caso clinico

1. ♀ 31años HCL:620467
• TE: 8 MESES
• SyS: dolor abdominal a predominio de hipocondrio izquierdo, anorexia y
pérdida de peso
• Antecedentes : Niega
• TAC AP DE OI ( 06/06/17 ): tumoración heterogÉnea, bordes lobulados,
componente necrótico en su interior de 12 x10 cm; localizado por
delante del cuerpo y cola del páncreas, considerar GIST VS TUMOR
SÓLIDO PSEUDO PAPILAR
• TEM AP(03/12/17): tumoración heterogénea de 18.3x13.7x18 cm
dependiente de cuerpo y cola de páncreas
• EXAMEN FÍSICO:
• Abdomen con masa prominente en hipocondrio izquierdo poco móvil
de bordes definidos que desplaza parrilla costal en sentido cefálico.
IDX: TUMOR RETROPERITONEAL: D/C TUMOR DE FRANTZ-
GRUBER
Hb: 8.4
Leuc: 34.0
Plaq:633
INR: 1.2
Glucosa: 2.3
Cr:23
BT:2.1
Albúmina: 20
CA 19-9: 21.2
CEA: 1.7

2. • OPERCIÓN EFECTUADA(27/12/17)
• LE+ RESECCIÓN DE TUMOR INTRAABDOMINAL+PANCREATECTOMÍA CORPOROCAUDAL+
ESPLENECTOMÍA EN BLOCK+GASTRECTOMÍA SUBTOTAL+ SEGMENTECTOMÍA HEPÁTICA 3+
COLECISTECTOMÍA+BIOPSIA DE NÓDULO PERITONEAL+ ARTERIZACIÓN
PORTAL+GASTROENTEROANASTOMOSIS BILLROTH 2 TIPO HOFMEISTER FINSTERER+BRAUN
COMPLEMENTARIO+ COLOCACIÓN DE DREN TUBULAR
• HALLASGOS:
• Habierta cavidada abdominal abundante líquido de color amarillo citrino de 3000cc
aproximadamente, en retroperitoneo se evidencia nódulos sugestivos de metástasis, se
reseca el más representativo(congelación: nódulo inflamatorio), no se observa implantes
hepáticos; se evidencia tumoración de 20x20cm de característica heterogénea, bordes
irregulares, poco movil, impresiona depender del cuerpo del páncreas, se encuentra
adherido al segmento 3 del hígado, vesícula biliar, segunda porción del duodeno, cuerpo y
antro gástrico.
• ANATOMÍA PATOLÓGICA:
• SARCOMA DE CÉLULAS DENDRÍTICAS FOLICULARES EXTRANODAL Y NODAL
• Tamaño:20cm
• Mitosis: 6x10 CAP
• Sin invasión linfovascular ni perineural
• Hígado, estómago infiltrado por neoplasia
• Pancreas, bazo, vesícula biliar y epiplon libre de neoplasia maligna.
• BORDE QX. HEPÁTICO <1MM
• MACROMETÁSTASIS GANGLIONAR(7MM) CON ESTENSIÓN EXTRACAPSULAR (12/31)

5. • In general, surgery is the mosteffective treatment modality
and adjuvant radiotherapy has no significant effect on overall
survival of patients. The role of chemotherapy forthe
management of advanced disease is controversial.

6. • Reticulum cells are structural and immune accessory cells
contained in peripheral lymphoid organs.
• Three major subtype sinclude:
• follicular dendritic cells (FDCs)
• interdigitating den-dritic cells (IDCs)
• fibroblastic reticular cells (FRCs)

7. • FDCs are cardinal members of primary and secondary
lymphoid follicles in which they present antigen in a spatial
form to the B cells and maintain humoral immune responses
• They are of mesenchymal origin and express markers of FDC
differentiation including :
• CD21
• CD35
• R4/23
• KiM4

8. Follicular dendritic cell
sarcoma (FDCS)
• 343 FDCS cases were found (English literature)
majority of the patients appeared to have an Asian originwhen the percent of cases
reported from Far East was con-cerned.

