Download - Catalyst

Mass around the pediatric knee
2 year old Female
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2wk h/o L Popliteal mass presents to o/p clinic
Asymptomatic: ROM, systemic
ROS: no F/C, normal growth
PE: VS wnl, cheerful 2 yo, full ROM w no pain
– L 4x6cm soft mass, defined boarder central fossa,
texture as surrounding tissue. No TTP, erythema.
Motor, sense, pulses intact.
What Now?
Observation
Ultrasound
CT scan
MRI
Biopsy
Surgery
Amputation
Diagnosis
Dr Kenneth Gow, MD. Associate Professor, Division of Pediatric Surgery, Department of Surgery, UW
U/S for Initial Evaluation of Suspicious
Soft Tissue Mass
• 100:1::Benign:malignant present to Primary MD
• 358 pt’s initial eval by US
• 8 categories:
– 1-5: cyst, vasc lesion, lipoma
– 6-8: further eval: Pain, Mass >5 cm, Deep to fascia,
increasing size, heterogeneous, distort anatomy, alt
doppler flow.
• 78% Benign: cat 1-5
– 2+ yr f/u: No false neg
• 74/358 cat 6+
– 62/74 f/u MRI
• 10 sarcoma = 2.8%
Clinical Radiology (2009) 64, 615-621 Ultrasound for initial evaluation and triage of clinically suspicious
soft-tissue massesA. Lakkarajua, R. Sinhaa,1, R. Garikipatia,2, S. Edwardb, P. Robinsona,*
US findings
• The site of palpable abnormality along the
posteromedial aspect of the left popliteal
fossa reveals a prominent ovoid, flattened,
intermediate echogenicity subcutaneous
structure likely corresponding to fat. This
region measures approximately 3.7 x 1.4 x 4.2
cm (11.4 cc). Region is soft to transducer
pressure. No cystic masses are noted. Lesion
characteristics are most consistent with a
lipoma.
Pediatric Adipose Tumors
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Lipoma
Lipoblastoma
Hibernoma
Liposarcoma
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Coffin CM & Williams RA. Pediatr Pathol 1992. 12:857-864
64%
30%
2%
4%
Lipoblastoma
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“Infantile lipoma”
Rare, benign, mesenchymal tumor
More common in Trunk and Extremities
Tx: excision with close f/u for recurrence
• 16 cases of lipoblastomas in Taiwan
• 4 recurrences: neck (n=2) and lower extremities (n=2)
• 3 year follow-up recommended
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Coffin CM. Adipose and myxoid tumors. In: Coffin CM, Dehner LP, O'Shea PA. Pediatric Soft Tissue Tumors: A Clinical,
Pathological, and Therapeutic Approach. New Salt Lake City, Utah: JA Majors Company; 1997:254-276.
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Journal of Pediatric Surgery, Vol 35, No 10 (October), 2000: pp 1511-1513. Is Surgical Treatment of Lipoblastoma Always Necessary? By
G. Mognato, G. Cecchetto, M. Carli, E. Talenti, E.S.G. d’Amore, F. Pederzini and M. Guglielmi Padua, Italy and Trento, Italy
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Jung SM, et al. Pediatr Surg Int 2005. 23:1-4
Lipoblastoma
Lipoblastoma
• 59 patients
• + S100, + CD34
• 10 pt’s had other medical conditions:
– Macrocephaly, seizures, autism, Sturge-Weber
Coffin CM, Lowichik A, Putnam A. Am J Surg Pathol. 2009 Nov;33(11):1705-12. Lipoblastoma (LPB): a
clinicopathologic and immunohistochemical analysis of 59 cases.
Hibernoma
• “Brown fat” tumor
– Term from hibernating animals
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Multiple vacuoles in brown fat cells
Very rare tumor <100 reported in literature
Typically found on trunk, thigh
Peak 3rd decade
Hibernoma of the Thigh: A CASE REPORTANTHONY F. MERLINO and RALPH F. PIKE J Bone Joint
Surg Am. 1973;55:406-408.
Pediatric Baker cyst
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Synovial fluid collection. Medial gastroc: semitendiosis.
Not associated with meniscus tear, ACL tear, trauma.
Incidental finding of parent
Spontaneous resolution 10-20 months
Recurrence after Sx, reported 42-50%
Typical treatment is conservative: parent anxiety
driving force.
• Attempt aspiration – unlike adults, no communication
with articular capsule.
Chen, JC et al. Modified surgical method for treating Baker’s cyst in
children. The Knee 15 (2008) 9-14
Malignant Bone tumors in children
• Osteosacroma
• Ewing’s sarcoma
– Aggressive, classically found in bone diaphysis
Osteosarcoma
• Most common primary tumor of bone
• Common presentation: painful, hard, fixed
poorly defined mass
• Irradiation, Family h/o retinoblastoma
• XR:
– ‘star-burst’ – spiculated pattern from calcified
malignant osteoid.
