Mycosis fungoides

Name
Mycosis fungoides
ICD-O-1 Morphology
9700/3
Effective 1978 - 1991
ICD-O-2 Morphology
9700/3
Effective 1992 - 2000
ICD-O-3 Morphology
9700/3
Effective 2001 and later
Reportable
for cases diagnosed 1978 and later
Primary Site(s)
C440-C449, C510-C512, C518-C519, C600-C602, C608-C609, C632

Help me code for diagnosis year :

Grade
5 - T-cell
Module Rule
None
Alternate Names
CTCL
Folliculotropic MF
Granulomatous slack skin
GSS
Ketron-Goodman disease
Kiel: small cell, cerebriform
Lukes -Collins: cerebriform T
MF
Pagetoid reticulosis
Woringer-Kolopp type
Definition
Variants include Follicular MF, Pagetoid reticulosis, Granulomatous slack skin.

Mycosis fungoides is a mature T-cell lymphoma presenting in the skin with patches/plaques and characterized by epidermal and dermal infiltration of small to medium-sized T-cells with cerebriform nuclei. So-called "Pautrier microabscesses," consisting of aggregates of cerebriform (highly folded) cells in the epidermis are highly characteristic.

Although patients with mycosis fungoides (MF) typically experience an indolent disease course, a minority undergo a process of large-cell transformation (LCT), which often heralds more aggressive disease and shortened survival. Regrettably, most dermatologists are unfamiliar with LCT, and even fewer understand how to recognize it clinically. Because a diagnosis of LCT typically triggers more aggressive therapy and/or referral to cutaneous T-cell lymphoma (CTCL) centers, it is paramount for clinicians to be able to recognize suspect lesions visually.
Abstractor Notes
Mycosis fungoides (MF) as a rule is limited to the skin with widespread distribution for a protracted period. Extracutaneous disease may occur in advanced stages mainly to lymph nodes, liver, spleen, lungs, and blood. MF has an indolent clinical course with slow progression over years or sometimes decades, from patches to more infiltrated plaques and eventually tumors. MF characteristically shows a combination of patches, plaques, and tumors which show ulceration. Uncommonly, patients present with or develop an erythrodermic stage of disease that lack the hematalogic criteria of Sezary syndrome.

There are three variants to mycosis fungoides: folliculotropic MF, pagetoid reticulosis, and granulomatous slack skin. All are coded to 9700/3.

Folliculotropic MF is characterized by the presence of follicular infiltrates of atypical (cerebriform) CD4+ T lymphocytes often with sparing of the epidermis. Most cases show mucinous degeneration of the hair folicles (follicular mucinosis) but similar cases without follicular mucinosis have been reported. The lesions preferentially involve the head and neck area and often present with grouped follicular papules associated with alopecia. The disease is less accessible to skin-targeted therapies. The disease-specific 5-year survival is approximately 70-80%, which is significantly worse than that of patients with classical plaque stage MF.

Pagetoid reticulosis is characterized by the presence of patches or plaques with an intraepidermal proliferator of neoplastic T-cells.

Granulomatous slack skin (GSS) is an extremely rare subtype of Cutaneous T=cell lymphoma characterized by the slow development of folds of skin in the major skin folds (axilla, groin) and histologically by a granulomatous infiltrate. Most patients have an indolent clinical course.
Definitive Diagnostic Methods
Histological confirmation
Genetics Data
T-cell receptor genes are clonally rearranged
Immunophenotyping
CD2+
CD3+
CD4+
CD4- Pagetoid reticulosis
CD5+
CD7-
CD8+ pediatric
CD8-
CLA+
TCRa beta +
Treatments
Chemotherapy
Immunotherapy
Other RX
Radiation
Surgery
Transformations to
No Transformations
Transformations from
No Transformations
Corresponding ICD-9 Codes
202.1 Mycosis fungoides
Corresponding ICD-10 Codes
C84.0 Mycosis fungoides
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C84.0 Mycosis fungoides
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None