9. • 105 patients(31%) had only nodal disease
• 194 (58%) cases had isolatedextranodal
• 35 patients (10%) had both nodal and extran-odal
involvement.

10. A Other rare extranodal sites of FDCS included omentum, peritoneum, ovary, thymus, adrenal gland, abdominal wall,
diaphragm, infratemporal fossa, piriformsinus, oropharynx, hypopharynx, ethmoid/sphenoid sinus, muscle, lip and dura mater.

11. Clinical features.
• Forty-five percent of the cases presented with a local mass(98
out of 217).
• Abdominal pain was the chief manifestationfor most patients
with abdominal involvement (78/236 cases),followed in order
by systemic symptoms (i.e. fever, weightloss, fatigue),
intestinal obstruction, rectal bleeding and dys-pepsia.
• Of note, 11 patients with abdominal disease
wereasymptomatic and diagnosed incidentally.
• Dyspnea, cough,dysphagia and dizziness were among the
chief complaints ofother patients.

12. Clinical features.
• Follow-up information was available in 224 patients
andranged from 2 to 360 months (median, 20 months).
• Localrecurrence and/or distant metastasis occurred in 44.6%
of patients after initial treatment.
• 63 patients (28.1%) experi-enced local recurrence at a median
of 15 months and 61 cases(27.2%) developed distant
metastasis at a median of 18.5 months.
• Twenty-four patients had local and distant recur-rence at the
same time.
• Common sites of metastasis were the lung (9.4%), lymph
nodes (8.9%), liver (9.4%) and bone(3%).

13. prognostic variables
• Data on tumor size were available in 189 cases with anaverage
value of 7 cm (range 1–22 cm).
• Necrosis was presentin 54.1% of the cases (79 out of 146)
• the mitotic countranged between zero and fifty mitotic figures
per 10 high-power fields.

14. Associationsbetweentheseclinicopathologicalfeaturesandadverseoutcomewere
investigatedunivariately
• statistically significant for:
• age (p = 0.016)
• lymphoplasmacytic infiltration (p = 0.011)
• tumor size(p = 0.04)
• mitotic count (p = 0.024)

16. • Youngage at diagnosis (≤40 years)
• absence of lymphoplasmacyticresponse
• large tumor size (>6 cm)
• high mitotic count(≥5/10 high-power field)
• predicted poor prognosis.

17. multivariate regression
analysis
• of 50 FDCS cases with
• sufficient clinicopathological data,
• only the lymphoplasma-cytic infiltration and tumor size
retained their predictor values

18. • at least 18.6%of the patients were diagnosed erroneously at
presentation(64 out of 343).
• Entities most commonly confused with FDCS included:
• - undifferentiated carcinoma
• - lymphoma
• - malignant fibrous histiocytoma
• - peripheral nerve sheathtumor
• - ectopic meningioma
• - inflammatory pseudotumor
• - granulomatous inflammation
• - gastrointestinal stromal tumor
• - unclassified sarcoma.

19. Treatment and outcome.
• Two-hundred and eighty-two cases presented with an early disease
(85.4%)
• as compared to 21 patients (6.4%) with locally advanced dis-ease
• 27 patients (8.2%) with distant metastasis at presentation
• A total of 216 cases had sufficient follow updata for statistical
analysis:
• - 179 early
• - 18 locally advanced
• - 19 distant
• Disease stage was not shown to be a sig-nificant factor on OS of
patient

20. Diseasestagewasnotshowntobeasig-nificantfactoronOSof
patient
Fig. 1. Kaplan–Meier curve of overall survival (OS) of patients with follicular dendritic cell
sarcoma (FDCS). (A) Stage at presentation (early, locally advanced,or with distant metastasis)
had no significant influence on OS of cases (p = 0.44).

21. • Median survival for local disease was 168 months(range 2–360
months)
• it could not be reached in patients with locally advanced and
distant disease due to significantly lower number of these
cases and their shorter follow-up time.
• 2-year survival rates:
• - for early  82.4%,
• - locally advanced  80%,
• - distant metastatic diseases  42.8%

22. • Most of the patients with local disease were treated with
surgery with or without adjuvant therapy (147 cases).
• Others received combined chemotherapy and radiotherapy(2
cases)
• radiotherapy alone (3 cases)
• chemotherapy alone(2 cases)
• 1 case received no treatment.
• Treatment was not reported for 24 cases.

