fernandez-Jorge B et al. An unusual congential presentation of infantile digital fibromatosis. J Pediatr Child Health 2007;43:505-506 15. Campbell LB, Petrick MG. Mohs micrographic surgery for problematic infantile digital fibroma. Dermatol Surg 2007; 33:385-387 16. Talbot C et al. Infantile digital fibromatosis. J Pedatric Orthop B 2007; 16:110-112 17. Oh CK et al. Intralesional fluorouracil injection in infantile digital fibromatosis. Arch Dermatol 2002;549-550 18. Albertini JG et al. Infantile digital fibroma treated with mohs micrography surgery. Dermatol Surg 2002;28:959-961 19. Netscher DT et al. Infantile myofibromatosis: case report of solitary hand lesion with emphasis on different diagnosis and management. Ann Plast Surg 2001;46:62-67 20. Falco NA, Upton J. Infantile digital fibromatosis. J Hand Surg Am 1995;20:1014-1020 21. Hardy JD. The ubiquitous fibroblast. Multiple oncogenic potentials with illustrative cases. Ann Surg 1987;205:445-455 22. Sarma DP, Hoffmann EO. Infantile digital fibroma-like tumor in an adult. Arch Dermatol 1980; 116:578-579 23. Bhawan J et al. A myofibroblastic tumor: Infantile digital fibroma (recurrent digital fibrous tumor of childhood). Arch Dermatol 1979; 94:19-36 24. O’Gorman DJ. Infantile digital fibromatosis . Proc R Soc Med 1974; 67:880 25. Bean SF. Infantile digital fibromatosis. Arch Dermatol 1969; 100:124 Anna L. Avik DO* § , Mark N. Halikis MD § Riverside County Regional Medical Center* and Children’s Hospital of Orange County § Left Hand XR- (A) PA (B) oblique B Ultrasound image Preoperative Images A B A Follow-up At 4.5 months postoperative the patient presents for re-evaluation. He has no motion at the DIP joint and there is concern for scar hypertropy vs recurrence. Patient is referred to physical therapy for evaluation of scar therapy and range of motion. Left Hand XR – (A) PA view (B) lateral A B Conclusions Infantile Digital Fibromatosis is a rare benign tumor of children. This case demonstrates the need to further understand the pathology as it affects the normal anatomy. Significant complications can arise in the digit of a child that may need additional reconstructive procedures in the future if intervention is not pursued early in the tumor’s presentation. Although spontaneous regression can occur in 3mo-5 years after the presentation of the lesion, a deformity could have arisen. Thus, in support of the literature observation is recommended for 6 months, then consider intervention based on the criteria listed below. Future research with two large populations of patients with early wide excision versus observation needs to be performed to better understand this condition. However, due to the rare nature of the tumor it is unlikely that this could be accomplished. Mass should be excised when: 1) Size exceeds 1.5 x 1.5 cm squared, or increased progression in size over a 6-month period 2) Loss of range of motion at joint involved, usually DIP 3) Tendon or ligament involvement 4) Deformity of digit or contracture 5) Patient hygiene is compromised Author Year # pts (# lesions) recurrence F/u Treatment deformity tissue involved mass size SF Bean 1969 1 (1) 0 8mo excision 0 0 6x8mm DG O'Gorman 1974 1(multiple) 0 NA steroid cream, obs 0 0 NA Bhawan et al 1979 1 0 1.5yrs wide excision 0 eponychium 1.2cm Sarma &Hoffmann 1980 1(adult) 0 4yrs excision 0 0 1cm, 0.2cm JD Hardy 1987 1 NA NA observation congenital 0 1x3cm Ishii et al 1989 1 (3) 0 3 yrs observation 0 0 0.8,0.1,1 cm Falco and Upton 1995 8(15) 1 3yrs obs then wide exc 0 collateral lig NA Sungur et al 2001 1 0 3yrs local excision contracture 0 1cm Chirayil et al 2001 1 0 1yr excision 0 0 2x2cm Kang et al 2002 1 NA NA excision 0 0 3x3cm Kanwar et al 2002 1 NA NA observation 0 0 2x2.5cm Albertini et al 2002 1(2) 0 2 yrs MMS 0 nail bed 4x5, 10x15mm Oh et al 2005 1 0 2yrs Fluorouracil (5 inj) 0 0 1.4x0.8cm Campbell et al 2007 1 1-size ↑ 22mo shave --> MMS 0 0 2 cm Niamba et al 2007 4(7) 0 1yr observation 0 0 largest 2.5cm Talbot et al 2007 4 0 3-38mo wide exc, amp 0 0 1x1, 1.8x1.2cm Fernandez et al 2007 1 3 NA obs --> local exc Y nail and tendon NA Taylor et al 2008 1(4) postop synd NA exc + recon Y NA NA Failla et al 2009 1(3) 0 14mo imiquimod -->diflucortolone 0 0 NA Netscher et al 2009 1 1 (6mo) NA obs --> wide exc 0 poss collateral lig NA Holmes et al 2011 10(12) 3(sx),2(inj) 5.5yrs surgery vs steroid inj Y 0 NA Spingardi et al 2011 7 7(4 multiple) 8.7yrs surgery 3,axial 2 NA MMS = Mohs microsurgery B
Transcript of Infantile Digital Fibromatosis- Surgical Indications · Infantile Digital Fibromatosis- Surgical...