– ‘Codman’s triangle’ periosteal lifting.
Osteosarcoma
Malignant Soft tissue tumors at Knee
• Soft tissue Sarcoma 8% of all neoplasms in
children.
• Rhabdomyosarcoma (RMS)
– 60% of Soft tissue sarcomas (STS)
• non-RMS overall
– Synovial sarcoma most common 32%
– Includes: adipose, muscle, nerve, vascular, fibrous,
and extra-skeletal types
Rhabdomyosarcoma
• sarcomas of skeletal muscle
• two forms:
– Embryonal –
• most common form
• children and young adolescents
• occurs in head, neck, retroperitoneum and genitourinary system,
rarely in the extremities
• metastasis is frequent and prognosis is grave
– alveolar rhabdomyosarcoma:
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older children and adolescents
Occurs in extremeties
Lymph node metastasis is common
w/o recurrent metastatic lesions, survival may be 80% or higher
Rhabdomyosarcoma - Histology
• muscle spindle cells w/ blunt
ended nuclei
• cross-striations in malignant
cells needs to be established
by electron microscopy to
validate the diagnosis
– this helps r/o malignant
fibrous histiocystoma
Dermatologyoutlines.com
Rhabdomyosarcoma
– RMS –alveolar most common in SEER study
• Typically axial skeleton but poor prognosis when
involves extremities
• More common in kids <4 yo (non RMS STS more
common >4 yo)
• Tx: 80% underwent surgical extirpation
• Nodal spread 50% of bx’d pts
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SEER database 1175pt 33yrs J Surg Res. 2010 May 21. Incidence and Outcomes of Extremity Soft-Tissue
Sarcomas in Children. Cheung MC, Zhuge Y, Yang R, Ogilvie MP, Koniaris LG, Rodríguez MM, Sola JE.
Rhabdomyosarcoma
– Positive predictors:
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Local dz 5y:80%/30
NR STS 5y:85%/56
Sx resect 10y:75%/43
adipose, fibrous tissue
– Neg predictors: Muscle (except synovial sarcoma),
Nerve
– Strongest predictor of out come: stage of disease
at diagnosis
Ghavimi F. Mandell LR. Heller G. Hajdu SI. Exelby P. Department of Pediatrics,
Memorial Sloan-Kettering Cancer Center, New Cancer. 64(11):2233-7, 1989 Dec 1.
From 1970 to 1987, 34
Non-RMS
• appendicular skeleton more favorable prognosis
• More common in older kids
• Treatment: Surgical resection
Clinical Decision Making
Summary
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Broaden differential
Ultrasound: screen
Surgical excision if symptomatic
Close communication with Pathology
Monitor for recurrence
Works Cited
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Dr Kenneth Gow, MD. Associate Professor, Division of Pediatric Surgery, Department of Surgery, UW
Clinical Radiology (2009) 64, 615-621 Ultrasound for initial evaluation and triage of clinically suspicious soft-tissue
massesA. Lakkarajua, R. Sinhaa,1, R. Garikipatia,2, S. Edwardb, P. Robinsona
Coffin CM & Williams RA. Pediatr Pathol 1992. 12:857-864
Coffin CM. Adipose and myxoid tumors. In: Coffin CM, Dehner LP, O'Shea PA. Pediatric Soft Tissue Tumors: A
Clinical, Pathological, and Therapeutic Approach. New Salt Lake City, Utah: JA Majors Company; 1997:254-276.
G. Mognato, G. Cecchetto, M. Carli, E. Talenti, E.S.G. d’Amore, F. Pederzini and M. Guglielmi Padua Is Surgical
Treatment of Lipoblastoma Always Necessary? Journal of Pediatric Surgery, Vol 35, No 10 (October), 2000: pp
1511-1513.
Coffin CM, Lowichik A, Putnam A.Am J Surg Pathol. 2009 Nov;33(11):1705-12. Lipoblastoma (LPB): a
clinicopathologic and immunohistochemical analysis of 59 cases.
ANTHONY F. MERLINO and RALPH F. PIKE. Hibernoma of the Thigh: A CASE REPORT J Bone Joint Surg Am.
1973;55:406-408.
Ward EE, Jacobson JA, Fessell DP, Hayes CW, van Holsbeeck M. Sonographic detection of Baker's cysts: comparison
with MR imaging. AJR Am J Roentgenol. 2001 Feb;176(2):373-80
Ghavimi F. Mandell LR. Heller G. Hajdu SI. Exelby P. Department of Pediatrics, Memorial Sloan-Kettering Cancer
Center, New Cancer. 64(11):2233-7, 1989 Dec 1. From 1970 to 1987, 34
SEER database 1175pt 33yrs J Surg Res. 2010 May 21. Incidence and Outcomes of Extremity Soft-Tissue Sarcomas
in Children. Cheung MC, Zhuge Y, Yang R, Ogilvie MP, Koniaris LG, Rodríguez MM, Sola JE.