23. Patientswhoweremanagedwithsurgeryalone(78cases)hadsignificantly
betterOSwhencomparedto7caseswhoreceivedothertreatment
modalities(p<0.001)
(B) Localized FDCS cases who received surgery had a significantly better OS when
compared to patients who had other treatment modalities (p < 0.001). There was no
significant difference between OS of patients who received adjuvantradiotherapy
and surgery alone (p = 0.474).

24. • A comparison was made betweenpatients who had surgery
alone and 51 patients who receivedadjuvant radiotherapy
following surgery (Fig. 1B)
• Our anal-ysis revealed that adjuvant radiotherapy had no
statistically significant influence on OS of patients with early
disease(p = 0.47).

25. Therapies
• late disease were diverse:
• - Fourteen out of 18 patients (77.8%) with locallyadvanced disease had surgical resection
as a primary treat-ment modality.
• Three of them received adjuvant radiotherapy
• two cases had adjuvant chemotherapy
• one case received both of them.
• The agents used consisted mostly of the regimens designed for the management of
aggressive lym-phoma, including :
• - CHOP (cyclophosphamide, vincristine,doxorubicine, prednisolone)
• - ICE (ifosfamide, carboplatin,etoposide)
• - CHEOP and ABVD (adriamycin, bleomycin,vincristine, dacarbazine).
• Of the remaining 19 cases with distant metastasis at presentation:
• - 9 received chemotherapy primarily with or without radiotherapy
• - one received neoad-juvant chemotherapy preceding portal vein embolization
and surgery
- one received surgery alone
- four received no ther-apy.
- Treatment data could not be obtained in four cases.
Due to the low number of cases with various treatment modalities,further analysis could not
be performed.

26. Discussion
• FDCS is the most common histological subtype of den-dritic
cell tumors with a fairly benign course.
• It behaves like an intermediate grade sarcoma
• risk of:
• - localrecurrence (28.1%)
• - distant metastasis (27.2%).

27. • Similar toother soft tissue sarcomas:
• - large tumor size (≥6 cm)
• - presence of coagulative necrosis
• - high mitotic count (≥5 per 10 high-power fields)
• - significant cytologic atypia were shownto be associated with
poor prognosis
In contrastto other reports, with univariate and multivariate
analyses:
- necrosis and intra-abdominal involvement were not found to
be the predictors of adverse outcome in this study.

28. • stage was not associated with poor survival in FDCS patients
as shown by us and one other study [a].
• asso-ciation between:
• - young age
• - high mitotic count
• adverseoutcome could not be demonstrated with multivariate
anal-ysis.
• only the tumor size and lymphoplasmacyticinfiltration in
tumor tissue are accepted to be important pro-gnostic
parameters.
(a) Perkins SM, Shinohara ET. Interdigitating and follicular dendritic cellsarcomas. A
SEER analysis. American Journal of Clinical Oncology2012 (in press).

29. • Surgery should be themainstay of treatment for early FDCS
cases as patients treatedwith surgery had better OS when
compared to other treatmentmodalities.
• However, adjuvant radiotherapy did not have asignificant
influence on OS. Prior studies have also demon-strated that
adjuvant treatments had no significant effect ondisease free
survival after a radical surgical resection [b].
(b) De Pas T, Spitaleri G, Pruneri G, et al. Dendritic cell sarcoma: ananalytic overview of the literature and
presentation of original fivecases. Critical Reviews in Oncology/Hematology 2008;65:1–7.

30. • the number of patients withlocally advanced and distant
metastatic diseases was so lowand therapies received by them
were diverse.
• In most of thesecases, surgery was performed to reduce
tumor load. The roleof surgery in late disease is not clear, but
only 2 out of 23patients who received combined adjuvant
chemotherapy andradiotherapy succumbed to their disease
(both had metastaticdisease at onset). This highlights the
importance of adjuvanttherapies in advanced FDCS patients.