Infantile Digital Fibromatosis- Surgical Indications
Abstract Infantile digital fibromatosis (IDF) was first described by Reye in 1965 as involving the fingers and toes exclusively. This is a rare, benign tumor that affects the distal digits on the dorsal or border surfaces. The pink, smooth surfaced tumor can present in a nodular pattern or as individual tumors on neighboring digits as kissing lesions. Demonstrating a predictive histology, the tumor appears as with eosinophilic inclusion bodies with spindle-like fibroblasts. The other key finding are dense sheets of small actin filaments. IFD has a very high potential for recurrence, several studies quoting up to 60%. This makes surgical management challenging. Further, the tumor can spontaneously regress as the child ages, altering the potential for necessitating a surgical excision. This project reviews the English publications on management of this rare entity and hopes to elucidate more clear guidelines to surgical indications.
History of Present Illness 11 month old male presents to clinic with history of dorsal mass on left long finger. Birth history was normal. After his birth the mother questioned that he was having difficulty moving the long finger spontaneously with flexion and extension. His mother states the mass developed since 2 months of age. The mass has continued to increase in size. No family history of soft tissue masses.
Physical Exam Capillary brisk. Firm nonblanchable multi-lobed mass at the dorsal aspect of left long finger, crossing the distal interphalangeal (DIP) joint. Sensation patient moves all fingers to stimulation. No transillumination. PROM of MP and PIP joint normal. DIP greatly to 0-15 degrees. Mass size estimated at 3 x 3 x 2.5 cm.
Impression Left LF dorsal soft tissue mass- fibrous, no cystic component
Surgical intervention deemed necessary An ultrasound was obtained of the left long finger to evaluate the nature of the mass and the underlying structure. The mass did not have a distinct separation from the underlying extensor tendon at the distal aspect of the mass. Indications: Loss of range of motion at DIP joint Suspected involvement into the extensor mechanism Patient hygiene compromised Surgery performed Excision of left long finger infantile digital fibroma (Figure A) Split thickness skin graft to left long finger from left groin (Figure B)
Literature Review of management
References: 1. Reye RDK. Recurring digital fibrous tumors of childhood. Arch Pathol 1965; 80:228-231 2. Kang SK et al. A case of congenital infantile digital fibromatosis. Pediatr Dermatol 2002; 19:462-463 3. Kanwar AJ et al. Congenital infantile digital fibromatosis. Pediatr Dermatol 2002; 19:370-371 4. Sungur N et al. Infantile digital fibromatosis: an unusual localization. J Pediatr Surg 2001; 36:1587-1589 5. Chirayil PT et al. Infantile digital fibromatosis: a case report. Burns 2001; 27:89-90 6. Ishii N et al. A case of infantile digital fibromatosis showing spontaneous regression. Br J Dermatol 1989;121:129-133 7. Kawaguchi M et al. A case of infantile digital fibromatosis with spontaneous regression. J Dermatol 1998;25:523-526 8. Spingardi O et al. Infantile digital fibromatosis: our experience and long-term results. Chir Main 2011;30:62-65 9. Holmes WJ et al. Intralesional steroid for management of symptomatic Infantile Digital Fibromatosis J Plast Reconstr Aesthet Surg 2011;64:632-637 10. Failla V et al. Congenital infantile digital fibromatosis: a case report and review of literature. Rare tumors 2009;1:e47 11. Netscher DT et al. Non-malignant fibrosing tumors in the pediatric hand: a clinicopathologic case review. Hand 2009;4:2-11 12. Taylor HO et al. Infantile digital fibromatosis. Ann Plast Surg 2008; 61:472-476 13. Niamba P et al. Further documentation of spontaneous regression of infantile digital fibromatosis Pediatr Dermatol 2007;24:280-284 14. Fernandez-Jorge B et al. An unusual congential presentation of infantile digital fibromatosis. J Pediatr Child Health 2007;43:505-506 15. Campbell LB, Petrick MG. Mohs micrographic surgery for problematic infantile digital fibroma. Dermatol Surg 2007; 33:385-387 16. Talbot C et al. Infantile digital fibromatosis. J Pedatric Orthop B 2007; 16:110-112 17. Oh CK et al. Intralesional fluorouracil injection in infantile digital fibromatosis. Arch Dermatol 2002;549-550 18. Albertini JG et al. Infantile digital fibroma treated with mohs micrography surgery. Dermatol Surg 2002;28:959-961 19. Netscher DT et al. Infantile myofibromatosis: case report of solitary hand lesion with emphasis on different diagnosis and management. Ann Plast Surg 2001;46:62-67 20. Falco NA, Upton J. Infantile digital fibromatosis. J Hand Surg Am 1995;20:1014-1020 21. Hardy JD. The ubiquitous fibroblast. Multiple oncogenic potentials with illustrative cases. Ann Surg 1987;205:445-455 22. Sarma DP, Hoffmann EO. Infantile digital fibroma-like tumor in an adult. Arch Dermatol 1980; 116:578-579 23. Bhawan J et al. A myofibroblastic tumor: Infantile digital fibroma (recurrent digital fibrous tumor of childhood). Arch Dermatol 1979; 94:19-36 24. O’Gorman DJ. Infantile digital fibromatosis . Proc R Soc Med 1974; 67:880 25. Bean SF. Infantile digital fibromatosis. Arch Dermatol 1969; 100:124
Anna L. Avik DO*§, Mark N. Halikis MD§
Riverside County Regional Medical Center* and Children’s Hospital of Orange County §
Left Hand XR- (A) PA (B) oblique
B
Ultrasound image
Preoperative Images
A
B A
Follow-up At 4.5 months postoperative the patient presents for re-evaluation. He has no motion at the DIP joint and there is concern for scar hypertropy vs recurrence. Patient is referred to physical therapy for evaluation of scar therapy and range of motion. Left Hand XR – (A) PA view (B) lateral
A B
Conclusions Infantile Digital Fibromatosis is a rare benign tumor of children. This case demonstrates the need to further understand the pathology as it affects the normal anatomy. Significant complications can arise in the digit of a child that may need additional reconstructive procedures in the future if intervention is not pursued early in the tumor’s presentation. Although spontaneous regression can occur in 3mo-5 years after the presentation of the lesion, a deformity could have arisen. Thus, in support of the literature observation is recommended for 6 months, then consider intervention based on the criteria listed below. Future research with two large populations of patients with early wide excision versus observation needs to be performed to better understand this condition. However, due to the rare nature of the tumor it is unlikely that this could be accomplished. Mass should be excised when: 1) Size exceeds 1.5 x 1.5 cm squared, or increased progression in size over a 6-month period 2) Loss of range of motion at joint involved, usually DIP 3) Tendon or ligament involvement 4) Deformity of digit or contracture 5) Patient hygiene is compromised
Author Year # pts (# lesions) recurrence F/u Treatment deformity tissue involved mass size SF Bean 1969 1 (1) 0 8mo excision 0 0 6x8mm
DG O'Gorman 1974 1(multiple) 0 NA steroid cream, obs 0 0 NA Bhawan et al 1979 1 0 1.5yrs wide excision 0 eponychium 1.2cm
Sarma &Hoffmann 1980 1(adult) 0 4yrs excision 0 0 1cm, 0.2cm JD Hardy 1987 1 NA NA observation congenital 0 1x3cm Ishii et al 1989 1 (3) 0 3 yrs observation 0 0 0.8,0.1,1 cm
Falco and Upton 1995 8(15) 1 3yrs obs then wide exc 0 collateral lig NA
Sungur et al 2001 1 0 3yrs local excision contracture 0 1cm Chirayil et al 2001 1 0 1yr excision 0 0 2x2cm
Kang et al 2002 1 NA NA excision 0 0 3x3cm Kanwar et al 2002 1 NA NA observation 0 0 2x2.5cm Albertini et al 2002 1(2) 0 2 yrs MMS 0 nail bed 4x5, 10x15mm
Oh et al 2005 1 0 2yrs Fluorouracil (5 inj) 0 0 1.4x0.8cm Campbell et al 2007 1 1-size↑ 22mo shave --> MMS 0 0 2 cm Niamba et al 2007 4(7) 0 1yr observation 0 0 largest 2.5cm
Talbot et al 2007 4 0 3-38mo wide exc, amp 0 0 1x1, 1.8x1.2cm Fernandez et al 2007 1 3 NA obs --> local exc Y nail and tendon NA
Taylor et al 2008 1(4) postop synd NA exc + recon Y NA NA
Failla et al 2009 1(3) 0 14mo imiquimod -->diflucortolone 0 0 NA
Netscher et al 2009 1 1 (6mo) NA obs --> wide exc 0 poss collateral lig NA Holmes et al 2011 10(12) 3(sx),2(inj) 5.5yrs surgery vs steroid inj Y 0 NA
Spingardi et al 2011 7 7(4 multiple) 8.7yrs surgery 3,axial 2 NA
MMS = Mohs microsurgery
B
